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ID

44146

Description

Background and Purpose: The purpose of the DESCRIBE study is to document the natural course of diseases in context of medical care of neurodegenerative disorders as well as cerebrovascular diseases and to perform phenotyping of the test persons. Disease-spanning analyses of the measured clincial data, the results of the imaging processes, results of the analysis of the biometerials including genetic analysis are planed. Principal Investigator: Prof. Klockgether, Dr. Spottke DZNE, Bonn. Source: DESCRIBE-Studie http://www.dzne.de/forschung/forschungsbereiche/klinische-forschung/studien/describe-bn006.html Participating Study Sites: Berlin, Bonn, Göttingen, Dresden, Köln, Magdeburg, München, Rostock/Greifswald, Tübingen

Lien

http://www.dzne.de/forschung/forschungsbereiche/klinische-forschung/studien/describe-bn006.html

Mots-clés

  1. 24/02/2016 24/02/2016 -
  2. 01/03/2016 01/03/2016 -
  3. 02/03/2016 02/03/2016 -
  4. 03/03/2016 03/03/2016 -
  5. 03/03/2016 03/03/2016 -
  6. 04/03/2016 04/03/2016 - Julian Varghese
  7. 07/03/2016 07/03/2016 -
  8. 09/03/2016 09/03/2016 -
  9. 09/03/2016 09/03/2016 -
  10. 09/03/2016 09/03/2016 -
  11. 10/03/2016 10/03/2016 -
  12. 10/03/2016 10/03/2016 -
  13. 13/04/2021 13/04/2021 - Dr. rer. medic Philipp Neuhaus
  14. 20/09/2021 20/09/2021 -
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20 septembre 2021

DOI

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Creative Commons BY-NC 3.0

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    DESCRIBE (DZNE – Clinical Register Study of neurodegenerative Disorders) CRF Diagnostic Criteria

    CRF Diagnostic Criteria

    Date of visit
    Description

    Date of visit

    Alias
    UMLS CUI-1
    C1320303
    Date of visit
    Description

    date of visit

    Type de données

    date

    Alias
    UMLS CUI [1]
    C1320303
    Study-ID:
    Description

    study id

    Type de données

    text

    Alias
    UMLS CUI [1]
    C2826693
    Visit:
    Description

    visit name

    Type de données

    text

    Alias
    UMLS CUI [1]
    C2826704
    Mild cognitive impairment (MCI)
    Description

    Mild cognitive impairment (MCI)

    Alias
    UMLS CUI-1
    C1270972
    Is the patient currently diagnosed with a MCI according to Winblad & Petersen 2004?
    Description

    current MCI

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1270972
    UMLS CUI [1,2]
    C0150312
    Cognitive Impairment 1. Subjective deterioration of cognition observed by the patient or an informant and/or...
    Description

    cognitive degration

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1270972
    UMLS CUI [1,2]
    C0854193
    2. Evidence of degradation of cognition in time due to psychometric tests
    Description

    psychometric test proving cognitive degradation

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1270972
    UMLS CUI [1,2]
    C0033920
    3. Ability to perform activities of everyday living/ minimal impairment of complex instrumental everyday functions
    Description

    everyday functioning

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0562507
    4. No signs of disturbed consciousness, of a delirium, of a dementia or of an amnesic aphasia that cannot be explained better by other psychotic illnesses (e.g. a disorder in connection with psychotropic substances or major depression).
    Description

    signs of disturbed consciousness

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0234428
    UMLS CUI [2]
    C0011206
    UMLS CUI [3]
    C0497327
    Operationalization 1. Wordlist - delayed word retrieval
    Description

    At least <= 1.5*SD deficit (in relation to age, gender and professional status), determined with Baseler CERAD Tools, scored in one of the following CERAD tests

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0456660
    2. Visuospatial ability - Drawing
    Description

    At least <= 1.5*SD deficit (in relation to age, gender and professional status), determined with Baseler CERAD Tools, scored in one of the following CERAD tests

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0814069
    UMLS CUI [2]
    C0565772
    3. Visuospatial ability - Delayed retrieval
    Description

    At least <= 1.5*SD deficit (in relation to age, gender and professional status), determined with Baseler CERAD Tools, scored in one of the following CERAD tests

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0814069
    4. Trial making test A or B
    Description

    At least <= 1.5*SD deficit (in relation to age, gender and professional status), determined with Baseler CERAD Tools, scored in one of the following CERAD tests

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0040604
    5. Semantic or phonetic fluency
    Description

    At least <= 1.5*SD deficit (in relation to age, gender and professional status), determined with Baseler CERAD Tools, scored in one of the following CERAD tests

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0233714
    6. Object naming
    Description

    At least <= 1.5*SD deficit (in relation to age, gender and professional status), determined with Baseler CERAD Tools, scored in one of the following CERAD tests

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0451342
    7. No abnormal finding FAQ
    Description

    normal finding FAQ

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C3827007
    UMLS CUI [1,2]
    C0205307
    Classification a. Amnesic MCI single domain (only memory)
    Description

    amnesic MCI single domain

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1270972
    b. Amnesic MCI multi domain (memory and one additional area)
    Description

    amnesic MCI multi domain

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1270972
    c. Non-amnesic MCI single domain (one area, except memory)
    Description

    non-amnesic MCI single domain

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1270972
    d. Non-amnesic MCI multi domain (several areas, except memory)
    Description

    non-amnesic MCI multi domain

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1270972
    Dementia in Alzheimer's Disease (AD)
    Description

    Dementia in Alzheimer's Disease (AD)

    Alias
    UMLS CUI-1
    C3665464
    Is the patient currently diagnosed with AD according to NINCDS/ADRDA-Criteria (McKhann et al. 1984)?
    Description

    NINCDS/ADRDA-Criteria AD diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C3665464
    UMLS CUI [1,2]
    C2828081
    I. Necessary requirements Evidence of dementia in a clinical examination including neuropsychological tests
    Description

    Clinical probable AD

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0497327
    UMLS CUI [2]
    C0027902
    Deficits in at least two cognitive areas
    Description

    cognitive deficits

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0009241
    Progressive disturbance of memory and other cognitive functions
    Description

    progressive disturbance of memory

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1864985
    UMLS CUI [1,2]
    C0025260
    UMLS CUI [2,1]
    C1864985
    UMLS CUI [2,2]
    C0392335
    No consciousness disturbance
    Description

    no consciousness disturbance

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0424525
    Onset between age of 40 and 90, mostly 65 and older
    Description

    age at onset

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0150907
    No evidence of other system- or brain diseases as cause
    Description

    other system- or brain diseases

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0442893
    UMLS CUI [2]
    C0006111
    II. Subsidiary Findings Progressive deterioration of specific cognitive functions, e.g. of speech (aphasia), motor activity (apraxia) and perception (agnosia)
    Description

    progressive deterioration of specific cognitive functions

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0854193
    UMLS CUI [1,2]
    C0205329
    Impairment of everyday activities and emerging of behavior modifications
    Description

    impairment of everyday activities and behavioral change

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0562507
    UMLS CUI [1,2]
    C0221099
    UMLS CUI [2]
    C0542299
    Family history of similar disorders (especially if neurologically proven)
    Description

    family history of similar disorders

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0241889
    Results of additional examinations: Evidence of - progressively evolving in follow ups- cerebral atrophy due to imaging diagnostics
    Description

    cerebral atrophy

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0235946
    Normal finding or unspecific changes in EEG
    Description

    normal finding or unspecific changes in EEG

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0234550
    UMLS CUI [1,2]
    C0205307
    UMLS CUI [2,1]
    C0234550
    UMLS CUI [2,2]
    C0750540
    Normal finding of cerebrospinal fluid
    Description

    normal finding of cerebrospinal fluid

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0007806
    UMLS CUI [1,2]
    C0205307
    III. Matching with a probable diagnosis of Alzheimer's disease Temporal stagnation in course of disease
    Description

    diagnosis of Alzheimers disease

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1848980
    UMLS CUI [1,2]
    C0242656
    Attendant symptoms like depression, insomnia, incontinence, illusion, hallucination, delusion, sudden aggressive outbreaks, sexual dysfunction and weight loss
    Description

    attendant symptoms

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0011581
    UMLS CUI [2]
    C0917801
    UMLS CUI [3]
    C0021167
    UMLS CUI [4]
    C0020903
    UMLS CUI [5]
    C0018524
    UMLS CUI [6]
    C0011253
    UMLS CUI [7]
    C0001807
    UMLS CUI [8]
    C0549622
    UMLS CUI [9]
    C0936227
    Neurological distinctive feature (particularly in case of advanced disease) like elevated muscle tonus, myoclonus or gait disturbance
    Description

    neurological distinctive feature

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1704258
    UMLS CUI [1,2]
    C0205494
    UMLS CUI [2]
    C0026841
    UMLS CUI [3]
    C0027066
    UMLS CUI [4]
    C1856566
    Epileptic seizure (in case of advanced disease)
    Description

    epileptic seizure

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1411824
    CT commensurate with age
    Description

    CT commensurate with age

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0040405
    UMLS CUI [1,2]
    C0001783
    IV. Exclusion Criteria Sudden, apoplectic onset
    Description

    sudden apoplectic onset

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1388315
    UMLS CUI [1,2]
    C1276802
    Focal neurological signs like hemiparesis, sensorial failures, visual field defects or problems with coordination in early stage of disease
    Description

    focal neurological signs

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0018989
    UMLS CUI [2]
    C0152027
    UMLS CUI [3]
    C0004134
    UMLS CUI [4]
    C3887875
    Epileptic seizures or gait disturbance at onset or in early stage of disease
    Description

    epileptic seizures or gait disturbance

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1411824
    UMLS CUI [2]
    C1856566
    V. Clinical possible AD Dementia syndrome with atypical symptomatic with regard to onset, course and deficit profile, in absence of other neurological, psychiatric or internal disease that could cause the dementia syndrome
    Description

    dementia syndrome with atypical symptomatic

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0497327
    UMLS CUI [1,2]
    C0231220
    Second systemic or brain disease that could cause dementia, but is commonly not regarded as a substantial cause
    Description

    second systemic or brain disease

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0442893
    UMLS CUI [2]
    C0006111
    Progressive severe cognitive deficit without notable different cause
    Description

    progressive severe cognitive deficit

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0009241
    UMLS CUI [1,2]
    C0205329
    UMLS CUI [1,3]
    C0205082
    Posterior cortical atrophy (PCA)
    Description

    Posterior cortical atrophy (PCA)

    Alias
    UMLS CUI-1
    C0235946
    UMLS CUI-2
    C0205095
    Is the patient currently diagnosed with PCA according to the criteria of Mendez et.al. 2002?
    Description

    PCA diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0235946
    UMLS CUI [1,2]
    C0205095
    Main symptoms (all symptoms have to be present) A. Insidious onset and gradual progression
    Description

    insidious onset and gradual progression

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1298634
    UMLS CUI [2]
    C1854645
    B. Presentation with visual disorders while visual function intact
    Description

    visual disorders

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0042790
    C. Evidence of prominent complex visual deficits
    Description

    prominent complex visual deficits

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C3665347
    UMLS CUI [1,2]
    C0205402
    UMLS CUI [1,3]
    C0439855
    Elements of Balint syndrome
    Description

    Balint syndrome

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C4707368
    Visual agnosia
    Description

    visual agnosia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0234502
    Dressing apraxis
    Description

    dressing apraxia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0234529
    Topographic disorientation
    Description

    topographic disorientation

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0233407
    UMLS CUI [1,2]
    C1639223
    D. Relatively small memory deficits and word fluency deficits
    Description

    relatively small deficits of memory and word fluency

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0233794
    UMLS CUI [1,2]
    C1843272
    UMLS CUI [2,1]
    C2018029
    UMLS CUI [2,2]
    C1843272
    E. Relatively good awareness of disability with or without depression
    Description

    relatively good awareness of disability

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0424227
    UMLS CUI [1,2]
    C0205170
    Subsidiary diagnosis A. Presenile onset
    Description

    presenile onset

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0750901
    B. Alexia
    Description

    alexia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0002018
    C. Elements of Gerstmann syndrome
    Description

    Gerstmann syndrome

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0017494
    D. Ideomotor apraxia
    Description

    ideomotor apraxia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0234523
    E. Physical examination within the norms
    Description

    physical examination

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0031809
    F. Neuropsychiatric: Prominent impairment of perceptual functions
    Description

    impairment of perceptual functions

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0233746
    Imaging: Prominent occipitoparietal abnormality (especially in functional imaging) with relative recess of frontal and mesiotemporal regions
    Description

    occipitoparietal abnormality

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1704258
    UMLS CUI [1,2]
    C1184145
    UMLS CUI [1,3]
    C0442030
    Vascular dementia
    Description

    Vascular dementia

    Alias
    UMLS CUI-1
    C0011269
    Is the patient currently diagnosed with VD according to the criteria of NINDS/AIREN (Roman et al. 1993?)
    Description

    VD diagnoses

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0011269
    I. Dementia Cognitive impairment regarding previous higher functional status manifested by disturbance of memory
    Description

    cognitive impairment

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0009241
    UMLS CUI [2]
    C0233794
    Orientation
    Description

    and at least two of the following abilities

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0029266
    Attention
    Description

    attention

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0004268
    Speech
    Description

    speech

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0037817
    Spatial visualization ability
    Description

    spatial visualization ability

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0679024
    Ability to judge
    Description

    ability to judge

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0022423
    Action ability
    Description

    action ability

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1820715
    Abstraction ability
    Description

    abstraction ability

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0679195
    Motor control
    Description

    motor control

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0517961
    Praxia
    Description

    praxia

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0871470
    UMLS CUI [1,2]
    C0234621
    Activities of daily living
    Description

    everyday activities

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0001288
    Exclusion criteria Disturbed consciousness
    Description

    disturbed consciousness

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0549249
    Delirium
    Description

    delirium

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0011206
    Psychosis
    Description

    psychosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0033975
    Severe aphasia
    Description

    severe aphasia

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0003537
    UMLS CUI [1,2]
    C0205082
    Prominent sensomotoric disorder, which makes testing impossible
    Description

    sensomotoric disorder

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0036675
    Systemic or other brain diseases, that can cause cognitive disorders for their part
    Description

    systemic or other brain diseases

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2874347
    II. Cerebrovascular disease Central focal neurological signs with or without anamnestic stroke and signs of relevant cerebrovascular disease in CT/MRT
    Description

    cerebrovascular disease

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1392298
    UMLS CUI [2]
    C0038454
    UMLS CUI [3]
    C0007820
    A. cerebri anterior, bilateral
    Description

    Cerebrovascular lesions in radiologic finding considered relevant: Localization Strokes great vessel region:

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0149561
    UMLS CUI [1,2]
    C0238767
    A. cerebri anterior
    Description

    Cerebrovascular lesions in radiologic finding considered relevant: Localization Strokes great vessel region:

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0149561
    Parietotemporal or temporoparietal association complex
    Description

    parietotemporal or temporoparietal association complex

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3498498
    UMLS CUI [2]
    C3495412
    Superiore frontale and parietal watershed zone
    Description

    watershed infarction

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0333557
    Disease of small vessels: Basal ganglia and frontal lacuna of white matter
    Description

    basal ganglia and frontal lacuna of white matter

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0152295
    UMLS CUI [1,2]
    C1459585
    UMLS CUI [1,3]
    C0004781
    UMLS CUI [2,1]
    C0152295
    UMLS CUI [2,2]
    C1459585
    UMLS CUI [2,3]
    C0016733
    Broad periventricular white matter lesions
    Description

    periventricular white matter lesions

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C2675929
    UMLS CUI [1,2]
    C0228157
    Bilateral thalamus lesions
    Description

    bilateral thalamus lesions

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C2026261
    UMLS CUI [1,2]
    C0238767
    Severity: great vessel lesions in dominant hemisphere
    Description

    great vessel lesions

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0221198
    UMLS CUI [1,2]
    C3641002
    UMLS CUI [1,3]
    C0228465
    UMLS CUI [1,4]
    C1527180
    Bilateral lesions of great vessel in hemisphere
    Description

    bilateral lesions of great vessel

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0221198
    UMLS CUI [1,2]
    C3641002
    UMLS CUI [1,3]
    C0228465
    UMLS CUI [1,4]
    C0238767
    Leukoencephalopathy => 25% of white matter
    Description

    leukoencephalopathy

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0023524
    III. Combination of I. and II. Onset of dementia within three months after stroke
    Description

    onset of dementia

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0011269
    UMLS CUI [1,2]
    C0332162
    Abrupt degeneration of cognitive functions
    Description

    abrupt degeneration of cognitive functions

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0338656
    UMLS CUI [1,2]
    C1276802
    Fluctuating or gradual progression of cognitive deficits
    Description

    progression of cognitive deficits

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1854645
    UMLS CUI [1,2]
    C0338656
    UMLS CUI [2,1]
    C0338656
    UMLS CUI [2,2]
    C0231241
    Supporting Symptoms: Early occuring gait disturbance
    Description

    gait disturbance

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1856566
    UMLS CUI [1,2]
    C0596473
    Motoric instability and frequent support
    Description

    motoric instability and frequent support

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0260026
    UMLS CUI [2]
    C2197383
    Bladder problem (frequent urge to urinate, not urological explainable)
    Description

    bladder problem

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0579152
    Pseudobulbar paralysis
    Description

    pseudobulbar paralysis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1390905
    Personality disorder or mood alteration
    Description

    personality disorder or mood alteration

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0031212
    UMLS CUI [2]
    C2939186
    Abulia
    Description

    abulia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0919974
    Depression
    Description

    depression

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0011581
    Emotional incontinence
    Description

    emotional incontinence

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1142234
    Other subcortical deficits
    Description

    other subcortical deficits

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C2987487
    UMLS CUI [1,2]
    C0815275
    UMLS CUI [1,3]
    C0205394
    Frontotemporal dementia, behavioral variant
    Description

    Frontotemporal dementia, behavioral variant

    Alias
    UMLS CUI-1
    C4011788
    Is the patient currently diagnosed with bvFTD according to the criteria of Raskovsky et. al. 2011?
    Description

    bvFTD diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C4011788
    I. Signs of neurodegenerative disease: Slowly progressing disturbance of behavior or of cognition in order to distinguish it from acute beginning or stable disturbance for a long time, which is typical for psychiatric disease
    Description

    slowly progressing disturbance of behavior or of cognition

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0149623
    UMLS CUI [2]
    C0338656
    II. "Possible" bvFTD Three of the symptoms listed in A to F have to be fulfilled. It should be ensured that these symptoms are prominent and persistent and are not rare or even nonrecurring clinical pictures. A. Early onset of behavior disturbance with the symptom "disinhibition" A.1.Socially inappropriate behavior
    Description

    socially inappropriate behavior

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2220010
    A.2. Loss of manners and decency
    Description

    loss of manners and decency

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0564122
    A.3. Impulsive, unreflected or reckless behavior
    Description

    impulsive or reckless behavior

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0021125
    UMLS CUI [2]
    C0683304
    B. Early onset of symptoms of apathy or inactivity B.1. Apathy
    Description

    apathy

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0085632
    B.2. Inactivity
    Description

    inactivity

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3890554
    C. Early loss of sympathy or empathy (one symptom has to be present) C.1. Reduced sympathy for needs and feelings or other human beings
    Description

    reduced sympathy

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0683287
    UMLS CUI [1,2]
    C0392756
    C.2. Reduced interest in social contacts or human affection
    Description

    reduced interest in social contacts or human affection

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0237170
    UMLS CUI [2,1]
    C0424091
    UMLS CUI [2,2]
    C0562561
    D. Early onset of perservative, stereotypic or compulsive, ritualized behaviour patterns (one of the symptoms has to be present) D.1. Simple repetitive movements
    Description

    repetitive movements

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3806732
    D.2. Complex, compulsive executed or ritualized behaviour patterns
    Description

    compulsive or ritualized behaviour patterns

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0600104
    UMLS CUI [2]
    C2266670
    D.3. Stereotypes in language
    Description

    stereotypes in language

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0038271
    UMLS CUI [1,2]
    C0037817
    E. Hyperorality or changes in eating behavior (one of the symptoms has to be present) E.1. Disturbed preference in choice of food
    Description

    disturbed preference in choice of food

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C2699787
    UMLS CUI [1,2]
    C0016483
    E.2. Eating orgy, increased consumption of alcohol or cigarettes
    Description

    eating orgy or increased consumption of alcohol or cigarettes

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1843777
    UMLS CUI [2]
    C2169567
    UMLS CUI [3,1]
    C0037369
    UMLS CUI [3,2]
    C0205217
    E.3. Oral exposure behavior or even oral consumption of uneatable objects
    Description

    hyperorality

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1838320
    F. Typical neuropsychological profile: Disturbance of executive functions along with relatively intact memory and intact visuoconstructive skills (all symptoms must be present) F.1. Disturbance in executive functions, including complex cognitive functions like working memory, planing, generalization of rules and abstracting of rules, problem solution and mental flexibility
    Description

    disturbance in executive functions

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2678119
    F.2. Relatively intact performance of episodic memory
    Description

    performance of episodic memory

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0561843
    F.3. Relatively intact performance testing visuocontructive functions
    Description

    visuocontructive functions

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3852968
    III. "Probable" bvFTD All of the following criteria(A-C) have to be fulfilled for a diagnosis of a probable bvFTD A. Diagnosis criteria of a probable FTD have to be fulfilled
    Description

    FTD diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0338451
    UMLS CUI [1,2]
    C0332148
    B. The amount of disturbance causes disturbance in everyday functioning
    Description

    everyday functioning

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0562507
    C. Imaging is compatible with the behavioral variant of FTD (one of the two attributes has to be present) C.1. CT or MRI imaging show a frontal and/or anterior temporal atrophy
    Description

    anterior temporal atrophy

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3281302
    UMLS CUI [2]
    C0011923
    C.2. PET or SPECT examinations showing frontal and/or temporal hypo-perfusion/hypo-metabolism
    Description

    frontal or temporal hypo-perfusion

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0442856
    UMLS CUI [2]
    C0347938
    UMLS CUI [3]
    C1699633
    UMLS CUI [4]
    C0183126
    IV. bvFTD with ensured FTD-pathology (criterion A and one additional criterion (B or C) have to be fulfilled) A. Criteria of possible or probable FTD fulfilled
    Description

    FTD criteria fulfilled

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0338451
    B. Histopathological evidence for the disease (by biopsy or post mortem)
    Description

    histopathological evidence

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0428093
    C. Presence of a known mutation which causes the behavioural variant of FTD
    Description

    mutation causing the behavioural variant of FTD

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0596611
    UMLS CUI [1,2]
    C0678227
    UMLS CUI [1,3]
    C4011788
    V. Exclusion criteria for bvFTD A. The pattern of disturbance can be better explained by another (neurodegenerative) disease
    Description

    disease pattern better explained by another disease

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0449782
    UMLS CUI [1,2]
    C0678226
    UMLS CUI [1,3]
    C0524851
    UMLS CUI [1,4]
    C0205394
    B. The behavioral disturbances can be better explained by a psychiatric disease
    Description

    behavioral disturbances explained by a psychiatric disease

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0233514
    UMLS CUI [1,2]
    C0678226
    UMLS CUI [1,3]
    C0004936
    C. Biomarkers emphasize Alzheimer's disease or another neurodegenarative disease
    Description

    biomarkers emphasize Alzheimers disease or another neurodegenarative disease

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0005516
    UMLS CUI [2,1]
    C0002395
    UMLS CUI [2,2]
    C0524851
    UMLS CUI [2,3]
    C0205394
    Primary progressive aphasia (PPA)
    Description

    Primary progressive aphasia (PPA)

    Alias
    UMLS CUI-1
    C0282513
    Is the patient currently diagnosed with PPA according to the criteria of Mesulam et. al. 2001?
    Description

    PPA diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0282513
    1. Gradually starting and slowly progressing impairment in word formation, object naming, syntax or word comprehension in conversation or applying a well-established neuropsychologic speech tests.
    Description

    impairment of speech characteristics

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0233715
    UMLS CUI [2]
    C0566058
    UMLS CUI [3]
    C0027902
    UMLS CUI [4]
    C0513762
    2. All restrictions in everyday life in the first two years after onset of symptoms can be attributed to impairment of speech.
    Description

    restrictions in everylife due to speech impairment

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0562507
    UMLS CUI [1,2]
    C0439801
    UMLS CUI [1,3]
    C0678226
    UMLS CUI [1,4]
    C0233715
    3. The premobid function of speech is intact (a dyslexia during development is possible).
    Description

    intact premobid function of speech

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0037817
    UMLS CUI [1,2]
    C0871122
    UMLS CUI [1,3]
    C0205266
    4. A prominent apathy, disinhibition, disturbance of short-term memory, visuo-spatial disturbances, disability of visual recognition and sensomotoric deficits are missing in the first two years of disease in consideration of anamnesis, everyday activities or neuropsychologic results, so that the participant fulfills no diagnostic criteria of another dementia.
    Description

    symtoms missing in first two years

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0085632
    UMLS CUI [1,2]
    C0332197
    UMLS CUI [2,1]
    C0234410
    UMLS CUI [2,2]
    C0332197
    UMLS CUI [3,1]
    C2363813
    UMLS CUI [3,2]
    C0332197
    UMLS CUI [4,1]
    C2676705
    UMLS CUI [4,2]
    C0332197
    UMLS CUI [5,1]
    C0030709
    UMLS CUI [5,2]
    C0332197
    UMLS CUI [6,1]
    C0221163
    UMLS CUI [6,2]
    C0332197
    5. Dyscalculia, ideomotoric apraxia and problems tracing can be present in the first two years of disease. However these are not developed so that everyday activities are influenced.
    Description

    dyscalculia, ideomotoric apraxia and problems tracing

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0869474
    UMLS CUI [2]
    C0234523
    UMLS CUI [3]
    C0565772
    6. Other cognitive functions can be affected after the first two years after disease, but speech remains the strongest impaired function during the whole course of disease and declines faster than every other function.
    Description

    other cognitive functions

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0392335
    UMLS CUI [1,2]
    C0205394
    UMLS CUI [2,1]
    C0037817
    UMLS CUI [2,2]
    C0242656
    UMLS CUI [2,3]
    C1838681
    7. Other reasons for aphasia like stroke or tumor are excluded by imaging.
    Description

    other reasons for aphasia excluded

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C2828389
    UMLS CUI [1,2]
    C0015127
    UMLS CUI [1,3]
    C0003537
    Progressive nonfluent aphasia (PNFA)
    Description

    Progressive nonfluent aphasia (PNFA)

    Alias
    UMLS CUI-1
    C0751706
    Is the patient currently diagnosed with PNFA according to the criteria of Gorno-Tempini et. al. 2011?
    Description

    PNFA diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0751706
    I. Clinical diagnosis of PNFA At least one of the diagnostic main criteria has to be fulfilled: 1. Speech with agrammatism
    Description

    agrammatism

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0242186
    2. Labored, haltingly speech with inconsistent prosody and speech attitudes (speech apraxia)
    Description

    speech apraxia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0264611
    At least two of the following features have to be fulfilled: 1. Speech intelligibility disturbances formulating sentences with complex syntax
    Description

    speech intelligibility disturbances

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0037824
    UMLS CUI [1,2]
    C0221099
    2. Maintained speech intelligibility of single words
    Description

    speech intelligibility

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0037824
    3. Maintained object knowledge
    Description

    object knowledge

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0599621
    II. Imaging results supporting diagnosis of PNFA Both criteria have to be fulfilled 1. Clinical diagnosis of PNFA
    Description

    clinical diagnosis of PNFA

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0332140
    UMLS CUI [1,2]
    C0751706
    2. Imaging diagnostics have to show one of the following findings: 2a. MRI: Emphasized atrophy in posterior part of frontal lobe/island region of left hemisphere
    Description

    atrophy in posterior part of frontal lobe in MRI

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0016733
    UMLS CUI [1,2]
    C0205095
    UMLS CUI [2,1]
    C0228176
    UMLS CUI [2,2]
    C3496404
    UMLS CUI [3]
    C0024485
    UMLS CUI [4]
    C0333641
    2b. SPECT/PET: Emphasized hypoperfusion and hypometabolism in posterior part of frontal lobe/insular region of left hemisphere
    Description

    hypoperfusion and hypometabolism in posterior part of frontal lobe

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0442856
    UMLS CUI [2]
    C0347938
    UMLS CUI [3]
    C1699633
    UMLS CUI [4]
    C0183126
    UMLS CUI [5,1]
    C0016733
    UMLS CUI [5,2]
    C0205095
    UMLS CUI [6,1]
    C0228176
    UMLS CUI [6,2]
    C3496404
    III. Diagnostic criteria for PNFA and clear histopathologic finding The clinical diagnosis (criterion 1) an criterion 2 or criterion 3 have to be fulfilled: 1. Clinical diagnosis of PNFA
    Description

    clinical diagnosis PNFA

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0332140
    UMLS CUI [1,2]
    C0751706
    2. Histopathological evidence of a specific neurodegenerative pathology (e.g. FTD-tau, FTD-TDP, AD or another specific pathology)
    Description

    neurodegenarative pathology

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0524851
    UMLS CUI [1,2]
    C0243140
    3. Evidence of a common mutation
    Description

    mutation

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0026882
    Logopenic progressive aphasia (LPA)
    Description

    Logopenic progressive aphasia (LPA)

    Alias
    UMLS CUI-1
    C0282513
    UMLS CUI-2
    C0037825
    Is the patient currently diagnosed with LPA according to the criteria of Gorno-Tempini et. al. 2011 and Raskovsky et. al. 2011?
    Description

    diagnosis of LPA

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0282513
    UMLS CUI [1,2]
    C0037825
    I. Clinical diagnosis of LPA: Both diagnostic main criteria have to be fulfilled: 1. Disturbed ability to fetch words in sponteneous speech and in naming
    Description

    disturbed sponteneous speech

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0855217
    2. Repeating of sentences and sentence elements disturbed
    Description

    disturbed repetition of sentences

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0233741
    UMLS CUI [1,2]
    C0221099
    UMLS CUI [2,1]
    C0233740
    UMLS CUI [2,2]
    C0221099
    At least 3 of the following characteristics have to be fulfilled: 1. Phonologic paraphrasia in spontaneous speech and naming
    Description

    phonologic paraphrasia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0233722
    UMLS CUI [2]
    C0454640
    2. Maintained speech intelligibility of single words and object knowledge
    Description

    speech intelligibility and object knowledge

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0037824
    UMLS CUI [2]
    C0599621
    3. Maintained motor speech (no speech apraxia)
    Description

    motor speech

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C4035655
    4. No distinctive agrammatism
    Description

    no distinctive agrammatism

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0242186
    UMLS CUI [1,2]
    C0332197
    I. Diagnosis of LPA supported by imaging Both of the following criteria have to be fulfilled 1. Clinical diagnosis of LPA
    Description

    clinical diagnosis of LPA

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0332140
    UMLS CUI [1,2]
    C0282513
    UMLS CUI [1,3]
    C0037825
    2. Imaging diagnostics have to show one or several of the following findings: 2a. MRI: Emphasized atrophy in posterior sections of perisylvian area or parietal atrophy
    Description

    atrophy in posterior sections of perisylvian area or parietal atrophy

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0333641
    UMLS CUI [1,2]
    C3495412
    UMLS CUI [2]
    C4023041
    UMLS CUI [3]
    C0024485
    2b. SPECT/PET: Emphasized hypoperfusion and hypometabolism in a section of perisylvian area or parietal lobe
    Description

    hypoperfusion and hypometabolism in a section of perisylvian area or parietal lobe

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0442856
    UMLS CUI [2]
    C0347938
    UMLS CUI [3]
    C1699633
    II. Diagnostic criteria for LPA and clear histopathologic finding The clinical diagnosis (criterion 1) and criterion 2 or criterion 3 have to be fulfilled: 1. Clinical diagnosis of LPA
    Description

    clinical diagnosis LPA

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0332140
    UMLS CUI [1,2]
    C0282513
    UMLS CUI [1,3]
    C0037825
    2. Histopathological evidence of a specific neurodegenerative pathology (e.g. FTD-tau, FTD-TDP, AD or another specific pathology)
    Description

    histopathological evidence of a specific neurodegenerative pathology

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0524851
    UMLS CUI [1,2]
    C0243140
    3. Evidence of a common mutation
    Description

    evidence of mutation

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0026882
    Semantic dementia (SemD)
    Description

    Semantic dementia (SemD)

    Alias
    UMLS CUI-1
    C0338462
    Is the patient currently diagnosed with SemD according to the criteria of Gorno-Tempini et. al. 2011?
    Description

    diagnosis of SemD

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0338462
    I. Clinical diagnosis of SemD: Both diagnostic main criteria have to be fulfilled: 1. Disturbed behavior (anomia)
    Description

    anomia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0003113
    2. Disturbed speech intelligibility of single words
    Description

    disturbed speech intelligibility

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0037824
    UMLS CUI [1,2]
    C0221099
    At least three of the following characteristics have to be fulfilled: 1. Disturbed object knowledge, especially of words which are rarely used or have a lower familiarity
    Description

    disturbed object knowledge

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0599621
    UMLS CUI [1,2]
    C0221099
    2. Dyslexia or agraphia
    Description

    dyslexia or agraphia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0476254
    UMLS CUI [2]
    C0001825
    3. Undisturbed repetition
    Description

    undisturbed repetition

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0589243
    4. Maintained fluent production of speech (grammatically correct and without apraxic disorders)
    Description

    fluent production of speech

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0564240
    II. Imaging results supporting diagnosis of SemD: Both of the following criteria have to be fulfilled 1. Clinical diagnosis of SemD
    Description

    clinical diagnosis of SemD

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0332140
    UMLS CUI [1,2]
    C0338462
    2. Imaging diagnostics have to show one of the following findings: 2a. MRI: Emphasized atrophy in anterior part of frontal lobe
    Description

    atrophy in anterior part of frontal lobe

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0333641
    UMLS CUI [1,2]
    C0016733
    UMLS CUI [1,3]
    C0205094
    UMLS CUI [2]
    C0024485
    2b. SPECT/PET: Emphasized hypoperfusion and hypometabolism in anterior part of frontal lope
    Description

    hypoperfusion and hypometabolism in anterior part of frontal lope

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0442856
    UMLS CUI [2]
    C0347938
    UMLS CUI [3]
    C1699633
    UMLS CUI [4,1]
    C0016733
    UMLS CUI [4,2]
    C0205094
    III. Diagnostic criteria for SemD and clear histopathologic finding 1. Clinical diagnosis of SemD
    Description

    clinical diagnosis SemD

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0332140
    UMLS CUI [1,2]
    C0338462
    2. Histopathological evidence of a specific neurodegenerative pathology (e.g. FTD-tau, FTD-TDP, AD or another specific pathology)
    Description

    neurodegenarative pathology

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0524851
    UMLS CUI [1,2]
    C0243140
    3. Evidence of a common mutation
    Description

    mutation

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0026882
    Idiopathic Parkinson's disease (IPS)
    Description

    Idiopathic Parkinson's disease (IPS)

    Alias
    UMLS CUI-1
    C0030567
    Is the patient currently diagnosed with IPS according to the UKBB criteria (Litvan et. al. 2003)?
    Description

    IPS diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0030567
    Hypo-bradykinesia
    Description

    At least two of the following criteria are fulfilled, one of these attributes have to be hypo-bradykinesia:

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3176307
    Resting tremor
    Description

    resting tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0234379
    Rigor
    Description

    rigor

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0026837
    Postural instability
    Description

    postural instability

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1843921
    Unilateral onset
    Description

    Supportive symptoms (at least 3 'Yes' answers)

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0332162
    UMLS CUI [1,2]
    C0205092
    Resting tremor manifest
    Description

    resting tremor manifest

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0234379
    UMLS CUI [1,2]
    C0205319
    Progressive course of disease
    Description

    progressive course of disease

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0242656
    UMLS CUI [1,2]
    C0205329
    Laterality
    Description

    laterality

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0332304
    Excellent response to L-Dopa (70-100%)
    Description

    response to L-Dopa

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3280274
    L-Dopa induced chorea
    Description

    L-Dopa induced chorea

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0008489
    UMLS CUI [1,2]
    C0678226
    UMLS CUI [1,3]
    C0023570
    Response to L-Dopa over 5 years
    Description

    response to L-Dopa over 5 years

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3280274
    Response to L-Dopa over 10 years
    Description

    response to L-Dopa over 10 years

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3280274
    Medical history of brain damage (e.g. stroke, craniocerebral trauma)
    Description

    Exclusion criteria (No symptom is allowed to be fulfilled)

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0262926
    UMLS CUI [1,2]
    C0270611
    Medical history of encephalitis
    Description

    medical history of encephalitis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0014038
    Oculogyric crisis
    Description

    oculogyric crisis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0085637
    Therapy using neuroleptic drugs at onset of disease
    Description

    neuroleptic drugs at onset of disease

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0040615
    UMLS CUI [1,2]
    C0277793
    Remission of disease
    Description

    remission of disease

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0544452
    Unilateral motor impairment after 3 years of disease
    Description

    unilateral motor impairment

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0221163
    UMLS CUI [1,2]
    C0205092
    Supranuclear palsy
    Description

    supranuclear palsy

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1720037
    Cerebellar signs
    Description

    cerebellar signs

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0742038
    Severe autonomous dysfunction (e.g. urinary incontinence, cardiovascular system problem)
    Description

    severe autonomous dysfunction

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1397846
    UMLS CUI [1,2]
    C0205082
    Early development of dementia (within first year of disease) with special impairment of memory, speech and in practice
    Description

    early development of dementia

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1833334
    UMLS CUI [1,2]
    C0497327
    Babinski sign
    Description

    babinski sign

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0034935
    Poor response to L-Dopa
    Description

    poor response to L-Dopa

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2751071
    1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MTPT) exposition
    Description

    MTPT exposition

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0046312
    Morbus parkinson along with MCI (PD-MCI)
    Description

    Morbus parkinson along with MCI (PD-MCI)

    Alias
    UMLS CUI-1
    C0030567
    UMLS CUI-3
    C1270972
    Is the patient currently diagnosed with PD-MCI according to the criteria Litvan et. al. 2012?
    Description

    PD-MCI diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0030567
    UMLS CUI [2]
    C1270972
    Diagnosis of Parkinson disease according to UKPD BB criteria
    Description

    Main criteria of PD-MCI

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0030567
    UMLS CUI [1,2]
    C0679228
    Insidious onset and progressive course of disease of cognitive dysfunction under PD disease reported by participant, relatives and treating physician
    Description

    insidious onset and progressive course of disease of cognitive dysfunction

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0338656
    UMLS CUI [1,2]
    C1298634
    UMLS CUI [1,3]
    C0338656
    UMLS CUI [1,4]
    C0242656
    UMLS CUI [1,5]
    C0205329
    Loss of power (<=1.5 times standard deviation below the mean value of reference group) in (A) two assessments of a domain or (B) in each assessment of two different domains. Following domains can be affected: -attention & working memory -executive functions -visuo-constructive/visuospatial performance -memory -speech
    Description

    loss of power

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0548863
    Cognitive deficits do not interfere with lost of independence regarding everyday activities, although mild impairment may occur performing complex tasks
    Description

    cognitive deficits do not interfere everyday activities

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0009241
    UMLS CUI [1,2]
    C0521103
    UMLS CUI [1,3]
    C0001288
    Parkinson disease with dementia according to criteria of Movement Disorder Society Task Force
    Description

    Exclusion criteria of PD-MCI

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1828079
    Diseases that can explain cognitive impairment primarily (e.g. delir, stroke, traumatic brain injury, metabolic disorder, side effects of medication, severe depressive episode)
    Description

    diseases that can explain cognitive impairment

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0009241
    UMLS CUI [1,2]
    C0678226
    UMLS CUI [1,3]
    C0009488
    Other PD associated comorbidities like severe motor impairment, high anxiety, depression,increasing daytime sleepiness, psychosis, which have a significant influence on test performance according to a clinician's opinion.
    Description

    comorbidities influence on test performance

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0009488
    UMLS CUI [1,2]
    C0521102
    UMLS CUI [1,3]
    C0679605
    Dementia in Parkinsons disease
    Description

    Dementia in Parkinsons disease

    Alias
    UMLS CUI-1
    C1828079
    Is the patient currently diagnosed with PDD according to the criteria Emre et. al. 2007?
    Description

    PDD diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1828079
    I. Main criteria of PDD Diagnosis of Parkonson disease according to UKPD BB criteria
    Description

    diagnostic criteria for PD

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0030567
    UMLS CUI [1,2]
    C0679228
    Dementia syndrome with insidious onset and progressive course of disease with respect to Parkinson's disease (onset of dementia > 1 year after diagnosis of Parkinson's disease
    Description

    course of disease of dementia

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0497327
    UMLS CUI [1,2]
    C1298634
    UMLS CUI [2]
    C1828079
    Loss of power (<=1.5 times standard deviation below mean of reference group/normal population) affecting more than one cognitive domain and represent an impairment in contrast to the premobid level of performance
    Description

    loss of power

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0548863
    Cognitive deficits interfer with loss of independence in everyday activities (concerning social, occupational or personal aspects of life). These limitations of activities in everyday living should be primarily caused by cognitive factors and not by motor impairment or an autonomous functional disorder.
    Description

    interference with everyday living activities

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0009241
    UMLS CUI [1,2]
    C0521102
    UMLS CUI [1,3]
    C0001288
    II. Additional criteria a) Following domains can be affected: -attention & working memory -executive functions -visuo-constructive/visuospatial performance -memory -speech
    Description

    affected domains

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0004268
    UMLS CUI [2]
    C0025265
    UMLS CUI [3,1]
    C0935584
    UMLS CUI [3,2]
    C2370887
    UMLS CUI [4]
    C0025260
    UMLS CUI [5]
    C0037817
    b) The following behavior changes can occur: -apathy (reduced spontaneity, loss of motivation, interest and performance) -change of personality like depressive or anxious symptomatic -hallucinations (at most visual, complex visions of humans, animals or objects) -delusions (at most of paranoid nature like cheating of the partner or foreign persons living in the household) -increased sleepiness at daytime or doziness at daytime
    Description

    behavior changes

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0542299
    III. Findings, which make PDD diagnosis less probable Diseases that can also cause cognitive impairment as a result (e.g. vascular distinctive feature in MRI), but cannot be considered primary cause of dementia
    Description

    disease also causing cognitive impairment

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0009488
    UMLS CUI [1,2]
    C0678227
    UMLS CUI [1,3]
    C0009241
    Time interval between onset of cognitive and motor symptoms unknown
    Description

    time interval between onset of cognitive and motor symptoms unknown

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0872291
    UMLS CUI [1,2]
    C0332162
    UMLS CUI [1,3]
    C0426980
    UMLS CUI [1,4]
    C0392335
    IV. Exclusion criteria of PDD Cognitive symptomes or behavioral disorders occor exclusively as part of another disease or concomitants, like e.g. delir caused by a systemic disease or as consequence of a certain medication or a severe depressive episode
    Description

    symptoms exclusive due to comorbidity

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0011206
    UMLS CUI [2]
    C0442893
    UMLS CUI [3]
    C2364092
    Prevalence of a probable vascular dementia according to NINDS-AIREN-criteria
    Description

    probable vascular dementia

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0011269
    UMLS CUI [1,2]
    C0033204
    Probable PDD -All criteria in section I have to be fulfilled -Typical profile of cognitive impairment along with distinctive features in more than one cognitive domain (IIa), especially deficits of attention, a dysexecutive syndrome, an impaired visuospatial perception, limitations regarding direct recall of memory performance with improvement after provided assistance in recall. -presence of at least one behavior disorder (IIb) -no criteria mentioned in section III and IV must be present
    Description

    probable PDD

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1828079
    UMLS CUI [1,2]
    C0033204
    Possible PDD -atypical profile of cognitive impairment with abnormalities in more than one cognitive domain (IIa) like fluent aphasia or memory impairment or attention deficit -behavior disorders (IIb) can be present but do not have to be present -one or more criteria in III may be present -no criterion mentioned in section IV must be present
    Description

    possible PDD

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1828079
    UMLS CUI [1,2]
    C0332149
    Dementia with Lewy bodies (DLB)
    Description

    Dementia with Lewy bodies (DLB)

    Alias
    UMLS CUI-1
    C0752347
    Is the patient currently diagnosed with DLB according to the criteria Mc Keith et. al. 2005?
    Description

    DLB diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0752347
    Central Criterion 1. Dementia, defined as progressive loss, which causes loss of independence in everyday living activities (concerning social, occupational or personal life aspects)
    Description

    A prominent and persistent memory impairment has to be present in earlier stage of disease, but mostly occurs at disease progression. Deficits are particularly proven by tests of attention, executive function and visuospatial perception.

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0497327
    UMLS CUI [2,1]
    C0085862
    UMLS CUI [2,2]
    C0001288
    UMLS CUI [2,3]
    C1517945
    Main criteria 2. Fluctuations in cognition, especially attention performance
    Description

    fluctuation in cognition

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0853574
    UMLS CUI [1,2]
    C0009240
    3. Recurrent visual hallucinations, that typically consists of figures and are detailed.
    Description

    recurrent visual hallucinations

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0233763
    UMLS CUI [1,2]
    C2945760
    Spontaneous Parkinson symptomatic
    Description

    spontaneous Parkinson symptomatic

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0030569
    UMLS CUI [1,2]
    C0205359
    Additional criteria 5. REM sleep behavior disorder
    Description

    REM sleep behavior disorder

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0751772
    6. Severe side effects due to neuroleptic medication
    Description

    severe side effects due to neuroleptic medication

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0041755
    UMLS CUI [1,2]
    C0205082
    UMLS CUI [1,3]
    C0040615
    Reduced dopamine transporter ligand storage in basal ganglia (SPECT/PET)
    Description

    dopamine transporter ligand storage in basal ganglia

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0114838
    UMLS CUI [1,2]
    C0392756
    UMLS CUI [1,3]
    C0004781
    UMLS CUI [2]
    C3828509
    8. Dementia occurred before or at the same time (resp. within first year) symptoms of Parkinson's disease did?
    Description

    Course of symptoms

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0683368
    UMLS CUI [1,2]
    C0497327
    UMLS CUI [1,3]
    C3843404
    Probable DLB (item 1 and at least one feature in 2-4 and at least one feature 5-7 applies, item 8 is fulfilled)
    Description

    probable DLB

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0752347
    UMLS CUI [1,2]
    C0332148
    Possible DLB (item 1 and at least one feature in 5-7 apply; item 8 is fulfilled)
    Description

    possible DLB

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0752347
    UMLS CUI [1,2]
    C0332149
    Supportive Symptoms without diagnostic specifity Frequent falls or syncopes
    Description

    frequent falls or syncopes

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0850703
    UMLS CUI [2]
    C0039070
    Transient loss of consciousness
    Description

    transient loss of consciousness

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0041657
    Severe autonomous dysfunction
    Description

    severe autonomous functional disorder

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1397846
    UMLS CUI [1,2]
    C0205082
    Hallucinations in other modalities
    Description

    hallucinations in other modalities

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0018524
    UMLS CUI [1,2]
    C0695347
    UMLS CUI [1,3]
    C0205394
    Delusions
    Description

    delusion

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0011253
    Depression
    Description

    depression

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0011581
    Medial temporal lobe slightly changed in MRI
    Description

    medial temporal lobe

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0039485
    UMLS CUI [1,2]
    C0205098
    UMLS CUI [1,3]
    C0871641
    UMLS CUI [1,4]
    C0750482
    UMLS CUI [2]
    C0024485
    Light take in SPECT/PET perfusion scan at reduced occipital activity
    Description

    SPECT/PET perfusion scan at reduced occipital activity

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2094594
    UMLS CUI [2,1]
    C0443158
    UMLS CUI [2,2]
    C1184145
    Abnormally light take in MIBG myocardial scintiscan
    Description

    abnormally light take in MIBG myocardial scintiscan

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1704258
    UMLS CUI [1,2]
    C2229074
    Prominent slow-wave-activity in EEG with temporal transient shape waves
    Description

    slow-wave-activity in EEG with temporal transient shape waves

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2206519
    UMLS CUI [2]
    C2678116
    Findings making diagnosis less probable medical history of brain injury (e.g. stroke, traumatic brain injury)
    Description

    medical history of brain injury

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0262926
    UMLS CUI [1,2]
    C0270611
    Presence of another medical or neurological condition explaining deficits
    Description

    presence of another medical or neurological condition explaining deficits

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0009488
    UMLS CUI [1,2]
    C0678226
    UMLS CUI [1,3]
    C2987487
    Symptoms of Parkinson's disease occur in a severe stadium of dementia for the first time
    Description

    first occurrence of symptoms of Parkinsons disease

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0030567
    UMLS CUI [1,2]
    C0205435
    UMLS CUI [2,1]
    C0497327
    UMLS CUI [2,2]
    C0699749
    UMLS CUI [2,3]
    C0205082
    Progressive supranuclear palsy
    Description

    Progressive supranuclear palsy

    Alias
    UMLS CUI-1
    C0038868
    Is the patient currently diagnosed with PSP according to the criteria NINDS-PSP-criteria (Litvan et. al. 1996)?
    Description

    PSP diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0038868
    Conditions Gradual progressive disease with onset of disease at age of at least 40
    Description

    gradual progressive disease

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1335499
    UMLS CUI [1,2]
    C0439833
    UMLS CUI [2]
    C0206132
    Main criteria 1. Vertical palsy (up or down)
    Description

    vertical gaze palsy

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0339652
    2. Slowing of vertical saccades
    Description

    slowing of vertical saccades

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C4011795
    3. Postural instability with falls < 1 year after onset of disease
    Description

    postural instability with falls

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1843921
    UMLS CUI [2]
    C0085639
    Probable PSP (item 1 and item 3 have to be answered with 'Yes'
    Description

    probable PSP

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0038868
    UMLS CUI [1,2]
    C0033204
    Possible PSP (item 1 or 2 und item 3 have to be answered with 'Yes')
    Description

    possible PSP

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0038868
    UMLS CUI [1,2]
    C0332149
    Supportive symptoms Symmetric akinesia or rigor, proximal more than distal
    Description

    symmetric akinesia or rigor,

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0085623
    UMLS CUI [1,2]
    C0332516
    UMLS CUI [2]
    C0026837
    Abnormal head posture especially retrocollis
    Description

    abnormal head posture especially retrocollis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1536116
    UMLS CUI [2]
    C3887667
    Poor or low response to L-Dopa
    Description

    poor response to L-Dopa

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2751071
    Earlier dysphagia or dysarthria
    Description

    dysphagia or dysarthria

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0011168
    UMLS CUI [2]
    C0013362
    Earlier onset of a cognitive impairment along with presence at least two of the following symptoms: Apathy, impairment of abstract thinking, reduced verbal fluency, tool use behavior, imitation and frontal lobe syndrome
    Description

    symptoms of cognitive impairment

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0085632
    UMLS CUI [2]
    C0233642
    UMLS CUI [3]
    C0751514
    UMLS CUI [4]
    C1720915
    UMLS CUI [5]
    C3826143
    UMLS CUI [6]
    C0549117
    Exclusion criteria Medical history of brain injury (e.g. encephalitis)
    Description

    medical history of brain injury

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0262926
    UMLS CUI [1,2]
    C0270611
    Presence of another medical or neurological condition explaining deficits
    Description

    presence of another medical or neurological condition explaining deficits

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0009488
    UMLS CUI [1,2]
    C0678226
    UMLS CUI [1,3]
    C2987487
    Presence of alien limb syndrome
    Description

    presence of alien limb syndrome

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3160753
    Presence of a cortical perception disorder
    Description

    cortical perception disorder

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0030975
    Evidence of focal frontal or temporal-parietal atrophy
    Description

    focal frontal or temporal-parietal atrophy

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2674464
    Occurrence of hallucinations and delusions independently of a dopaminergic therapy
    Description

    hallucinations and delusions

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0018524
    UMLS CUI [2]
    C0011253
    Prominent early signs of cerebellar diseases
    Description

    cerebellar diseases

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0007760
    Evidence of severe autonomous functional disorder with unknown cause
    Description

    severe autonomous functional disorder

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0277785
    UMLS CUI [1,2]
    C0205082
    Severe asymmetric signs of Parkinson's syndrome
    Description

    severe asymmetric Parkinson syndrome

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0030567
    UMLS CUI [1,2]
    C0332514
    UMLS CUI [1,3]
    C0205082
    Neuroradiological evidence of relevant structural abnormalities
    Description

    evidence of relevant structural abnormalities

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1866933
    Evidence of Morbus Whipple
    Description

    Morbus Whipple

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0023788
    Corticobasal degeneration (CBD)
    Description

    Corticobasal degeneration (CBD)

    Alias
    UMLS CUI-1
    C0393570
    Is the patient currently diagnosed with CBD according to the criteria of Boeve et. al. 1996?
    Description

    CBD diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0393570
    Main criteria 1. Insidious onset and progressive course of disease
    Description

    insidious onset and progressive course of disease

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0814125
    UMLS CUI [2,1]
    C0242656
    UMLS CUI [2,2]
    C0205329
    2. No other medical or neurological disease can explain the deficits
    Description

    no other medical or neurological disease can explain the deficits

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0009488
    UMLS CUI [1,2]
    C0678226
    UMLS CUI [1,3]
    C2987487
    3. Cortical dysfunction with at least one of the following symptoms: 3a. Focal or asymmetric ideomotor apraxia
    Description

    ideomotor apraxia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0234523
    3b. Alien limb syndrome
    Description

    alien limb syndrome

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3160753
    3c. Cortical sensibility loss
    Description

    cortical sensibility loss

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0030975
    3d. Visual or sensorial hemineglect
    Description

    visual or sensorial hemineglect

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0751418
    3e. Constructional apraxia
    Description

    constructional apraxia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2243023
    3e. Focal or asymmetric myoclonus
    Description

    myoclonus

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0027066
    3g. Speech apraxia/ non-fluent aphasia
    Description

    speech apraxia or non-fluent aphasia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0264611
    UMLS CUI [2]
    C0349390
    4. Extrapyramidal dysfunction with at least one of the following symptomes: 4a. Focal or asymmetrical rigor of extremities without clear reponse to L-Dopa
    Description

    rigor of extremities without clear reponse to L-Dopa

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0026837
    UMLS CUI [1,2]
    C0278454
    UMLS CUI [2]
    C3806728
    4b. Focal or asymmetrical dystonia of extremity muscles
    Description

    focal or asymmetrical dystonia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0743332
    UMLS CUI [2]
    C0332514
    Diagnosis of CBD If item 1,2 and at least one item in 3a-3g and at least one in item 4a, item 4b is fulfilled
    Description

    diagnosis of CBD

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0393570
    UMLS CUI [1,2]
    C0679228
    Supportive symptoms Neuropsychological testing: Evidenz of focal or lateralised cognitive impairment with relatively maintained learning and memory function
    Description

    neuropsychological showing cognitive impairment

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0027902
    UMLS CUI [2]
    C0338656
    UMLS CUI [3]
    C0233832
    UMLS CUI [4]
    C0025260
    Evidence in MRI/ CT of focal or asymmetric atrophy, typically manifesting in frontoparietal cortex
    Description

    atrophy showing in MRI or CT

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2674464
    UMLS CUI [2]
    C0011923
    Evidence in SPECT or PET of focal or asymmetrical hypoperfusion, typically manifested in frontoparietal cortex at maximum, with or without involvement of basal ganglia resp. the thalamus
    Description

    focal or asymmetrical hypoperfusion showing in SPECT or PET

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1695782
    UMLS CUI [2]
    C0183126
    UMLS CUI [3]
    C0032743
    Multiple system atrophy (MSA)
    Description

    Multiple system atrophy (MSA)

    Alias
    UMLS CUI-1
    C0393571
    Is the patient currently diagnosed with MSA according to the criteria of Gilman et. al. 2008?
    Description

    MSA diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0393571
    Conditions Episodic, progressive disease with onset of symptoms after age of 30
    Description

    episodic progressive disease

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1335499
    UMLS CUI [1,2]
    C1455761
    UMLS CUI [2]
    C0150907
    Conditions Episodic, progressive disease with onset of symptoms after age of 30
    Description

    episodic progressive disease

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1335499
    UMLS CUI [1,2]
    C1455761
    UMLS CUI [2]
    C0150907
    1. Parkinson's disease (bradykinesia with rigor, tremor or postural instability)
    Description

    Signs of a possible multiple system atrophy

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0030567
    Or a cerebellar syndrome (gait ataxia with cerebellar dysarthria, limb ataxia or cerebellar oculomotor disturbance)
    Description

    cerebellar syndrome

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0231691
    UMLS CUI [2]
    C0454599
    UMLS CUI [3]
    C0231691
    UMLS CUI [4]
    C1411869
    2. At least one sign suggesting autonomous dysfunction: -bladder incontinence that cannot be explained by another disease -incomplete urination -erectile dysfunction -significant decrease of orthostatic blood pressure without fulfilling criteria of a probable MSA
    Description

    signs of autonomous dysfunction

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0042024
    UMLS CUI [2]
    C0426373
    UMLS CUI [3]
    C0242350
    UMLS CUI [4]
    C1095974
    3. At least one additional sign have to be fulfilled
    Description

    additional sign

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0679228
    Possible MSA (items 1-3 apply)
    Description

    possible MSA

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0393571
    UMLS CUI [1,2]
    C0332149
    Signs of a probable multiple system atrophy 4. Autonomous disorder -with bladder incontinence (inability to control urination along with erectile dysfunction among men) -orthostatic decrease of blood pressure standing for 3 minutes of >30mmHg systolic or >15mmHg
    Description

    autonomous dysfunction

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0042024
    UMLS CUI [2]
    C0242350
    UMLS CUI [3]
    C1095974
    5. Parkinson's syndrome (bradykinesia with rigor, tremor or postural instability) with poor response to Levodopa
    Description

    parkinsons syndrome

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0030567
    UMLS CUI [2]
    C2751071
    Or a cerebellar syndrome (gait ataxia with cerebellar dysarthria, limb ataxia or cerebellar oculomotor disturbance)
    Description

    cerebellar syndrome

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0231691
    UMLS CUI [2]
    C0454599
    UMLS CUI [3]
    C0231691
    UMLS CUI [4]
    C1411869
    Probable MSA (item 4-5 apply)
    Description

    probable MSA

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0393571
    UMLS CUI [1,2]
    C0033204
    additional signs of MSA-P or MSA-C
    Description

    Additional signs for a possible multiple system atrophy of Parkinson's type (MSA-P) or of cerebellar type (MSA-C)

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2732311
    UMLS CUI [2]
    C2733447
    Babinski sign with increased reflex
    Description

    Possible MSA-P or MSA-C

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0034935
    UMLS CUI [2]
    C0151889
    Stridor
    Description

    stridor

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0038450
    Rapid progression of Parkinson's syndrome
    Description

    Possible MSA-P

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1834700
    Poor response to L-Dopa
    Description

    poor response to L-Dopa

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2751071
    Postural instability within three years after first motor symptomes
    Description

    postural instability

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1843921
    Gait ataxia, cerebellar dysarthria, ataxia of extremities or cerebellar occulomotor disorder
    Description

    cerebellar disorders

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0751837
    UMLS CUI [2]
    C0454599
    UMLS CUI [3]
    C4015302
    UMLS CUI [4]
    C1411869
    Dysphagia within 5 years after onset of motor disease
    Description

    dysphagia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0011168
    Atrophy of putamen, cerebellar peduncle, pons or cerebellum proven in MRI
    Description

    atrophy of putamen, cerebellar peduncle, pons or cerebellum

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1860217
    UMLS CUI [2,1]
    C0228515
    UMLS CUI [2,2]
    C0333641
    UMLS CUI [3]
    C3552972
    UMLS CUI [4]
    C0740279
    Hypometabolism in putamen, brain stem or cerebellum proven in FDG-PET
    Description

    hypometabolism

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0347938
    UMLS CUI [2]
    C3641247
    Parkinson's syndrome (bradykinesia and rigor)
    Description

    Possible MSA-C

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0030567
    UMLS CUI [2]
    C0233565
    UMLS CUI [3]
    C0026837
    Putamen atrophy, middling cerebellar peduncle atrophy or pons atrophy proven in MRI
    Description

    atrophies

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1860217
    UMLS CUI [2,1]
    C0228515
    UMLS CUI [2,2]
    C0333641
    UMLS CUI [3]
    C3552972
    Presynaptic striatonigral dopaminergic denervation
    Description

    presynaptic striatonigral dopaminergic denervation

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0011307
    UMLS CUI [1,2]
    C0206129
    UMLS CUI [1,3]
    C3498773
    UMLS CUI [1,4]
    C0034798
    Orofacial dystonia
    Description

    Supportive symptoms

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1395979
    Unproportional antecollis
    Description

    antecollis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1698476
    Camptocormia
    Description

    camptocormia

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0264162
    Contractions of hands and feet
    Description

    contractions of hands and feet

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1140999
    UMLS CUI [1,2]
    C0018563
    UMLS CUI [2,1]
    C1140999
    UMLS CUI [2,2]
    C0016504
    Inspiratory sighs
    Description

    inspiratory sighs

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0425481
    Severe dysphonia
    Description

    severe dysphonia

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1527344
    UMLS CUI [1,2]
    C0205082
    Severe dysarthria
    Description

    severe dysarthria

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0013362
    UMLS CUI [1,2]
    C0205082
    Incipient snoring
    Description

    incipient snoring

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0037384
    UMLS CUI [1,2]
    C0205256
    Cold hand or feet
    Description

    cold hand or feet

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0857073
    Pathological laughter or crying
    Description

    pathological laughter or crying

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0424304
    UMLS CUI [2]
    C0860609
    Jerky, myoclonic resting or action tremor
    Description

    resting or action tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0234379
    UMLS CUI [2]
    C0234376
    Classic pill rolling tremor
    Description

    Findings making the diagnosis more probable

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0751564
    Clinically significant neuropathy
    Description

    clinically significant neuropathy

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0442874
    UMLS CUI [1,2]
    C2985739
    Hallucinations which are not induced by medication
    Description

    hallucinations

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0018524
    Onset of disease after age of 75
    Description

    age at onset of disease

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0150907
    Positive family history of ataxia or parkinsonism
    Description

    family history of ataxia or parkinsonism

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C2939151
    UMLS CUI [2,1]
    C0004134
    UMLS CUI [2,2]
    C0241889
    Dementia diagnosis
    Description

    dementia diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0497327
    Lesions of white substance in MRI hinting for diagnosis of multiple sclerosis
    Description

    lesions of white substance in MRI hinting for diagnosis of multiple sclerosis

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0682708
    UMLS CUI [1,2]
    C0221198
    UMLS CUI [2]
    C0026769
    Essential tremor (ET)
    Description

    Essential tremor (ET)

    Alias
    UMLS CUI-1
    C0270736
    Is the patient currently diagnosed with ET according to the criteria of Deutschi et. al. 1998?
    Description

    ET diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0270736
    Known family genesis of tremor?
    Description

    family genesis of tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0241889
    UMLS CUI [1,2]
    C0270736
    I. Signs of a definite essential tremor 1. Bilateral , at most symmetric resting or action tremor, concerning hands and forearms, that is persistent and visible (Persistent tremor in other body regions can occur in addition; bilateral tremor can have different severity grades; tremor is described by participant as permanent continuous, but amplitude of the tremor can vary; tremor can cause disabilities in everyday living, but do not have to.
    Description

    bilateral permanent resting or action tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0234379
    UMLS CUI [1,2]
    C0238767
    UMLS CUI [1,3]
    C0205355
    UMLS CUI [2,1]
    C0234376
    UMLS CUI [2,2]
    C0238767
    UMLS CUI [2,3]
    C0205355
    2. Duration of illness > 5 years
    Description

    duration of illness

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3176590
    II. Exclusion criteria of definite essential tremor 3. Presence of other abnormal neurological symptoms
    Description

    other abnormal neurological symptoms

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1533281
    UMLS CUI [1,2]
    C0205394
    4. Presence of indications or known reasons for occurrence of physiologic tremor
    Description

    indications of tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0040822
    UMLS CUI [1,2]
    C0392360
    5. Current or earlier intake of psychogenic substances that can cause tremor or the current withdrawal symptoms
    Description

    intake of psychotics

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0458006
    UMLS CUI [1,2]
    C0013227
    UMLS CUI [1,3]
    C0678227
    UMLS CUI [1,4]
    C0040822
    6. Other medical or neurological diseases, that can explain the tremor (especially brain injury, peripheral injury or trauma, 3 months before onset of tremor.
    Description

    other disease causing tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0009488
    UMLS CUI [1,2]
    C0678227
    UMLS CUI [1,3]
    C0040822
    7. Anamnestic indication or clinical evidence for psychosomatic tremor
    Description

    evidence for psychosomatic tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0040822
    UMLS CUI [1,2]
    C1537048
    8. Indication for acute onset or stepped course of disease
    Description

    indication for acute onset

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0242656
    UMLS CUI [1,2]
    C1276802
    III. Signs of probable essential tremor III1. Bilateral, mainly symmetric resting or action tremor, which concerns hands and forearms but other body parts as well like head or legs, visible and persistent (bilateral tremor can be asymmetric; tremor is described by participant as permanent continuous, but amplitude of the tremor can vary; tremor can cause disabilities in everyday living, but do not have to)
    Description

    signs of probable essential tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0234379
    UMLS CUI [1,2]
    C0238767
    UMLS CUI [1,3]
    C0205355
    UMLS CUI [2,1]
    C0234376
    UMLS CUI [2,2]
    C0238767
    UMLS CUI [2,3]
    C0205355
    III2. Duration of disease > 3 years
    Description

    duration of disease

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C3176590
    III Exclusion criteria for possible essential tremor III.3 At least one exclusion criterion mentioned in I3-I8 is true.
    Description

    diagnostic exclusion criteria

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0679228
    III4. Presence of primary orthostatic tremor
    Description

    primary orthostatic tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0878578
    III5. Presence of isolated tremor of the voice
    Description

    isolated tremor of the voice

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0234808
    UMLS CUI [1,2]
    C0205409
    III6. Presence of isolated tremor specific to position and tasks, including action-specific tremor or a primary writing tremor
    Description

    tremor specific to position and tasks

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0040822
    UMLS CUI [1,2]
    C1262869
    UMLS CUI [1,3]
    C0205409
    III7. Presence of isolated tongue or chin tremor
    Description

    isolated tongue or chin tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0241446
    UMLS CUI [1,2]
    C0205409
    UMLS CUI [2,1]
    C3809829
    UMLS CUI [2,2]
    C0205409
    III. Signs of possible tremor Type I: Persons fulfilling criteria of definite or probable essential tremor but suffering from another neurological disease like parkinsonism, a dystonia, a peripheral neuropathology or restless leg syndrome. Persons fulfilling criteria of definite or probable essential tremor but suffering from other neurological symptoms that are not sufficient enough to make the diagnosis of another neurological illness. These symptoms concern for example minimal extra pyramidal signs like hypomimia, reduced arm drive or mild bradykinesia.
    Description

    type I essential tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0270736
    Mono-symptomatic and isolated tremor with uncertain relation to essential tremor, like for example isolated tremor specific to position and task, including action-specific tremor or a primary writing tremor, a primary orthostatic tremor, an isolated voice tremor, an isolated leg tremor or a unilateral resting tremor of the hand.
    Description

    type II essential tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1865810
    III. Exclusion criteria for possible essential tremor At least one exclusion criterion mentioning in I4-I6 is true
    Description

    exclusion criteria possible essential tremor

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0679228
    UMLS CUI [1,2]
    C0270736
    UMLS CUI [1,3]
    C0332149
    Amyotrophic lateral sclerosis (ALS)
    Description

    Amyotrophic lateral sclerosis (ALS)

    Alias
    UMLS CUI-1
    C0002736
    Is the patient currently diagnosed with ALS according to the criteria of El-Escorial (revised 1998)?
    Description

    ALS diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0002736
    Symptoms that have to be present: Signs of lesion of 1. motor neuron
    Description

    lesion of first motor neuron

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0235834
    Signs of lesion of 2. motor neuron (including EMG changes in clinically not affected muscles)
    Description

    lesion of second motor neuron

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0221198
    UMLS CUI [1,2]
    C0524459
    Progression
    Description

    progression

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0449258
    Symptoms, excluding a diagnosis of ALS emotional disorder
    Description

    emotional disorder

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0233459
    sphincter disturbance
    Description

    sphincter disturbance

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C1409894
    UMLS CUI [1,2]
    C2699787
    Vision disorder
    Description

    vision disorders

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0042790
    Autonomous dysfunctions (e.g. urinary incontinence, cerebrovascular circulation)
    Description

    autonomous dysfunctions

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1397846
    Alzheimer's disease
    Description

    alzheimers disease

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0002395
    Syndromes similar to ALS
    Description

    syndromes similar to ALS

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0039082
    UMLS CUI [1,2]
    C2348205
    UMLS CUI [1,3]
    C0002736
    Supportive symptoms Fasciculations in one or several region
    Description

    fasciculations

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0015644
    Neurogenic change in EMG
    Description

    neurogenic change in EMG

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C1843506
    Normal motor and sensory nerve conduction study
    Description

    nerve conduction study

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0200125
    Missing nerve conduction block
    Description

    missing nerve conduction block

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C4012631
    UMLS CUI [1,2]
    C1705492
    Definite ALS Signs of damage of 1. and 2. motor neuron in 3 of 4 regions (bulbar, cervical, thoracic, lumbosacral)
    Description

    damage of 1. and 2. motor neuron

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0026609
    UMLS CUI [1,2]
    C1883709
    Probable ALS Signs of damage of 1. and 2. motor neuron in 2 of 4 regions, where the damage signs of 2. motor neuron has to be in position rostral of the damage of the 2. motor neuron
    Description

    signs of probable ALS

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0026609
    UMLS CUI [1,2]
    C1883709
    Probable, laboratory supported symptoms Signs of damage of 1. and 2. motor neuron in 1 of 4 regions (or only one region of 1. motor neuron) and signs of degeneration in EMG in at least two extremities
    Description

    damage of 1. and 2. motor neuron in 1 of 4 regions

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0026609
    UMLS CUI [1,2]
    C1883709
    Possible ALS Signs of damage of 1. and 2. motor neuron in one of 4 regions
    Description

    signs of possible ALS

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0026609
    UMLS CUI [1,2]
    C1883709
    Depression
    Description

    Depression

    Alias
    UMLS CUI-1
    C0011581
    Is the patient currently diagnosed with an episode of major depression according to the criteria of DSM-IV?
    Description

    depression diagnosis

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0011581
    Key syndrome: Episode of major depression Five (or more) of the following symptoms have been present during the same 2-week period and represent a change from previous functioning; at least one of the symptoms is either (1) depressed mood or (2) loss of interest or pleasure. 1 depressed mood most of the day, nearly every day, as indicated by either subjective report (e.g., feels sad or empty) or observation made by others (e.g., appears tearful). 2 markedly diminished interest or pleasure in all, or almost all, activities most of the day, nearly every day (as indicated by either subjective account or observation made by others) 3 significant weight loss when not dieting or weight gain (e.g., a change of more than 5% of body weight in a month), or decrease or increase in appetite nearly every day. 4 insomnia or hypersomnia nearly every day 5 psychomotor agitation or retardation nearly every day (observable by others, not merely subjective feelings of restlessness or being slowed down) 6 fatigue or loss of energy nearly every day 7 feelings of worthlessness or excessive or inappropriate guilt (which may be delusional) nearly every day (not merely self-reproach or guilt about being sick) 8 diminished ability to think or concentrate, or indecisiveness, nearly every day (either by subjective account or as observed by others) 9 recurrent thoughts of death (not just fear of dying), recurrent suicidal ideation without a specific plan, or a suicide attempt or a specific plan for committing suicide
    Description

    symptoms of episode of major depression

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0024517
    B The symptoms do not meet criteria for a Mixed Episode
    Description

    no symptoms of mixed episode

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C2874862
    UMLS CUI [1,2]
    C0332197
    C. The symptoms cause clinically significant distress or impairment in social, occupational, or other important areas of functioning
    Description

    clinically significant distress

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0700361
    UMLS CUI [2]
    C0679477
    D. The symptoms are not due to the direct physiological effects of a substance (e.g., a drug of abuse, a medication) or a general medical condition (e.g., hypothyroidism).
    Description

    symtoms not caused by substances or a general medical condition

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0728866
    UMLS CUI [2]
    C0236747
    E. The symptoms are not better accounted for by bereavement, i.e., after the loss of a loved one, the symptoms persist for longer than 2 months or are characterized by marked functional impairment, morbid preoc-cupation with worthlessness, suicidal ideation, psychotic symptoms, or psychomotor retardation.
    Description

    symptoms are not better accounted for by bereavement

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0005119
    UMLS CUI [1,2]
    C0683368
    A. Presence of a single Major Depressive Episode
    Description

    Major depression, single episode

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0024517
    B. The Major Depressive Episode is not better accounted for by Schizoaffective Disorder and is not superimposed on Schizophrenia, Schizophreniform Disorder, Delusional Disorder, or Psychotic disorder not otherwise specified.
    Description

    major depressive episode is not better accounted for by schizoaffective disorder

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0036337
    UMLS CUI [1,2]
    C0683368
    C. There has never been a Manic Episode, a Mixed Episode, or a Hypomanic Episode.
    Description

    never manic mixed or hypomanic episode

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0349208
    UMLS CUI [1,2]
    C2003901
    UMLS CUI [2,1]
    C1608433
    UMLS CUI [2,2]
    C2003901
    UMLS CUI [3,1]
    C1396834
    UMLS CUI [3,2]
    C2003901
    Major depression, recurrent A. Presence of two or more Major Depressive Episodes. (To be considered separate episodes, there must be an interval of at least 2 consecutive months in which criteria are not met for a Major Depressive Episode)
    Description

    two or more major depressive episodes

    Type de données

    boolean

    Alias
    UMLS CUI [1]
    C0154409
    B. The Major Depressive Episodes are not better accounted for by Schizoaffective Disorder and are not superimposed on Schizophrenia, Schizophreniform Disorder, Delusional Disorder, or Psychotic Disorder Not Otherwise Specified.
    Description

    episodes are not better accounted for by schizoaffective disorder

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0036337
    UMLS CUI [1,2]
    C0686906
    There has never been a Manic Episode, a Mixed Episode, or a Hypomanic Episode. (This exclusion does not apply if all of the manic-like, mixed-like, or hypomanic-like episodes are substance or treatment induced or are due to the direct physiological effects of a general medical condition.)
    Description

    no earlier manic mixed or hypomanic episode

    Type de données

    boolean

    Alias
    UMLS CUI [1,1]
    C0349208
    UMLS CUI [1,2]
    C2003901
    UMLS CUI [2,1]
    C1608433
    UMLS CUI [2,2]
    C2003901
    UMLS CUI [3,1]
    C1396834
    UMLS CUI [3,2]
    C2003901

    Similar models

    CRF Diagnostic Criteria

    Name
    Type
    Description | Question | Decode (Coded Value)
    Type de données
    Alias
    Item Group
    Date of visit
    C1320303 (UMLS CUI-1)
    date of visit
    Item
    Date of visit
    date
    C1320303 (UMLS CUI [1])
    study id
    Item
    Study-ID:
    text
    C2826693 (UMLS CUI [1])
    visit name
    Item
    Visit:
    text
    C2826704 (UMLS CUI [1])
    Item Group
    Mild cognitive impairment (MCI)
    C1270972 (UMLS CUI-1)
    current MCI
    Item
    Is the patient currently diagnosed with a MCI according to Winblad & Petersen 2004?
    boolean
    C1270972 (UMLS CUI [1,1])
    C0150312 (UMLS CUI [1,2])
    cognitive degration
    Item
    Cognitive Impairment 1. Subjective deterioration of cognition observed by the patient or an informant and/or...
    boolean
    C1270972 (UMLS CUI [1,1])
    C0854193 (UMLS CUI [1,2])
    psychometric test proving cognitive degradation
    Item
    2. Evidence of degradation of cognition in time due to psychometric tests
    boolean
    C1270972 (UMLS CUI [1,1])
    C0033920 (UMLS CUI [1,2])
    everyday functioning
    Item
    3. Ability to perform activities of everyday living/ minimal impairment of complex instrumental everyday functions
    boolean
    C0562507 (UMLS CUI [1])
    signs of disturbed consciousness
    Item
    4. No signs of disturbed consciousness, of a delirium, of a dementia or of an amnesic aphasia that cannot be explained better by other psychotic illnesses (e.g. a disorder in connection with psychotropic substances or major depression).
    boolean
    C0234428 (UMLS CUI [1])
    C0011206 (UMLS CUI [2])
    C0497327 (UMLS CUI [3])
    wordlist
    Item
    Operationalization 1. Wordlist - delayed word retrieval
    boolean
    C0456660 (UMLS CUI [1])
    visuospatial ability
    Item
    2. Visuospatial ability - Drawing
    boolean
    C0814069 (UMLS CUI [1])
    C0565772 (UMLS CUI [2])
    visuospatial ability delayed
    Item
    3. Visuospatial ability - Delayed retrieval
    boolean
    C0814069 (UMLS CUI [1])
    trail making test
    Item
    4. Trial making test A or B
    boolean
    C0040604 (UMLS CUI [1])
    semantic or phonetic fluency
    Item
    5. Semantic or phonetic fluency
    boolean
    C0233714 (UMLS CUI [1])
    object naming
    Item
    6. Object naming
    boolean
    C0451342 (UMLS CUI [1])
    normal finding FAQ
    Item
    7. No abnormal finding FAQ
    boolean
    C3827007 (UMLS CUI [1,1])
    C0205307 (UMLS CUI [1,2])
    amnesic MCI single domain
    Item
    Classification a. Amnesic MCI single domain (only memory)
    boolean
    C1270972 (UMLS CUI [1])
    amnesic MCI multi domain
    Item
    b. Amnesic MCI multi domain (memory and one additional area)
    boolean
    C1270972 (UMLS CUI [1])
    non-amnesic MCI single domain
    Item
    c. Non-amnesic MCI single domain (one area, except memory)
    boolean
    C1270972 (UMLS CUI [1])
    non-amnesic MCI multi domain
    Item
    d. Non-amnesic MCI multi domain (several areas, except memory)
    boolean
    C1270972 (UMLS CUI [1])
    Item Group
    Dementia in Alzheimer's Disease (AD)
    C3665464 (UMLS CUI-1)
    NINCDS/ADRDA-Criteria AD diagnosis
    Item
    Is the patient currently diagnosed with AD according to NINCDS/ADRDA-Criteria (McKhann et al. 1984)?
    boolean
    C3665464 (UMLS CUI [1,1])
    C2828081 (UMLS CUI [1,2])
    dementia
    Item
    I. Necessary requirements Evidence of dementia in a clinical examination including neuropsychological tests
    boolean
    C0497327 (UMLS CUI [1])
    C0027902 (UMLS CUI [2])
    cognitive deficits
    Item
    Deficits in at least two cognitive areas
    boolean
    C0009241 (UMLS CUI [1])
    progressive disturbance of memory
    Item
    Progressive disturbance of memory and other cognitive functions
    boolean
    C1864985 (UMLS CUI [1,1])
    C0025260 (UMLS CUI [1,2])
    C1864985 (UMLS CUI [2,1])
    C0392335 (UMLS CUI [2,2])
    no consciousness disturbance
    Item
    No consciousness disturbance
    boolean
    C0424525 (UMLS CUI [1])
    age at onset
    Item
    Onset between age of 40 and 90, mostly 65 and older
    boolean
    C0150907 (UMLS CUI [1])
    other system- or brain diseases
    Item
    No evidence of other system- or brain diseases as cause
    boolean
    C0442893 (UMLS CUI [1])
    C0006111 (UMLS CUI [2])
    progressive deterioration of specific cognitive functions
    Item
    II. Subsidiary Findings Progressive deterioration of specific cognitive functions, e.g. of speech (aphasia), motor activity (apraxia) and perception (agnosia)
    boolean
    C0854193 (UMLS CUI [1,1])
    C0205329 (UMLS CUI [1,2])
    impairment of everyday activities and behavioral change
    Item
    Impairment of everyday activities and emerging of behavior modifications
    boolean
    C0562507 (UMLS CUI [1,1])
    C0221099 (UMLS CUI [1,2])
    C0542299 (UMLS CUI [2])
    family history of similar disorders
    Item
    Family history of similar disorders (especially if neurologically proven)
    boolean
    C0241889 (UMLS CUI [1])
    cerebral atrophy
    Item
    Results of additional examinations: Evidence of - progressively evolving in follow ups- cerebral atrophy due to imaging diagnostics
    boolean
    C0235946 (UMLS CUI [1])
    normal finding or unspecific changes in EEG
    Item
    Normal finding or unspecific changes in EEG
    boolean
    C0234550 (UMLS CUI [1,1])
    C0205307 (UMLS CUI [1,2])
    C0234550 (UMLS CUI [2,1])
    C0750540 (UMLS CUI [2,2])
    normal finding of cerebrospinal fluid
    Item
    Normal finding of cerebrospinal fluid
    boolean
    C0007806 (UMLS CUI [1,1])
    C0205307 (UMLS CUI [1,2])
    diagnosis of Alzheimers disease
    Item
    III. Matching with a probable diagnosis of Alzheimer's disease Temporal stagnation in course of disease
    boolean
    C1848980 (UMLS CUI [1,1])
    C0242656 (UMLS CUI [1,2])
    attendant symptoms
    Item
    Attendant symptoms like depression, insomnia, incontinence, illusion, hallucination, delusion, sudden aggressive outbreaks, sexual dysfunction and weight loss
    boolean
    C0011581 (UMLS CUI [1])
    C0917801 (UMLS CUI [2])
    C0021167 (UMLS CUI [3])
    C0020903 (UMLS CUI [4])
    C0018524 (UMLS CUI [5])
    C0011253 (UMLS CUI [6])
    C0001807 (UMLS CUI [7])
    C0549622 (UMLS CUI [8])
    C0936227 (UMLS CUI [9])
    neurological distinctive feature
    Item
    Neurological distinctive feature (particularly in case of advanced disease) like elevated muscle tonus, myoclonus or gait disturbance
    boolean
    C1704258 (UMLS CUI [1,1])
    C0205494 (UMLS CUI [1,2])
    C0026841 (UMLS CUI [2])
    C0027066 (UMLS CUI [3])
    C1856566 (UMLS CUI [4])
    epileptic seizure
    Item
    Epileptic seizure (in case of advanced disease)
    boolean
    C1411824 (UMLS CUI [1])
    CT commensurate with age
    Item
    CT commensurate with age
    boolean
    C0040405 (UMLS CUI [1,1])
    C0001783 (UMLS CUI [1,2])
    sudden apoplectic onset
    Item
    IV. Exclusion Criteria Sudden, apoplectic onset
    boolean
    C1388315 (UMLS CUI [1,1])
    C1276802 (UMLS CUI [1,2])
    focal neurological signs
    Item
    Focal neurological signs like hemiparesis, sensorial failures, visual field defects or problems with coordination in early stage of disease
    boolean
    C0018989 (UMLS CUI [1])
    C0152027 (UMLS CUI [2])
    C0004134 (UMLS CUI [3])
    C3887875 (UMLS CUI [4])
    epileptic seizures or gait disturbance
    Item
    Epileptic seizures or gait disturbance at onset or in early stage of disease
    boolean
    C1411824 (UMLS CUI [1])
    C1856566 (UMLS CUI [2])
    dementia syndrome with atypical symptomatic
    Item
    V. Clinical possible AD Dementia syndrome with atypical symptomatic with regard to onset, course and deficit profile, in absence of other neurological, psychiatric or internal disease that could cause the dementia syndrome
    boolean
    C0497327 (UMLS CUI [1,1])
    C0231220 (UMLS CUI [1,2])
    second systemic or brain disease
    Item
    Second systemic or brain disease that could cause dementia, but is commonly not regarded as a substantial cause
    boolean
    C0442893 (UMLS CUI [1])
    C0006111 (UMLS CUI [2])
    progressive severe cognitive deficit
    Item
    Progressive severe cognitive deficit without notable different cause
    boolean
    C0009241 (UMLS CUI [1,1])
    C0205329 (UMLS CUI [1,2])
    C0205082 (UMLS CUI [1,3])
    Item Group
    Posterior cortical atrophy (PCA)
    C0235946 (UMLS CUI-1)
    C0205095 (UMLS CUI-2)
    PCA diagnosis
    Item
    Is the patient currently diagnosed with PCA according to the criteria of Mendez et.al. 2002?
    boolean
    C0235946 (UMLS CUI [1,1])
    C0205095 (UMLS CUI [1,2])
    insidious onset and gradual progression
    Item
    Main symptoms (all symptoms have to be present) A. Insidious onset and gradual progression
    boolean
    C1298634 (UMLS CUI [1])
    C1854645 (UMLS CUI [2])
    visual disorders
    Item
    B. Presentation with visual disorders while visual function intact
    boolean
    C0042790 (UMLS CUI [1])
    prominent complex visual deficits
    Item
    C. Evidence of prominent complex visual deficits
    boolean
    C3665347 (UMLS CUI [1,1])
    C0205402 (UMLS CUI [1,2])
    C0439855 (UMLS CUI [1,3])
    Balint syndrome
    Item
    Elements of Balint syndrome
    boolean
    C4707368 (UMLS CUI [1])
    visual agnosia
    Item
    Visual agnosia
    boolean
    C0234502 (UMLS CUI [1])
    dressing apraxia
    Item
    Dressing apraxis
    boolean
    C0234529 (UMLS CUI [1])
    topographic disorientation
    Item
    Topographic disorientation
    boolean
    C0233407 (UMLS CUI [1,1])
    C1639223 (UMLS CUI [1,2])
    relatively small deficits of memory and word fluency
    Item
    D. Relatively small memory deficits and word fluency deficits
    boolean
    C0233794 (UMLS CUI [1,1])
    C1843272 (UMLS CUI [1,2])
    C2018029 (UMLS CUI [2,1])
    C1843272 (UMLS CUI [2,2])
    relatively good awareness of disability
    Item
    E. Relatively good awareness of disability with or without depression
    boolean
    C0424227 (UMLS CUI [1,1])
    C0205170 (UMLS CUI [1,2])
    presenile onset
    Item
    Subsidiary diagnosis A. Presenile onset
    boolean
    C0750901 (UMLS CUI [1])
    alexia
    Item
    B. Alexia
    boolean
    C0002018 (UMLS CUI [1])
    Gerstmann syndrome
    Item
    C. Elements of Gerstmann syndrome
    boolean
    C0017494 (UMLS CUI [1])
    ideomotor apraxia
    Item
    D. Ideomotor apraxia
    boolean
    C0234523 (UMLS CUI [1])
    physical examination
    Item
    E. Physical examination within the norms
    boolean
    C0031809 (UMLS CUI [1])
    impairment of perceptual functions
    Item
    F. Neuropsychiatric: Prominent impairment of perceptual functions
    boolean
    C0233746 (UMLS CUI [1])
    occipitoparietal abnormality
    Item
    Imaging: Prominent occipitoparietal abnormality (especially in functional imaging) with relative recess of frontal and mesiotemporal regions
    boolean
    C1704258 (UMLS CUI [1,1])
    C1184145 (UMLS CUI [1,2])
    C0442030 (UMLS CUI [1,3])
    Item Group
    Vascular dementia
    C0011269 (UMLS CUI-1)
    VD diagnoses
    Item
    Is the patient currently diagnosed with VD according to the criteria of NINDS/AIREN (Roman et al. 1993?)
    boolean
    C0011269 (UMLS CUI [1])
    cognitive impairment
    Item
    I. Dementia Cognitive impairment regarding previous higher functional status manifested by disturbance of memory
    boolean
    C0009241 (UMLS CUI [1])
    C0233794 (UMLS CUI [2])
    orientation
    Item
    Orientation
    boolean
    C0029266 (UMLS CUI [1])
    attention
    Item
    Attention
    boolean
    C0004268 (UMLS CUI [1])
    speech
    Item
    Speech
    boolean
    C0037817 (UMLS CUI [1])
    spatial visualization ability
    Item
    Spatial visualization ability
    boolean
    C0679024 (UMLS CUI [1])
    ability to judge
    Item
    Ability to judge
    boolean
    C0022423 (UMLS CUI [1])
    action ability
    Item
    Action ability
    boolean
    C1820715 (UMLS CUI [1])
    abstraction ability
    Item
    Abstraction ability
    boolean
    C0679195 (UMLS CUI [1])
    motor control
    Item
    Motor control
    boolean
    C0517961 (UMLS CUI [1])
    praxia
    Item
    Praxia
    boolean
    C0871470 (UMLS CUI [1,1])
    C0234621 (UMLS CUI [1,2])
    everyday activities
    Item
    Activities of daily living
    boolean
    C0001288 (UMLS CUI [1])
    disturbed consciousness
    Item
    Exclusion criteria Disturbed consciousness
    boolean
    C0549249 (UMLS CUI [1])
    delirium
    Item
    Delirium
    boolean
    C0011206 (UMLS CUI [1])
    psychosis
    Item
    Psychosis
    boolean
    C0033975 (UMLS CUI [1])
    severe aphasia
    Item
    Severe aphasia
    boolean
    C0003537 (UMLS CUI [1,1])
    C0205082 (UMLS CUI [1,2])
    sensomotoric disorder
    Item
    Prominent sensomotoric disorder, which makes testing impossible
    boolean
    C0036675 (UMLS CUI [1])
    systemic or other brain diseases
    Item
    Systemic or other brain diseases, that can cause cognitive disorders for their part
    boolean
    C2874347 (UMLS CUI [1])
    cerebrovascular disease
    Item
    II. Cerebrovascular disease Central focal neurological signs with or without anamnestic stroke and signs of relevant cerebrovascular disease in CT/MRT
    boolean
    C1392298 (UMLS CUI [1])
    C0038454 (UMLS CUI [2])
    C0007820 (UMLS CUI [3])
    bilateral artery cerebri anterior
    Item
    A. cerebri anterior, bilateral
    boolean
    C0149561 (UMLS CUI [1,1])
    C0238767 (UMLS CUI [1,2])
    anterior cerebral artery
    Item
    A. cerebri anterior
    boolean
    C0149561 (UMLS CUI [1])
    parietotemporal or temporoparietal association complex
    Item
    Parietotemporal or temporoparietal association complex
    boolean
    C3498498 (UMLS CUI [1])
    C3495412 (UMLS CUI [2])
    watershed infarction
    Item
    Superiore frontale and parietal watershed zone
    boolean
    C0333557 (UMLS CUI [1])
    basal ganglia and frontal lacuna of white matter
    Item
    Disease of small vessels: Basal ganglia and frontal lacuna of white matter
    boolean
    C0152295 (UMLS CUI [1,1])
    C1459585 (UMLS CUI [1,2])
    C0004781 (UMLS CUI [1,3])
    C0152295 (UMLS CUI [2,1])
    C1459585 (UMLS CUI [2,2])
    C0016733 (UMLS CUI [2,3])
    periventricular white matter lesions
    Item
    Broad periventricular white matter lesions
    boolean
    C2675929 (UMLS CUI [1,1])
    C0228157 (UMLS CUI [1,2])
    bilateral thalamus lesions
    Item
    Bilateral thalamus lesions
    boolean
    C2026261 (UMLS CUI [1,1])
    C0238767 (UMLS CUI [1,2])
    great vessel lesions
    Item
    Severity: great vessel lesions in dominant hemisphere
    boolean
    C0221198 (UMLS CUI [1,1])
    C3641002 (UMLS CUI [1,2])
    C0228465 (UMLS CUI [1,3])
    C1527180 (UMLS CUI [1,4])
    bilateral lesions of great vessel
    Item
    Bilateral lesions of great vessel in hemisphere
    boolean
    C0221198 (UMLS CUI [1,1])
    C3641002 (UMLS CUI [1,2])
    C0228465 (UMLS CUI [1,3])
    C0238767 (UMLS CUI [1,4])
    leukoencephalopathy
    Item
    Leukoencephalopathy => 25% of white matter
    boolean
    C0023524 (UMLS CUI [1])
    onset of dementia
    Item
    III. Combination of I. and II. Onset of dementia within three months after stroke
    boolean
    C0011269 (UMLS CUI [1,1])
    C0332162 (UMLS CUI [1,2])
    abrupt degeneration of cognitive functions
    Item
    Abrupt degeneration of cognitive functions
    boolean
    C0338656 (UMLS CUI [1,1])
    C1276802 (UMLS CUI [1,2])
    progression of cognitive deficits
    Item
    Fluctuating or gradual progression of cognitive deficits
    boolean
    C1854645 (UMLS CUI [1,1])
    C0338656 (UMLS CUI [1,2])
    C0338656 (UMLS CUI [2,1])
    C0231241 (UMLS CUI [2,2])
    gait disturbance
    Item
    Supporting Symptoms: Early occuring gait disturbance
    boolean
    C1856566 (UMLS CUI [1,1])
    C0596473 (UMLS CUI [1,2])
    motoric instability and frequent support
    Item
    Motoric instability and frequent support
    boolean
    C0260026 (UMLS CUI [1])
    C2197383 (UMLS CUI [2])
    bladder problem
    Item
    Bladder problem (frequent urge to urinate, not urological explainable)
    boolean
    C0579152 (UMLS CUI [1])
    pseudobulbar paralysis
    Item
    Pseudobulbar paralysis
    boolean
    C1390905 (UMLS CUI [1])
    personality disorder or mood alteration
    Item
    Personality disorder or mood alteration
    boolean
    C0031212 (UMLS CUI [1])
    C2939186 (UMLS CUI [2])
    abulia
    Item
    Abulia
    boolean
    C0919974 (UMLS CUI [1])
    depression
    Item
    Depression
    boolean
    C0011581 (UMLS CUI [1])
    emotional incontinence
    Item
    Emotional incontinence
    boolean
    C1142234 (UMLS CUI [1])
    other subcortical deficits
    Item
    Other subcortical deficits
    boolean
    C2987487 (UMLS CUI [1,1])
    C0815275 (UMLS CUI [1,2])
    C0205394 (UMLS CUI [1,3])
    Item Group
    Frontotemporal dementia, behavioral variant
    C4011788 (UMLS CUI-1)
    bvFTD diagnosis
    Item
    Is the patient currently diagnosed with bvFTD according to the criteria of Raskovsky et. al. 2011?
    boolean
    C4011788 (UMLS CUI [1])
    slowly progressing disturbance of behavior or of cognition
    Item
    I. Signs of neurodegenerative disease: Slowly progressing disturbance of behavior or of cognition in order to distinguish it from acute beginning or stable disturbance for a long time, which is typical for psychiatric disease
    boolean
    C0149623 (UMLS CUI [1])
    C0338656 (UMLS CUI [2])
    socially inappropriate behavior
    Item
    II. "Possible" bvFTD Three of the symptoms listed in A to F have to be fulfilled. It should be ensured that these symptoms are prominent and persistent and are not rare or even nonrecurring clinical pictures. A. Early onset of behavior disturbance with the symptom "disinhibition" A.1.Socially inappropriate behavior
    boolean
    C2220010 (UMLS CUI [1])
    loss of manners and decency
    Item
    A.2. Loss of manners and decency
    boolean
    C0564122 (UMLS CUI [1])
    impulsive or reckless behavior
    Item
    A.3. Impulsive, unreflected or reckless behavior
    boolean
    C0021125 (UMLS CUI [1])
    C0683304 (UMLS CUI [2])
    apathy
    Item
    B. Early onset of symptoms of apathy or inactivity B.1. Apathy
    boolean
    C0085632 (UMLS CUI [1])
    inactivity
    Item
    B.2. Inactivity
    boolean
    C3890554 (UMLS CUI [1])
    reduced sympathy
    Item
    C. Early loss of sympathy or empathy (one symptom has to be present) C.1. Reduced sympathy for needs and feelings or other human beings
    boolean
    C0683287 (UMLS CUI [1,1])
    C0392756 (UMLS CUI [1,2])
    reduced interest in social contacts or human affection
    Item
    C.2. Reduced interest in social contacts or human affection
    boolean
    C0237170 (UMLS CUI [1])
    C0424091 (UMLS CUI [2,1])
    C0562561 (UMLS CUI [2,2])
    repetitive movements
    Item
    D. Early onset of perservative, stereotypic or compulsive, ritualized behaviour patterns (one of the symptoms has to be present) D.1. Simple repetitive movements
    boolean
    C3806732 (UMLS CUI [1])
    compulsive or ritualized behaviour patterns
    Item
    D.2. Complex, compulsive executed or ritualized behaviour patterns
    boolean
    C0600104 (UMLS CUI [1])
    C2266670 (UMLS CUI [2])
    stereotypes in language
    Item
    D.3. Stereotypes in language
    boolean
    C0038271 (UMLS CUI [1,1])
    C0037817 (UMLS CUI [1,2])
    disturbed preference in choice of food
    Item
    E. Hyperorality or changes in eating behavior (one of the symptoms has to be present) E.1. Disturbed preference in choice of food
    boolean
    C2699787 (UMLS CUI [1,1])
    C0016483 (UMLS CUI [1,2])
    eating orgy or increased consumption of alcohol or cigarettes
    Item
    E.2. Eating orgy, increased consumption of alcohol or cigarettes
    boolean
    C1843777 (UMLS CUI [1])
    C2169567 (UMLS CUI [2])
    C0037369 (UMLS CUI [3,1])
    C0205217 (UMLS CUI [3,2])
    hyperorality
    Item
    E.3. Oral exposure behavior or even oral consumption of uneatable objects
    boolean
    C1838320 (UMLS CUI [1])
    disturbance in executive functions
    Item
    F. Typical neuropsychological profile: Disturbance of executive functions along with relatively intact memory and intact visuoconstructive skills (all symptoms must be present) F.1. Disturbance in executive functions, including complex cognitive functions like working memory, planing, generalization of rules and abstracting of rules, problem solution and mental flexibility
    boolean
    C2678119 (UMLS CUI [1])
    performance of episodic memory
    Item
    F.2. Relatively intact performance of episodic memory
    boolean
    C0561843 (UMLS CUI [1])
    visuocontructive functions
    Item
    F.3. Relatively intact performance testing visuocontructive functions
    boolean
    C3852968 (UMLS CUI [1])
    FTD diagnosis
    Item
    III. "Probable" bvFTD All of the following criteria(A-C) have to be fulfilled for a diagnosis of a probable bvFTD A. Diagnosis criteria of a probable FTD have to be fulfilled
    boolean
    C0338451 (UMLS CUI [1,1])
    C0332148 (UMLS CUI [1,2])
    everyday functioning
    Item
    B. The amount of disturbance causes disturbance in everyday functioning
    boolean
    C0562507 (UMLS CUI [1])
    anterior temporal atrophy
    Item
    C. Imaging is compatible with the behavioral variant of FTD (one of the two attributes has to be present) C.1. CT or MRI imaging show a frontal and/or anterior temporal atrophy
    boolean
    C3281302 (UMLS CUI [1])
    C0011923 (UMLS CUI [2])
    frontal or temporal hypo-perfusion
    Item
    C.2. PET or SPECT examinations showing frontal and/or temporal hypo-perfusion/hypo-metabolism
    boolean
    C0442856 (UMLS CUI [1])
    C0347938 (UMLS CUI [2])
    C1699633 (UMLS CUI [3])
    C0183126 (UMLS CUI [4])
    FTD criteria fulfilled
    Item
    IV. bvFTD with ensured FTD-pathology (criterion A and one additional criterion (B or C) have to be fulfilled) A. Criteria of possible or probable FTD fulfilled
    boolean
    C0338451 (UMLS CUI [1])
    histopathological evidence
    Item
    B. Histopathological evidence for the disease (by biopsy or post mortem)
    boolean
    C0428093 (UMLS CUI [1])
    mutation causing the behavioural variant of FTD
    Item
    C. Presence of a known mutation which causes the behavioural variant of FTD
    boolean
    C0596611 (UMLS CUI [1,1])
    C0678227 (UMLS CUI [1,2])
    C4011788 (UMLS CUI [1,3])
    disease pattern better explained by another disease
    Item
    V. Exclusion criteria for bvFTD A. The pattern of disturbance can be better explained by another (neurodegenerative) disease
    boolean
    C0449782 (UMLS CUI [1,1])
    C0678226 (UMLS CUI [1,2])
    C0524851 (UMLS CUI [1,3])
    C0205394 (UMLS CUI [1,4])
    behavioral disturbances explained by a psychiatric disease
    Item
    B. The behavioral disturbances can be better explained by a psychiatric disease
    boolean
    C0233514 (UMLS CUI [1,1])
    C0678226 (UMLS CUI [1,2])
    C0004936 (UMLS CUI [1,3])
    biomarkers emphasize Alzheimers disease or another neurodegenarative disease
    Item
    C. Biomarkers emphasize Alzheimer's disease or another neurodegenarative disease
    boolean
    C0005516 (UMLS CUI [1])
    C0002395 (UMLS CUI [2,1])
    C0524851 (UMLS CUI [2,2])
    C0205394 (UMLS CUI [2,3])
    Item Group
    Primary progressive aphasia (PPA)
    C0282513 (UMLS CUI-1)
    PPA diagnosis
    Item
    Is the patient currently diagnosed with PPA according to the criteria of Mesulam et. al. 2001?
    boolean
    C0282513 (UMLS CUI [1])
    impairment of speech characteristics
    Item
    1. Gradually starting and slowly progressing impairment in word formation, object naming, syntax or word comprehension in conversation or applying a well-established neuropsychologic speech tests.
    boolean
    C0233715 (UMLS CUI [1])
    C0566058 (UMLS CUI [2])
    C0027902 (UMLS CUI [3])
    C0513762 (UMLS CUI [4])
    restrictions in everylife due to speech impairment
    Item
    2. All restrictions in everyday life in the first two years after onset of symptoms can be attributed to impairment of speech.
    boolean
    C0562507 (UMLS CUI [1,1])
    C0439801 (UMLS CUI [1,2])
    C0678226 (UMLS CUI [1,3])
    C0233715 (UMLS CUI [1,4])
    intact premobid function of speech
    Item
    3. The premobid function of speech is intact (a dyslexia during development is possible).
    boolean
    C0037817 (UMLS CUI [1,1])
    C0871122 (UMLS CUI [1,2])
    C0205266 (UMLS CUI [1,3])
    symtoms missing in first two years
    Item
    4. A prominent apathy, disinhibition, disturbance of short-term memory, visuo-spatial disturbances, disability of visual recognition and sensomotoric deficits are missing in the first two years of disease in consideration of anamnesis, everyday activities or neuropsychologic results, so that the participant fulfills no diagnostic criteria of another dementia.
    boolean
    C0085632 (UMLS CUI [1,1])
    C0332197 (UMLS CUI [1,2])
    C0234410 (UMLS CUI [2,1])
    C0332197 (UMLS CUI [2,2])
    C2363813 (UMLS CUI [3,1])
    C0332197 (UMLS CUI [3,2])
    C2676705 (UMLS CUI [4,1])
    C0332197 (UMLS CUI [4,2])
    C0030709 (UMLS CUI [5,1])
    C0332197 (UMLS CUI [5,2])
    C0221163 (UMLS CUI [6,1])
    C0332197 (UMLS CUI [6,2])
    dyscalculia, ideomotoric apraxia and problems tracing
    Item
    5. Dyscalculia, ideomotoric apraxia and problems tracing can be present in the first two years of disease. However these are not developed so that everyday activities are influenced.
    boolean
    C0869474 (UMLS CUI [1])
    C0234523 (UMLS CUI [2])
    C0565772 (UMLS CUI [3])
    other cognitive functions
    Item
    6. Other cognitive functions can be affected after the first two years after disease, but speech remains the strongest impaired function during the whole course of disease and declines faster than every other function.
    boolean
    C0392335 (UMLS CUI [1,1])
    C0205394 (UMLS CUI [1,2])
    C0037817 (UMLS CUI [2,1])
    C0242656 (UMLS CUI [2,2])
    C1838681 (UMLS CUI [2,3])
    other reasons for aphasia excluded
    Item
    7. Other reasons for aphasia like stroke or tumor are excluded by imaging.
    boolean
    C2828389 (UMLS CUI [1,1])
    C0015127 (UMLS CUI [1,2])
    C0003537 (UMLS CUI [1,3])
    Item Group
    Progressive nonfluent aphasia (PNFA)
    C0751706 (UMLS CUI-1)
    PNFA diagnosis
    Item
    Is the patient currently diagnosed with PNFA according to the criteria of Gorno-Tempini et. al. 2011?
    boolean
    C0751706 (UMLS CUI [1])
    agrammatism
    Item
    I. Clinical diagnosis of PNFA At least one of the diagnostic main criteria has to be fulfilled: 1. Speech with agrammatism
    boolean
    C0242186 (UMLS CUI [1])
    speech apraxia
    Item
    2. Labored, haltingly speech with inconsistent prosody and speech attitudes (speech apraxia)
    boolean
    C0264611 (UMLS CUI [1])
    speech intelligibility disturbances
    Item
    At least two of the following features have to be fulfilled: 1. Speech intelligibility disturbances formulating sentences with complex syntax
    boolean
    C0037824 (UMLS CUI [1,1])
    C0221099 (UMLS CUI [1,2])
    speech intelligibility
    Item
    2. Maintained speech intelligibility of single words
    boolean
    C0037824 (UMLS CUI [1])
    object knowledge
    Item
    3. Maintained object knowledge
    boolean
    C0599621 (UMLS CUI [1])
    clinical diagnosis of PNFA
    Item
    II. Imaging results supporting diagnosis of PNFA Both criteria have to be fulfilled 1. Clinical diagnosis of PNFA
    boolean
    C0332140 (UMLS CUI [1,1])
    C0751706 (UMLS CUI [1,2])
    atrophy in posterior part of frontal lobe in MRI
    Item
    2. Imaging diagnostics have to show one of the following findings: 2a. MRI: Emphasized atrophy in posterior part of frontal lobe/island region of left hemisphere
    boolean
    C0016733 (UMLS CUI [1,1])
    C0205095 (UMLS CUI [1,2])
    C0228176 (UMLS CUI [2,1])
    C3496404 (UMLS CUI [2,2])
    C0024485 (UMLS CUI [3])
    C0333641 (UMLS CUI [4])
    hypoperfusion and hypometabolism in posterior part of frontal lobe
    Item
    2b. SPECT/PET: Emphasized hypoperfusion and hypometabolism in posterior part of frontal lobe/insular region of left hemisphere
    boolean
    C0442856 (UMLS CUI [1])
    C0347938 (UMLS CUI [2])
    C1699633 (UMLS CUI [3])
    C0183126 (UMLS CUI [4])
    C0016733 (UMLS CUI [5,1])
    C0205095 (UMLS CUI [5,2])
    C0228176 (UMLS CUI [6,1])
    C3496404 (UMLS CUI [6,2])
    clinical diagnosis PNFA
    Item
    III. Diagnostic criteria for PNFA and clear histopathologic finding The clinical diagnosis (criterion 1) an criterion 2 or criterion 3 have to be fulfilled: 1. Clinical diagnosis of PNFA
    boolean
    C0332140 (UMLS CUI [1,1])
    C0751706 (UMLS CUI [1,2])
    neurodegenarative pathology
    Item
    2. Histopathological evidence of a specific neurodegenerative pathology (e.g. FTD-tau, FTD-TDP, AD or another specific pathology)
    boolean
    C0524851 (UMLS CUI [1,1])
    C0243140 (UMLS CUI [1,2])
    mutation
    Item
    3. Evidence of a common mutation
    boolean
    C0026882 (UMLS CUI [1])
    Item Group
    Logopenic progressive aphasia (LPA)
    C0282513 (UMLS CUI-1)
    C0037825 (UMLS CUI-2)
    diagnosis of LPA
    Item
    Is the patient currently diagnosed with LPA according to the criteria of Gorno-Tempini et. al. 2011 and Raskovsky et. al. 2011?
    boolean
    C0282513 (UMLS CUI [1,1])
    C0037825 (UMLS CUI [1,2])
    disturbed sponteneous speech
    Item
    I. Clinical diagnosis of LPA: Both diagnostic main criteria have to be fulfilled: 1. Disturbed ability to fetch words in sponteneous speech and in naming
    boolean
    C0855217 (UMLS CUI [1])
    disturbed repetition of sentences
    Item
    2. Repeating of sentences and sentence elements disturbed
    boolean
    C0233741 (UMLS CUI [1,1])
    C0221099 (UMLS CUI [1,2])
    C0233740 (UMLS CUI [2,1])
    C0221099 (UMLS CUI [2,2])
    phonologic paraphrasia
    Item
    At least 3 of the following characteristics have to be fulfilled: 1. Phonologic paraphrasia in spontaneous speech and naming
    boolean
    C0233722 (UMLS CUI [1])
    C0454640 (UMLS CUI [2])
    speech intelligibility and object knowledge
    Item
    2. Maintained speech intelligibility of single words and object knowledge
    boolean
    C0037824 (UMLS CUI [1])
    C0599621 (UMLS CUI [2])
    motor speech
    Item
    3. Maintained motor speech (no speech apraxia)
    boolean
    C4035655 (UMLS CUI [1])
    no distinctive agrammatism
    Item
    4. No distinctive agrammatism
    boolean
    C0242186 (UMLS CUI [1,1])
    C0332197 (UMLS CUI [1,2])
    clinical diagnosis of LPA
    Item
    I. Diagnosis of LPA supported by imaging Both of the following criteria have to be fulfilled 1. Clinical diagnosis of LPA
    boolean
    C0332140 (UMLS CUI [1,1])
    C0282513 (UMLS CUI [1,2])
    C0037825 (UMLS CUI [1,3])
    atrophy in posterior sections of perisylvian area or parietal atrophy
    Item
    2. Imaging diagnostics have to show one or several of the following findings: 2a. MRI: Emphasized atrophy in posterior sections of perisylvian area or parietal atrophy
    boolean
    C0333641 (UMLS CUI [1,1])
    C3495412 (UMLS CUI [1,2])
    C4023041 (UMLS CUI [2])
    C0024485 (UMLS CUI [3])
    hypoperfusion and hypometabolism in a section of perisylvian area or parietal lobe
    Item
    2b. SPECT/PET: Emphasized hypoperfusion and hypometabolism in a section of perisylvian area or parietal lobe
    boolean
    C0442856 (UMLS CUI [1])
    C0347938 (UMLS CUI [2])
    C1699633 (UMLS CUI [3])
    clinical diagnosis LPA
    Item
    II. Diagnostic criteria for LPA and clear histopathologic finding The clinical diagnosis (criterion 1) and criterion 2 or criterion 3 have to be fulfilled: 1. Clinical diagnosis of LPA
    boolean
    C0332140 (UMLS CUI [1,1])
    C0282513 (UMLS CUI [1,2])
    C0037825 (UMLS CUI [1,3])
    histopathological evidence of a specific neurodegenerative pathology
    Item
    2. Histopathological evidence of a specific neurodegenerative pathology (e.g. FTD-tau, FTD-TDP, AD or another specific pathology)
    boolean
    C0524851 (UMLS CUI [1,1])
    C0243140 (UMLS CUI [1,2])
    evidence of mutation
    Item
    3. Evidence of a common mutation
    boolean
    C0026882 (UMLS CUI [1])
    Item Group
    Semantic dementia (SemD)
    C0338462 (UMLS CUI-1)
    diagnosis of SemD
    Item
    Is the patient currently diagnosed with SemD according to the criteria of Gorno-Tempini et. al. 2011?
    boolean
    C0338462 (UMLS CUI [1])
    anomia
    Item
    I. Clinical diagnosis of SemD: Both diagnostic main criteria have to be fulfilled: 1. Disturbed behavior (anomia)
    boolean
    C0003113 (UMLS CUI [1])
    disturbed speech intelligibility
    Item
    2. Disturbed speech intelligibility of single words
    boolean
    C0037824 (UMLS CUI [1,1])
    C0221099 (UMLS CUI [1,2])
    disturbed object knowledge
    Item
    At least three of the following characteristics have to be fulfilled: 1. Disturbed object knowledge, especially of words which are rarely used or have a lower familiarity
    boolean
    C0599621 (UMLS CUI [1,1])
    C0221099 (UMLS CUI [1,2])
    dyslexia or agraphia
    Item
    2. Dyslexia or agraphia
    boolean
    C0476254 (UMLS CUI [1])
    C0001825 (UMLS CUI [2])
    undisturbed repetition
    Item
    3. Undisturbed repetition
    boolean
    C0589243 (UMLS CUI [1])
    fluent production of speech
    Item
    4. Maintained fluent production of speech (grammatically correct and without apraxic disorders)
    boolean
    C0564240 (UMLS CUI [1])
    clinical diagnosis of SemD
    Item
    II. Imaging results supporting diagnosis of SemD: Both of the following criteria have to be fulfilled 1. Clinical diagnosis of SemD
    boolean
    C0332140 (UMLS CUI [1,1])
    C0338462 (UMLS CUI [1,2])
    atrophy in anterior part of frontal lobe
    Item
    2. Imaging diagnostics have to show one of the following findings: 2a. MRI: Emphasized atrophy in anterior part of frontal lobe
    boolean
    C0333641 (UMLS CUI [1,1])
    C0016733 (UMLS CUI [1,2])
    C0205094 (UMLS CUI [1,3])
    C0024485 (UMLS CUI [2])
    hypoperfusion and hypometabolism in anterior part of frontal lope
    Item
    2b. SPECT/PET: Emphasized hypoperfusion and hypometabolism in anterior part of frontal lope
    boolean
    C0442856 (UMLS CUI [1])
    C0347938 (UMLS CUI [2])
    C1699633 (UMLS CUI [3])
    C0016733 (UMLS CUI [4,1])
    C0205094 (UMLS CUI [4,2])
    clinical diagnosis SemD
    Item
    III. Diagnostic criteria for SemD and clear histopathologic finding 1. Clinical diagnosis of SemD
    boolean
    C0332140 (UMLS CUI [1,1])
    C0338462 (UMLS CUI [1,2])
    neurodegenarative pathology
    Item
    2. Histopathological evidence of a specific neurodegenerative pathology (e.g. FTD-tau, FTD-TDP, AD or another specific pathology)
    boolean
    C0524851 (UMLS CUI [1,1])
    C0243140 (UMLS CUI [1,2])
    mutation
    Item
    3. Evidence of a common mutation
    boolean
    C0026882 (UMLS CUI [1])
    Item Group
    Idiopathic Parkinson's disease (IPS)
    C0030567 (UMLS CUI-1)
    IPS diagnosis
    Item
    Is the patient currently diagnosed with IPS according to the UKBB criteria (Litvan et. al. 2003)?
    boolean
    C0030567 (UMLS CUI [1])
    hypo-bradykinesia
    Item
    Hypo-bradykinesia
    boolean
    C3176307 (UMLS CUI [1])
    resting tremor
    Item
    Resting tremor
    boolean
    C0234379 (UMLS CUI [1])
    rigor
    Item
    Rigor
    boolean
    C0026837 (UMLS CUI [1])
    postural instability
    Item
    Postural instability
    boolean
    C1843921 (UMLS CUI [1])
    unilateral onset
    Item
    Unilateral onset
    boolean
    C0332162 (UMLS CUI [1,1])
    C0205092 (UMLS CUI [1,2])
    resting tremor manifest
    Item
    Resting tremor manifest
    boolean
    C0234379 (UMLS CUI [1,1])
    C0205319 (UMLS CUI [1,2])
    progressive course of disease
    Item
    Progressive course of disease
    boolean
    C0242656 (UMLS CUI [1,1])
    C0205329 (UMLS CUI [1,2])
    laterality
    Item
    Laterality
    boolean
    C0332304 (UMLS CUI [1])
    response to L-Dopa
    Item
    Excellent response to L-Dopa (70-100%)
    boolean
    C3280274 (UMLS CUI [1])
    L-Dopa induced chorea
    Item
    L-Dopa induced chorea
    boolean
    C0008489 (UMLS CUI [1,1])
    C0678226 (UMLS CUI [1,2])
    C0023570 (UMLS CUI [1,3])
    response to L-Dopa over 5 years
    Item
    Response to L-Dopa over 5 years
    boolean
    C3280274 (UMLS CUI [1])
    response to L-Dopa over 10 years
    Item
    Response to L-Dopa over 10 years
    boolean
    C3280274 (UMLS CUI [1])
    medical history of brain damage
    Item
    Medical history of brain damage (e.g. stroke, craniocerebral trauma)
    boolean
    C0262926 (UMLS CUI [1,1])
    C0270611 (UMLS CUI [1,2])
    medical history of encephalitis
    Item
    Medical history of encephalitis
    boolean
    C0014038 (UMLS CUI [1])
    oculogyric crisis
    Item
    Oculogyric crisis
    boolean
    C0085637 (UMLS CUI [1])
    neuroleptic drugs at onset of disease
    Item
    Therapy using neuroleptic drugs at onset of disease
    boolean
    C0040615 (UMLS CUI [1,1])
    C0277793 (UMLS CUI [1,2])
    remission of disease
    Item
    Remission of disease
    boolean
    C0544452 (UMLS CUI [1])
    unilateral motor impairment
    Item
    Unilateral motor impairment after 3 years of disease
    boolean
    C0221163 (UMLS CUI [1,1])
    C0205092 (UMLS CUI [1,2])
    supranuclear palsy
    Item
    Supranuclear palsy
    boolean
    C1720037 (UMLS CUI [1])
    cerebellar signs
    Item
    Cerebellar signs
    boolean
    C0742038 (UMLS CUI [1])
    severe autonomous dysfunction
    Item
    Severe autonomous dysfunction (e.g. urinary incontinence, cardiovascular system problem)
    boolean
    C1397846 (UMLS CUI [1,1])
    C0205082 (UMLS CUI [1,2])
    early development of dementia
    Item
    Early development of dementia (within first year of disease) with special impairment of memory, speech and in practice
    boolean
    C1833334 (UMLS CUI [1,1])
    C0497327 (UMLS CUI [1,2])
    babinski sign
    Item
    Babinski sign
    boolean
    C0034935 (UMLS CUI [1])
    poor response to L-Dopa
    Item
    Poor response to L-Dopa
    boolean
    C2751071 (UMLS CUI [1])
    MTPT exposition
    Item
    1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MTPT) exposition
    boolean
    C0046312 (UMLS CUI [1])
    Item Group
    Morbus parkinson along with MCI (PD-MCI)
    C0030567 (UMLS CUI-1)
    C1270972 (UMLS CUI-3)
    PD-MCI diagnosis
    Item
    Is the patient currently diagnosed with PD-MCI according to the criteria Litvan et. al. 2012?
    boolean
    C0030567 (UMLS CUI [1])
    C1270972 (UMLS CUI [2])
    Parkinson disease according to UKPD BB criteria
    Item
    Diagnosis of Parkinson disease according to UKPD BB criteria
    boolean
    C0030567 (UMLS CUI [1,1])
    C0679228 (UMLS CUI [1,2])
    insidious onset and progressive course of disease of cognitive dysfunction
    Item
    Insidious onset and progressive course of disease of cognitive dysfunction under PD disease reported by participant, relatives and treating physician
    boolean
    C0338656 (UMLS CUI [1,1])
    C1298634 (UMLS CUI [1,2])
    C0338656 (UMLS CUI [1,3])
    C0242656 (UMLS CUI [1,4])
    C0205329 (UMLS CUI [1,5])
    loss of power
    Item
    Loss of power (<=1.5 times standard deviation below the mean value of reference group) in (A) two assessments of a domain or (B) in each assessment of two different domains. Following domains can be affected: -attention & working memory -executive functions -visuo-constructive/visuospatial performance -memory -speech
    boolean
    C0548863 (UMLS CUI [1])
    cognitive deficits do not interfere everyday activities
    Item
    Cognitive deficits do not interfere with lost of independence regarding everyday activities, although mild impairment may occur performing complex tasks
    boolean
    C0009241 (UMLS CUI [1,1])
    C0521103 (UMLS CUI [1,2])
    C0001288 (UMLS CUI [1,3])
    Dementia in Parkinson disease
    Item
    Parkinson disease with dementia according to criteria of Movement Disorder Society Task Force
    boolean
    C1828079 (UMLS CUI [1])
    diseases that can explain cognitive impairment
    Item
    Diseases that can explain cognitive impairment primarily (e.g. delir, stroke, traumatic brain injury, metabolic disorder, side effects of medication, severe depressive episode)
    boolean
    C0009241 (UMLS CUI [1,1])
    C0678226 (UMLS CUI [1,2])
    C0009488 (UMLS CUI [1,3])
    comorbidities influence on test performance
    Item
    Other PD associated comorbidities like severe motor impairment, high anxiety, depression,increasing daytime sleepiness, psychosis, which have a significant influence on test performance according to a clinician's opinion.
    boolean
    C0009488 (UMLS CUI [1,1])
    C0521102 (UMLS CUI [1,2])
    C0679605 (UMLS CUI [1,3])
    Item Group
    Dementia in Parkinsons disease
    C1828079 (UMLS CUI-1)
    PDD diagnosis
    Item
    Is the patient currently diagnosed with PDD according to the criteria Emre et. al. 2007?
    boolean
    C1828079 (UMLS CUI [1])
    diagnostic criteria for PD
    Item
    I. Main criteria of PDD Diagnosis of Parkonson disease according to UKPD BB criteria
    boolean
    C0030567 (UMLS CUI [1,1])
    C0679228 (UMLS CUI [1,2])
    course of disease of dementia
    Item
    Dementia syndrome with insidious onset and progressive course of disease with respect to Parkinson's disease (onset of dementia > 1 year after diagnosis of Parkinson's disease
    boolean
    C0497327 (UMLS CUI [1,1])
    C1298634 (UMLS CUI [1,2])
    C1828079 (UMLS CUI [2])
    loss of power
    Item
    Loss of power (<=1.5 times standard deviation below mean of reference group/normal population) affecting more than one cognitive domain and represent an impairment in contrast to the premobid level of performance
    boolean
    C0548863 (UMLS CUI [1])
    interference with everyday living activities
    Item
    Cognitive deficits interfer with loss of independence in everyday activities (concerning social, occupational or personal aspects of life). These limitations of activities in everyday living should be primarily caused by cognitive factors and not by motor impairment or an autonomous functional disorder.
    boolean
    C0009241 (UMLS CUI [1,1])
    C0521102 (UMLS CUI [1,2])
    C0001288 (UMLS CUI [1,3])
    affected domains
    Item
    II. Additional criteria a) Following domains can be affected: -attention & working memory -executive functions -visuo-constructive/visuospatial performance -memory -speech
    boolean
    C0004268 (UMLS CUI [1])
    C0025265 (UMLS CUI [2])
    C0935584 (UMLS CUI [3,1])
    C2370887 (UMLS CUI [3,2])
    C0025260 (UMLS CUI [4])
    C0037817 (UMLS CUI [5])
    behavior changes
    Item
    b) The following behavior changes can occur: -apathy (reduced spontaneity, loss of motivation, interest and performance) -change of personality like depressive or anxious symptomatic -hallucinations (at most visual, complex visions of humans, animals or objects) -delusions (at most of paranoid nature like cheating of the partner or foreign persons living in the household) -increased sleepiness at daytime or doziness at daytime
    boolean
    C0542299 (UMLS CUI [1])
    disease also causing cognitive impairment
    Item
    III. Findings, which make PDD diagnosis less probable Diseases that can also cause cognitive impairment as a result (e.g. vascular distinctive feature in MRI), but cannot be considered primary cause of dementia
    boolean
    C0009488 (UMLS CUI [1,1])
    C0678227 (UMLS CUI [1,2])
    C0009241 (UMLS CUI [1,3])
    time interval between onset of cognitive and motor symptoms unknown
    Item
    Time interval between onset of cognitive and motor symptoms unknown
    boolean
    C0872291 (UMLS CUI [1,1])
    C0332162 (UMLS CUI [1,2])
    C0426980 (UMLS CUI [1,3])
    C0392335 (UMLS CUI [1,4])
    symptoms exclusive due to comorbidity
    Item
    IV. Exclusion criteria of PDD Cognitive symptomes or behavioral disorders occor exclusively as part of another disease or concomitants, like e.g. delir caused by a systemic disease or as consequence of a certain medication or a severe depressive episode
    boolean
    C0011206 (UMLS CUI [1])
    C0442893 (UMLS CUI [2])
    C2364092 (UMLS CUI [3])
    probable vascular dementia
    Item
    Prevalence of a probable vascular dementia according to NINDS-AIREN-criteria
    boolean
    C0011269 (UMLS CUI [1,1])
    C0033204 (UMLS CUI [1,2])
    probable PDD
    Item
    Probable PDD -All criteria in section I have to be fulfilled -Typical profile of cognitive impairment along with distinctive features in more than one cognitive domain (IIa), especially deficits of attention, a dysexecutive syndrome, an impaired visuospatial perception, limitations regarding direct recall of memory performance with improvement after provided assistance in recall. -presence of at least one behavior disorder (IIb) -no criteria mentioned in section III and IV must be present
    boolean
    C1828079 (UMLS CUI [1,1])
    C0033204 (UMLS CUI [1,2])
    possible PDD
    Item
    Possible PDD -atypical profile of cognitive impairment with abnormalities in more than one cognitive domain (IIa) like fluent aphasia or memory impairment or attention deficit -behavior disorders (IIb) can be present but do not have to be present -one or more criteria in III may be present -no criterion mentioned in section IV must be present
    boolean
    C1828079 (UMLS CUI [1,1])
    C0332149 (UMLS CUI [1,2])
    Item Group
    Dementia with Lewy bodies (DLB)
    C0752347 (UMLS CUI-1)
    DLB diagnosis
    Item
    Is the patient currently diagnosed with DLB according to the criteria Mc Keith et. al. 2005?
    boolean
    C0752347 (UMLS CUI [1])
    dementia causing loss of independence
    Item
    Central Criterion 1. Dementia, defined as progressive loss, which causes loss of independence in everyday living activities (concerning social, occupational or personal life aspects)
    boolean
    C0497327 (UMLS CUI [1])
    C0085862 (UMLS CUI [2,1])
    C0001288 (UMLS CUI [2,2])
    C1517945 (UMLS CUI [2,3])
    fluctuation in cognition
    Item
    Main criteria 2. Fluctuations in cognition, especially attention performance
    boolean
    C0853574 (UMLS CUI [1,1])
    C0009240 (UMLS CUI [1,2])
    recurrent visual hallucinations
    Item
    3. Recurrent visual hallucinations, that typically consists of figures and are detailed.
    boolean
    C0233763 (UMLS CUI [1,1])
    C2945760 (UMLS CUI [1,2])
    spontaneous Parkinson symptomatic
    Item
    Spontaneous Parkinson symptomatic
    boolean
    C0030569 (UMLS CUI [1,1])
    C0205359 (UMLS CUI [1,2])
    REM sleep behavior disorder
    Item
    Additional criteria 5. REM sleep behavior disorder
    boolean
    C0751772 (UMLS CUI [1])
    severe side effects due to neuroleptic medication
    Item
    6. Severe side effects due to neuroleptic medication
    boolean
    C0041755 (UMLS CUI [1,1])
    C0205082 (UMLS CUI [1,2])
    C0040615 (UMLS CUI [1,3])
    dopamine transporter ligand storage in basal ganglia
    Item
    Reduced dopamine transporter ligand storage in basal ganglia (SPECT/PET)
    boolean
    C0114838 (UMLS CUI [1,1])
    C0392756 (UMLS CUI [1,2])
    C0004781 (UMLS CUI [1,3])
    C3828509 (UMLS CUI [2])
    dementia occurred about the same time
    Item
    8. Dementia occurred before or at the same time (resp. within first year) symptoms of Parkinson's disease did?
    boolean
    C0683368 (UMLS CUI [1,1])
    C0497327 (UMLS CUI [1,2])
    C3843404 (UMLS CUI [1,3])
    probable DLB
    Item
    Probable DLB (item 1 and at least one feature in 2-4 and at least one feature 5-7 applies, item 8 is fulfilled)
    boolean
    C0752347 (UMLS CUI [1,1])
    C0332148 (UMLS CUI [1,2])
    possible DLB
    Item
    Possible DLB (item 1 and at least one feature in 5-7 apply; item 8 is fulfilled)
    boolean
    C0752347 (UMLS CUI [1,1])
    C0332149 (UMLS CUI [1,2])
    frequent falls or syncopes
    Item
    Supportive Symptoms without diagnostic specifity Frequent falls or syncopes
    boolean
    C0850703 (UMLS CUI [1])
    C0039070 (UMLS CUI [2])
    transient loss of consciousness
    Item
    Transient loss of consciousness
    boolean
    C0041657 (UMLS CUI [1])
    severe autonomous functional disorder
    Item
    Severe autonomous dysfunction
    boolean
    C1397846 (UMLS CUI [1,1])
    C0205082 (UMLS CUI [1,2])
    hallucinations in other modalities
    Item
    Hallucinations in other modalities
    boolean
    C0018524 (UMLS CUI [1,1])
    C0695347 (UMLS CUI [1,2])
    C0205394 (UMLS CUI [1,3])
    delusion
    Item
    Delusions
    boolean
    C0011253 (UMLS CUI [1])
    depression
    Item
    Depression
    boolean
    C0011581 (UMLS CUI [1])
    medial temporal lobe
    Item
    Medial temporal lobe slightly changed in MRI
    boolean
    C0039485 (UMLS CUI [1,1])
    C0205098 (UMLS CUI [1,2])
    C0871641 (UMLS CUI [1,3])
    C0750482 (UMLS CUI [1,4])
    C0024485 (UMLS CUI [2])
    SPECT/PET perfusion scan at reduced occipital activity
    Item
    Light take in SPECT/PET perfusion scan at reduced occipital activity
    boolean
    C2094594 (UMLS CUI [1])
    C0443158 (UMLS CUI [2,1])
    C1184145 (UMLS CUI [2,2])
    abnormally light take in MIBG myocardial scintiscan
    Item
    Abnormally light take in MIBG myocardial scintiscan
    boolean
    C1704258 (UMLS CUI [1,1])
    C2229074 (UMLS CUI [1,2])
    slow-wave-activity in EEG with temporal transient shape waves
    Item
    Prominent slow-wave-activity in EEG with temporal transient shape waves
    boolean
    C2206519 (UMLS CUI [1])
    C2678116 (UMLS CUI [2])
    medical history of brain injury
    Item
    Findings making diagnosis less probable medical history of brain injury (e.g. stroke, traumatic brain injury)
    boolean
    C0262926 (UMLS CUI [1,1])
    C0270611 (UMLS CUI [1,2])
    presence of another medical or neurological condition explaining deficits
    Item
    Presence of another medical or neurological condition explaining deficits
    boolean
    C0009488 (UMLS CUI [1,1])
    C0678226 (UMLS CUI [1,2])
    C2987487 (UMLS CUI [1,3])
    first occurrence of symptoms of Parkinsons disease
    Item
    Symptoms of Parkinson's disease occur in a severe stadium of dementia for the first time
    boolean
    C0030567 (UMLS CUI [1,1])
    C0205435 (UMLS CUI [1,2])
    C0497327 (UMLS CUI [2,1])
    C0699749 (UMLS CUI [2,2])
    C0205082 (UMLS CUI [2,3])
    Item Group
    Progressive supranuclear palsy
    C0038868 (UMLS CUI-1)
    PSP diagnosis
    Item
    Is the patient currently diagnosed with PSP according to the criteria NINDS-PSP-criteria (Litvan et. al. 1996)?
    boolean
    C0038868 (UMLS CUI [1])
    gradual progressive disease
    Item
    Conditions Gradual progressive disease with onset of disease at age of at least 40
    boolean
    C1335499 (UMLS CUI [1,1])
    C0439833 (UMLS CUI [1,2])
    C0206132 (UMLS CUI [2])
    vertical gaze palsy
    Item
    Main criteria 1. Vertical palsy (up or down)
    boolean
    C0339652 (UMLS CUI [1])
    slowing of vertical saccades
    Item
    2. Slowing of vertical saccades
    boolean
    C4011795 (UMLS CUI [1])
    postural instability with falls
    Item
    3. Postural instability with falls < 1 year after onset of disease
    boolean
    C1843921 (UMLS CUI [1])
    C0085639 (UMLS CUI [2])
    probable PSP
    Item
    Probable PSP (item 1 and item 3 have to be answered with 'Yes'
    boolean
    C0038868 (UMLS CUI [1,1])
    C0033204 (UMLS CUI [1,2])
    possible PSP
    Item
    Possible PSP (item 1 or 2 und item 3 have to be answered with 'Yes')
    boolean
    C0038868 (UMLS CUI [1,1])
    C0332149 (UMLS CUI [1,2])
    symmetric akinesia or rigor,
    Item
    Supportive symptoms Symmetric akinesia or rigor, proximal more than distal
    boolean
    C0085623 (UMLS CUI [1,1])
    C0332516 (UMLS CUI [1,2])
    C0026837 (UMLS CUI [2])
    abnormal head posture especially retrocollis
    Item
    Abnormal head posture especially retrocollis
    boolean
    C1536116 (UMLS CUI [1])
    C3887667 (UMLS CUI [2])
    poor response to L-Dopa
    Item
    Poor or low response to L-Dopa
    boolean
    C2751071 (UMLS CUI [1])
    dysphagia or dysarthria
    Item
    Earlier dysphagia or dysarthria
    boolean
    C0011168 (UMLS CUI [1])
    C0013362 (UMLS CUI [2])
    symptoms of cognitive impairment
    Item
    Earlier onset of a cognitive impairment along with presence at least two of the following symptoms: Apathy, impairment of abstract thinking, reduced verbal fluency, tool use behavior, imitation and frontal lobe syndrome
    boolean
    C0085632 (UMLS CUI [1])
    C0233642 (UMLS CUI [2])
    C0751514 (UMLS CUI [3])
    C1720915 (UMLS CUI [4])
    C3826143 (UMLS CUI [5])
    C0549117 (UMLS CUI [6])
    medical history of brain injury
    Item
    Exclusion criteria Medical history of brain injury (e.g. encephalitis)
    boolean
    C0262926 (UMLS CUI [1,1])
    C0270611 (UMLS CUI [1,2])
    presence of another medical or neurological condition explaining deficits
    Item
    Presence of another medical or neurological condition explaining deficits
    boolean
    C0009488 (UMLS CUI [1,1])
    C0678226 (UMLS CUI [1,2])
    C2987487 (UMLS CUI [1,3])
    presence of alien limb syndrome
    Item
    Presence of alien limb syndrome
    boolean
    C3160753 (UMLS CUI [1])
    cortical perception disorder
    Item
    Presence of a cortical perception disorder
    boolean
    C0030975 (UMLS CUI [1])
    focal frontal or temporal-parietal atrophy
    Item
    Evidence of focal frontal or temporal-parietal atrophy
    boolean
    C2674464 (UMLS CUI [1])
    hallucinations and delusions
    Item
    Occurrence of hallucinations and delusions independently of a dopaminergic therapy
    boolean
    C0018524 (UMLS CUI [1])
    C0011253 (UMLS CUI [2])
    cerebellar diseases
    Item
    Prominent early signs of cerebellar diseases
    boolean
    C0007760 (UMLS CUI [1])
    severe autonomous functional disorder
    Item
    Evidence of severe autonomous functional disorder with unknown cause
    boolean
    C0277785 (UMLS CUI [1,1])
    C0205082 (UMLS CUI [1,2])
    severe asymmetric Parkinson syndrome
    Item
    Severe asymmetric signs of Parkinson's syndrome
    boolean
    C0030567 (UMLS CUI [1,1])
    C0332514 (UMLS CUI [1,2])
    C0205082 (UMLS CUI [1,3])
    evidence of relevant structural abnormalities
    Item
    Neuroradiological evidence of relevant structural abnormalities
    boolean
    C1866933 (UMLS CUI [1])
    Morbus Whipple
    Item
    Evidence of Morbus Whipple
    boolean
    C0023788 (UMLS CUI [1])
    Item Group
    Corticobasal degeneration (CBD)
    C0393570 (UMLS CUI-1)
    CBD diagnosis
    Item
    Is the patient currently diagnosed with CBD according to the criteria of Boeve et. al. 1996?
    boolean
    C0393570 (UMLS CUI [1])
    insidious onset and progressive course of disease
    Item
    Main criteria 1. Insidious onset and progressive course of disease
    boolean
    C0814125 (UMLS CUI [1])
    C0242656 (UMLS CUI [2,1])
    C0205329 (UMLS CUI [2,2])
    no other medical or neurological disease can explain the deficits
    Item
    2. No other medical or neurological disease can explain the deficits
    boolean
    C0009488 (UMLS CUI [1,1])
    C0678226 (UMLS CUI [1,2])
    C2987487 (UMLS CUI [1,3])
    ideomotor apraxia
    Item
    3. Cortical dysfunction with at least one of the following symptoms: 3a. Focal or asymmetric ideomotor apraxia
    boolean
    C0234523 (UMLS CUI [1])
    alien limb syndrome
    Item
    3b. Alien limb syndrome
    boolean
    C3160753 (UMLS CUI [1])
    cortical sensibility loss
    Item
    3c. Cortical sensibility loss
    boolean
    C0030975 (UMLS CUI [1])
    visual or sensorial hemineglect
    Item
    3d. Visual or sensorial hemineglect
    boolean
    C0751418 (UMLS CUI [1])
    constructional apraxia
    Item
    3e. Constructional apraxia
    boolean
    C2243023 (UMLS CUI [1])
    myoclonus
    Item
    3e. Focal or asymmetric myoclonus
    boolean
    C0027066 (UMLS CUI [1])
    speech apraxia or non-fluent aphasia
    Item
    3g. Speech apraxia/ non-fluent aphasia
    boolean
    C0264611 (UMLS CUI [1])
    C0349390 (UMLS CUI [2])
    rigor of extremities without clear reponse to L-Dopa
    Item
    4. Extrapyramidal dysfunction with at least one of the following symptomes: 4a. Focal or asymmetrical rigor of extremities without clear reponse to L-Dopa
    boolean
    C0026837 (UMLS CUI [1,1])
    C0278454 (UMLS CUI [1,2])
    C3806728 (UMLS CUI [2])
    focal or asymmetrical dystonia
    Item
    4b. Focal or asymmetrical dystonia of extremity muscles
    boolean
    C0743332 (UMLS CUI [1])
    C0332514 (UMLS CUI [2])
    diagnosis of CBD
    Item
    Diagnosis of CBD If item 1,2 and at least one item in 3a-3g and at least one in item 4a, item 4b is fulfilled
    boolean
    C0393570 (UMLS CUI [1,1])
    C0679228 (UMLS CUI [1,2])
    neuropsychological showing cognitive impairment
    Item
    Supportive symptoms Neuropsychological testing: Evidenz of focal or lateralised cognitive impairment with relatively maintained learning and memory function
    boolean
    C0027902 (UMLS CUI [1])
    C0338656 (UMLS CUI [2])
    C0233832 (UMLS CUI [3])
    C0025260 (UMLS CUI [4])
    atrophy showing in MRI or CT
    Item
    Evidence in MRI/ CT of focal or asymmetric atrophy, typically manifesting in frontoparietal cortex
    boolean
    C2674464 (UMLS CUI [1])
    C0011923 (UMLS CUI [2])
    focal or asymmetrical hypoperfusion showing in SPECT or PET
    Item
    Evidence in SPECT or PET of focal or asymmetrical hypoperfusion, typically manifested in frontoparietal cortex at maximum, with or without involvement of basal ganglia resp. the thalamus
    boolean
    C1695782 (UMLS CUI [1])
    C0183126 (UMLS CUI [2])
    C0032743 (UMLS CUI [3])
    Item Group
    Multiple system atrophy (MSA)
    C0393571 (UMLS CUI-1)
    MSA diagnosis
    Item
    Is the patient currently diagnosed with MSA according to the criteria of Gilman et. al. 2008?
    boolean
    C0393571 (UMLS CUI [1])
    episodic progressive disease
    Item
    Conditions Episodic, progressive disease with onset of symptoms after age of 30
    boolean
    C1335499 (UMLS CUI [1,1])
    C1455761 (UMLS CUI [1,2])
    C0150907 (UMLS CUI [2])
    episodic progressive disease
    Item
    Conditions Episodic, progressive disease with onset of symptoms after age of 30
    boolean
    C1335499 (UMLS CUI [1,1])
    C1455761 (UMLS CUI [1,2])
    C0150907 (UMLS CUI [2])
    Parkinsons disease
    Item
    1. Parkinson's disease (bradykinesia with rigor, tremor or postural instability)
    boolean
    C0030567 (UMLS CUI [1])
    cerebellar syndrome
    Item
    Or a cerebellar syndrome (gait ataxia with cerebellar dysarthria, limb ataxia or cerebellar oculomotor disturbance)
    boolean
    C0231691 (UMLS CUI [1])
    C0454599 (UMLS CUI [2])
    C0231691 (UMLS CUI [3])
    C1411869 (UMLS CUI [4])
    signs of autonomous dysfunction
    Item
    2. At least one sign suggesting autonomous dysfunction: -bladder incontinence that cannot be explained by another disease -incomplete urination -erectile dysfunction -significant decrease of orthostatic blood pressure without fulfilling criteria of a probable MSA
    boolean
    C0042024 (UMLS CUI [1])
    C0426373 (UMLS CUI [2])
    C0242350 (UMLS CUI [3])
    C1095974 (UMLS CUI [4])
    additional sign
    Item
    3. At least one additional sign have to be fulfilled
    boolean
    C0679228 (UMLS CUI [1])
    possible MSA
    Item
    Possible MSA (items 1-3 apply)
    boolean
    C0393571 (UMLS CUI [1,1])
    C0332149 (UMLS CUI [1,2])
    autonomous dysfunction
    Item
    Signs of a probable multiple system atrophy 4. Autonomous disorder -with bladder incontinence (inability to control urination along with erectile dysfunction among men) -orthostatic decrease of blood pressure standing for 3 minutes of >30mmHg systolic or >15mmHg
    boolean
    C0042024 (UMLS CUI [1])
    C0242350 (UMLS CUI [2])
    C1095974 (UMLS CUI [3])
    parkinsons syndrome
    Item
    5. Parkinson's syndrome (bradykinesia with rigor, tremor or postural instability) with poor response to Levodopa
    boolean
    C0030567 (UMLS CUI [1])
    C2751071 (UMLS CUI [2])
    cerebellar syndrome
    Item
    Or a cerebellar syndrome (gait ataxia with cerebellar dysarthria, limb ataxia or cerebellar oculomotor disturbance)
    boolean
    C0231691 (UMLS CUI [1])
    C0454599 (UMLS CUI [2])
    C0231691 (UMLS CUI [3])
    C1411869 (UMLS CUI [4])
    probable MSA
    Item
    Probable MSA (item 4-5 apply)
    boolean
    C0393571 (UMLS CUI [1,1])
    C0033204 (UMLS CUI [1,2])
    additional signs of MSA-P or MSA-C
    Item
    boolean
    C2732311 (UMLS CUI [1])
    C2733447 (UMLS CUI [2])
    Babinski sign with increased reflex
    Item
    Babinski sign with increased reflex
    boolean
    C0034935 (UMLS CUI [1])
    C0151889 (UMLS CUI [2])
    stridor
    Item
    Stridor
    boolean
    C0038450 (UMLS CUI [1])
    rapid progression of Parkinsons syndrome
    Item
    Rapid progression of Parkinson's syndrome
    boolean
    C1834700 (UMLS CUI [1])
    poor response to L-Dopa
    Item
    Poor response to L-Dopa
    boolean
    C2751071 (UMLS CUI [1])
    postural instability
    Item
    Postural instability within three years after first motor symptomes
    boolean
    C1843921 (UMLS CUI [1])
    cerebellar disorders
    Item
    Gait ataxia, cerebellar dysarthria, ataxia of extremities or cerebellar occulomotor disorder
    boolean
    C0751837 (UMLS CUI [1])
    C0454599 (UMLS CUI [2])
    C4015302 (UMLS CUI [3])
    C1411869 (UMLS CUI [4])
    dysphagia
    Item
    Dysphagia within 5 years after onset of motor disease
    boolean
    C0011168 (UMLS CUI [1])
    atrophy of putamen, cerebellar peduncle, pons or cerebellum
    Item
    Atrophy of putamen, cerebellar peduncle, pons or cerebellum proven in MRI
    boolean
    C1860217 (UMLS CUI [1])
    C0228515 (UMLS CUI [2,1])
    C0333641 (UMLS CUI [2,2])
    C3552972 (UMLS CUI [3])
    C0740279 (UMLS CUI [4])
    hypometabolism
    Item
    Hypometabolism in putamen, brain stem or cerebellum proven in FDG-PET
    boolean
    C0347938 (UMLS CUI [1])
    C3641247 (UMLS CUI [2])
    Parkinsons syndrome
    Item
    Parkinson's syndrome (bradykinesia and rigor)
    boolean
    C0030567 (UMLS CUI [1])
    C0233565 (UMLS CUI [2])
    C0026837 (UMLS CUI [3])
    atrophies
    Item
    Putamen atrophy, middling cerebellar peduncle atrophy or pons atrophy proven in MRI
    boolean
    C1860217 (UMLS CUI [1])
    C0228515 (UMLS CUI [2,1])
    C0333641 (UMLS CUI [2,2])
    C3552972 (UMLS CUI [3])
    presynaptic striatonigral dopaminergic denervation
    Item
    Presynaptic striatonigral dopaminergic denervation
    boolean
    C0011307 (UMLS CUI [1,1])
    C0206129 (UMLS CUI [1,2])
    C3498773 (UMLS CUI [1,3])
    C0034798 (UMLS CUI [1,4])
    orofacial dystonia
    Item
    Orofacial dystonia
    boolean
    C1395979 (UMLS CUI [1])
    antecollis
    Item
    Unproportional antecollis
    boolean
    C1698476 (UMLS CUI [1])
    camptocormia
    Item
    Camptocormia
    boolean
    C0264162 (UMLS CUI [1])
    contractions of hands and feet
    Item
    Contractions of hands and feet
    boolean
    C1140999 (UMLS CUI [1,1])
    C0018563 (UMLS CUI [1,2])
    C1140999 (UMLS CUI [2,1])
    C0016504 (UMLS CUI [2,2])
    inspiratory sighs
    Item
    Inspiratory sighs
    boolean
    C0425481 (UMLS CUI [1])
    severe dysphonia
    Item
    Severe dysphonia
    boolean
    C1527344 (UMLS CUI [1,1])
    C0205082 (UMLS CUI [1,2])
    severe dysarthria
    Item
    Severe dysarthria
    boolean
    C0013362 (UMLS CUI [1,1])
    C0205082 (UMLS CUI [1,2])
    incipient snoring
    Item
    Incipient snoring
    boolean
    C0037384 (UMLS CUI [1,1])
    C0205256 (UMLS CUI [1,2])
    cold hand or feet
    Item
    Cold hand or feet
    boolean
    C0857073 (UMLS CUI [1])
    pathological laughter or crying
    Item
    Pathological laughter or crying
    boolean
    C0424304 (UMLS CUI [1])
    C0860609 (UMLS CUI [2])
    resting or action tremor
    Item
    Jerky, myoclonic resting or action tremor
    boolean
    C0234379 (UMLS CUI [1])
    C0234376 (UMLS CUI [2])
    pill rolling tremor
    Item
    Classic pill rolling tremor
    boolean
    C0751564 (UMLS CUI [1])
    clinically significant neuropathy
    Item
    Clinically significant neuropathy
    boolean
    C0442874 (UMLS CUI [1,1])
    C2985739 (UMLS CUI [1,2])
    hallucinations
    Item
    Hallucinations which are not induced by medication
    boolean
    C0018524 (UMLS CUI [1])
    age at onset of disease
    Item
    Onset of disease after age of 75
    boolean
    C0150907 (UMLS CUI [1])
    family history of ataxia or parkinsonism
    Item
    Positive family history of ataxia or parkinsonism
    boolean
    C2939151 (UMLS CUI [1])
    C0004134 (UMLS CUI [2,1])
    C0241889 (UMLS CUI [2,2])
    dementia diagnosis
    Item
    Dementia diagnosis
    boolean
    C0497327 (UMLS CUI [1])
    lesions of white substance in MRI hinting for diagnosis of multiple sclerosis
    Item
    Lesions of white substance in MRI hinting for diagnosis of multiple sclerosis
    boolean
    C0682708 (UMLS CUI [1,1])
    C0221198 (UMLS CUI [1,2])
    C0026769 (UMLS CUI [2])
    Item Group
    Essential tremor (ET)
    C0270736 (UMLS CUI-1)
    ET diagnosis
    Item
    Is the patient currently diagnosed with ET according to the criteria of Deutschi et. al. 1998?
    boolean
    C0270736 (UMLS CUI [1])
    family genesis of tremor
    Item
    Known family genesis of tremor?
    boolean
    C0241889 (UMLS CUI [1,1])
    C0270736 (UMLS CUI [1,2])
    bilateral permanent resting or action tremor
    Item
    I. Signs of a definite essential tremor 1. Bilateral , at most symmetric resting or action tremor, concerning hands and forearms, that is persistent and visible (Persistent tremor in other body regions can occur in addition; bilateral tremor can have different severity grades; tremor is described by participant as permanent continuous, but amplitude of the tremor can vary; tremor can cause disabilities in everyday living, but do not have to.
    boolean
    C0234379 (UMLS CUI [1,1])
    C0238767 (UMLS CUI [1,2])
    C0205355 (UMLS CUI [1,3])
    C0234376 (UMLS CUI [2,1])
    C0238767 (UMLS CUI [2,2])
    C0205355 (UMLS CUI [2,3])
    duration of illness
    Item
    2. Duration of illness > 5 years
    boolean
    C3176590 (UMLS CUI [1])
    other abnormal neurological symptoms
    Item
    II. Exclusion criteria of definite essential tremor 3. Presence of other abnormal neurological symptoms
    boolean
    C1533281 (UMLS CUI [1,1])
    C0205394 (UMLS CUI [1,2])
    indications of tremor
    Item
    4. Presence of indications or known reasons for occurrence of physiologic tremor
    boolean
    C0040822 (UMLS CUI [1,1])
    C0392360 (UMLS CUI [1,2])
    intake of psychotics
    Item
    5. Current or earlier intake of psychogenic substances that can cause tremor or the current withdrawal symptoms
    boolean
    C0458006 (UMLS CUI [1,1])
    C0013227 (UMLS CUI [1,2])
    C0678227 (UMLS CUI [1,3])
    C0040822 (UMLS CUI [1,4])
    other disease causing tremor
    Item
    6. Other medical or neurological diseases, that can explain the tremor (especially brain injury, peripheral injury or trauma, 3 months before onset of tremor.
    boolean
    C0009488 (UMLS CUI [1,1])
    C0678227 (UMLS CUI [1,2])
    C0040822 (UMLS CUI [1,3])
    evidence for psychosomatic tremor
    Item
    7. Anamnestic indication or clinical evidence for psychosomatic tremor
    boolean
    C0040822 (UMLS CUI [1,1])
    C1537048 (UMLS CUI [1,2])
    indication for acute onset
    Item
    8. Indication for acute onset or stepped course of disease
    boolean
    C0242656 (UMLS CUI [1,1])
    C1276802 (UMLS CUI [1,2])
    signs of probable essential tremor
    Item
    III. Signs of probable essential tremor III1. Bilateral, mainly symmetric resting or action tremor, which concerns hands and forearms but other body parts as well like head or legs, visible and persistent (bilateral tremor can be asymmetric; tremor is described by participant as permanent continuous, but amplitude of the tremor can vary; tremor can cause disabilities in everyday living, but do not have to)
    boolean
    C0234379 (UMLS CUI [1,1])
    C0238767 (UMLS CUI [1,2])
    C0205355 (UMLS CUI [1,3])
    C0234376 (UMLS CUI [2,1])
    C0238767 (UMLS CUI [2,2])
    C0205355 (UMLS CUI [2,3])
    duration of disease
    Item
    III2. Duration of disease > 3 years
    boolean
    C3176590 (UMLS CUI [1])
    diagnostic exclusion criteria
    Item
    III Exclusion criteria for possible essential tremor III.3 At least one exclusion criterion mentioned in I3-I8 is true.
    boolean
    C0679228 (UMLS CUI [1])
    primary orthostatic tremor
    Item
    III4. Presence of primary orthostatic tremor
    boolean
    C0878578 (UMLS CUI [1])
    isolated tremor of the voice
    Item
    III5. Presence of isolated tremor of the voice
    boolean
    C0234808 (UMLS CUI [1,1])
    C0205409 (UMLS CUI [1,2])
    tremor specific to position and tasks
    Item
    III6. Presence of isolated tremor specific to position and tasks, including action-specific tremor or a primary writing tremor
    boolean
    C0040822 (UMLS CUI [1,1])
    C1262869 (UMLS CUI [1,2])
    C0205409 (UMLS CUI [1,3])
    isolated tongue or chin tremor
    Item
    III7. Presence of isolated tongue or chin tremor
    boolean
    C0241446 (UMLS CUI [1,1])
    C0205409 (UMLS CUI [1,2])
    C3809829 (UMLS CUI [2,1])
    C0205409 (UMLS CUI [2,2])
    type I essential tremor
    Item
    III. Signs of possible tremor Type I: Persons fulfilling criteria of definite or probable essential tremor but suffering from another neurological disease like parkinsonism, a dystonia, a peripheral neuropathology or restless leg syndrome. Persons fulfilling criteria of definite or probable essential tremor but suffering from other neurological symptoms that are not sufficient enough to make the diagnosis of another neurological illness. These symptoms concern for example minimal extra pyramidal signs like hypomimia, reduced arm drive or mild bradykinesia.
    boolean
    C0270736 (UMLS CUI [1])
    type II essential tremor
    Item
    Mono-symptomatic and isolated tremor with uncertain relation to essential tremor, like for example isolated tremor specific to position and task, including action-specific tremor or a primary writing tremor, a primary orthostatic tremor, an isolated voice tremor, an isolated leg tremor or a unilateral resting tremor of the hand.
    boolean
    C1865810 (UMLS CUI [1])
    exclusion criteria possible essential tremor
    Item
    III. Exclusion criteria for possible essential tremor At least one exclusion criterion mentioning in I4-I6 is true
    boolean
    C0679228 (UMLS CUI [1,1])
    C0270736 (UMLS CUI [1,2])
    C0332149 (UMLS CUI [1,3])
    Item Group
    Amyotrophic lateral sclerosis (ALS)
    C0002736 (UMLS CUI-1)
    ALS diagnosis
    Item
    Is the patient currently diagnosed with ALS according to the criteria of El-Escorial (revised 1998)?
    boolean
    C0002736 (UMLS CUI [1])
    lesion of first motor neuron
    Item
    Symptoms that have to be present: Signs of lesion of 1. motor neuron
    boolean
    C0235834 (UMLS CUI [1])
    lesion of second motor neuron
    Item
    Signs of lesion of 2. motor neuron (including EMG changes in clinically not affected muscles)
    boolean
    C0221198 (UMLS CUI [1,1])
    C0524459 (UMLS CUI [1,2])
    progression
    Item
    Progression
    boolean
    C0449258 (UMLS CUI [1])
    emotional disorder
    Item
    Symptoms, excluding a diagnosis of ALS emotional disorder
    boolean
    C0233459 (UMLS CUI [1])
    sphincter disturbance
    Item
    boolean
    C1409894 (UMLS CUI [1,1])
    C2699787 (UMLS CUI [1,2])
    vision disorders
    Item
    Vision disorder
    boolean
    C0042790 (UMLS CUI [1])
    autonomous dysfunctions
    Item
    Autonomous dysfunctions (e.g. urinary incontinence, cerebrovascular circulation)
    boolean
    C1397846 (UMLS CUI [1])
    alzheimers disease
    Item
    Alzheimer's disease
    boolean
    C0002395 (UMLS CUI [1])
    syndromes similar to ALS
    Item
    Syndromes similar to ALS
    boolean
    C0039082 (UMLS CUI [1,1])
    C2348205 (UMLS CUI [1,2])
    C0002736 (UMLS CUI [1,3])
    fasciculations
    Item
    Supportive symptoms Fasciculations in one or several region
    boolean
    C0015644 (UMLS CUI [1])
    neurogenic change in EMG
    Item
    Neurogenic change in EMG
    boolean
    C1843506 (UMLS CUI [1])
    nerve conduction study
    Item
    Normal motor and sensory nerve conduction study
    boolean
    C0200125 (UMLS CUI [1])
    missing nerve conduction block
    Item
    Missing nerve conduction block
    boolean
    C4012631 (UMLS CUI [1,1])
    C1705492 (UMLS CUI [1,2])
    damage of 1. and 2. motor neuron
    Item
    Definite ALS Signs of damage of 1. and 2. motor neuron in 3 of 4 regions (bulbar, cervical, thoracic, lumbosacral)
    boolean
    C0026609 (UMLS CUI [1,1])
    C1883709 (UMLS CUI [1,2])
    signs of probable ALS
    Item
    Probable ALS Signs of damage of 1. and 2. motor neuron in 2 of 4 regions, where the damage signs of 2. motor neuron has to be in position rostral of the damage of the 2. motor neuron
    boolean
    C0026609 (UMLS CUI [1,1])
    C1883709 (UMLS CUI [1,2])
    damage of 1. and 2. motor neuron in 1 of 4 regions
    Item
    Probable, laboratory supported symptoms Signs of damage of 1. and 2. motor neuron in 1 of 4 regions (or only one region of 1. motor neuron) and signs of degeneration in EMG in at least two extremities
    boolean
    C0026609 (UMLS CUI [1,1])
    C1883709 (UMLS CUI [1,2])
    signs of possible ALS
    Item
    Possible ALS Signs of damage of 1. and 2. motor neuron in one of 4 regions
    boolean
    C0026609 (UMLS CUI [1,1])
    C1883709 (UMLS CUI [1,2])
    Item Group
    Depression
    C0011581 (UMLS CUI-1)
    depression diagnosis
    Item
    Is the patient currently diagnosed with an episode of major depression according to the criteria of DSM-IV?
    boolean
    C0011581 (UMLS CUI [1])
    symptoms of episode of major depression
    Item
    Key syndrome: Episode of major depression Five (or more) of the following symptoms have been present during the same 2-week period and represent a change from previous functioning; at least one of the symptoms is either (1) depressed mood or (2) loss of interest or pleasure. 1 depressed mood most of the day, nearly every day, as indicated by either subjective report (e.g., feels sad or empty) or observation made by others (e.g., appears tearful). 2 markedly diminished interest or pleasure in all, or almost all, activities most of the day, nearly every day (as indicated by either subjective account or observation made by others) 3 significant weight loss when not dieting or weight gain (e.g., a change of more than 5% of body weight in a month), or decrease or increase in appetite nearly every day. 4 insomnia or hypersomnia nearly every day 5 psychomotor agitation or retardation nearly every day (observable by others, not merely subjective feelings of restlessness or being slowed down) 6 fatigue or loss of energy nearly every day 7 feelings of worthlessness or excessive or inappropriate guilt (which may be delusional) nearly every day (not merely self-reproach or guilt about being sick) 8 diminished ability to think or concentrate, or indecisiveness, nearly every day (either by subjective account or as observed by others) 9 recurrent thoughts of death (not just fear of dying), recurrent suicidal ideation without a specific plan, or a suicide attempt or a specific plan for committing suicide
    boolean
    C0024517 (UMLS CUI [1])
    no symptoms of mixed episode
    Item
    B The symptoms do not meet criteria for a Mixed Episode
    boolean
    C2874862 (UMLS CUI [1,1])
    C0332197 (UMLS CUI [1,2])
    clinically significant distress
    Item
    C. The symptoms cause clinically significant distress or impairment in social, occupational, or other important areas of functioning
    boolean
    C0700361 (UMLS CUI [1])
    C0679477 (UMLS CUI [2])
    symtoms not caused by substances or a general medical condition
    Item
    D. The symptoms are not due to the direct physiological effects of a substance (e.g., a drug of abuse, a medication) or a general medical condition (e.g., hypothyroidism).
    boolean
    C0728866 (UMLS CUI [1])
    C0236747 (UMLS CUI [2])
    symptoms are not better accounted for by bereavement
    Item
    E. The symptoms are not better accounted for by bereavement, i.e., after the loss of a loved one, the symptoms persist for longer than 2 months or are characterized by marked functional impairment, morbid preoc-cupation with worthlessness, suicidal ideation, psychotic symptoms, or psychomotor retardation.
    boolean
    C0005119 (UMLS CUI [1,1])
    C0683368 (UMLS CUI [1,2])
    presence of a single Major Depressive Episode
    Item
    A. Presence of a single Major Depressive Episode
    boolean
    C0024517 (UMLS CUI [1])
    major depressive episode is not better accounted for by schizoaffective disorder
    Item
    B. The Major Depressive Episode is not better accounted for by Schizoaffective Disorder and is not superimposed on Schizophrenia, Schizophreniform Disorder, Delusional Disorder, or Psychotic disorder not otherwise specified.
    boolean
    C0036337 (UMLS CUI [1,1])
    C0683368 (UMLS CUI [1,2])
    never manic mixed or hypomanic episode
    Item
    C. There has never been a Manic Episode, a Mixed Episode, or a Hypomanic Episode.
    boolean
    C0349208 (UMLS CUI [1,1])
    C2003901 (UMLS CUI [1,2])
    C1608433 (UMLS CUI [2,1])
    C2003901 (UMLS CUI [2,2])
    C1396834 (UMLS CUI [3,1])
    C2003901 (UMLS CUI [3,2])
    two or more major depressive episodes
    Item
    Major depression, recurrent A. Presence of two or more Major Depressive Episodes. (To be considered separate episodes, there must be an interval of at least 2 consecutive months in which criteria are not met for a Major Depressive Episode)
    boolean
    C0154409 (UMLS CUI [1])
    episodes are not better accounted for by schizoaffective disorder
    Item
    B. The Major Depressive Episodes are not better accounted for by Schizoaffective Disorder and are not superimposed on Schizophrenia, Schizophreniform Disorder, Delusional Disorder, or Psychotic Disorder Not Otherwise Specified.
    boolean
    C0036337 (UMLS CUI [1,1])
    C0686906 (UMLS CUI [1,2])
    no earlier manic mixed or hypomanic episode
    Item
    There has never been a Manic Episode, a Mixed Episode, or a Hypomanic Episode. (This exclusion does not apply if all of the manic-like, mixed-like, or hypomanic-like episodes are substance or treatment induced or are due to the direct physiological effects of a general medical condition.)
    boolean
    C0349208 (UMLS CUI [1,1])
    C2003901 (UMLS CUI [1,2])
    C1608433 (UMLS CUI [2,1])
    C2003901 (UMLS CUI [2,2])
    C1396834 (UMLS CUI [3,1])
    C2003901 (UMLS CUI [3,2])

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