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ID

13756

Beschreibung

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

Link

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

Stichworte

  1. 2/24/16 2/24/16 -
  2. 3/1/16 3/1/16 -
  3. 3/2/16 3/2/16 -
  4. 3/3/16 3/3/16 -
  5. 3/3/16 3/3/16 -
  6. 3/4/16 3/4/16 - Julian Varghese
  7. 3/7/16 3/7/16 -
  8. 3/9/16 3/9/16 -
  9. 3/9/16 3/9/16 -
  10. 3/9/16 3/9/16 -
  11. 3/10/16 3/10/16 -
  12. 3/10/16 3/10/16 -
  13. 4/13/21 4/13/21 - Dr. rer. medic Philipp Neuhaus
  14. 9/20/21 9/20/21 -
Hochgeladen am

March 3, 2016

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
    Beschreibung

    Date of visit

    Alias
    UMLS CUI-1
    C1320303
    Date of visit
    Beschreibung

    date of visit

    Datentyp

    date

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

    study id

    Datentyp

    text

    Alias
    UMLS CUI [1]
    C2826693
    Visit:
    Beschreibung

    visit name

    Datentyp

    text

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

    Mild cognitive impairment (MCI)

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

    current MCI

    Datentyp

    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...
    Beschreibung

    cognitive degration

    Datentyp

    boolean

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

    psychometric test proving cognitive degradation

    Datentyp

    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
    Beschreibung

    everyday functioning

    Datentyp

    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).
    Beschreibung

    signs of disturbed consciousness

    Datentyp

    boolean

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

    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

    Datentyp

    boolean

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

    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

    Datentyp

    boolean

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

    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

    Datentyp

    boolean

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

    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

    Datentyp

    boolean

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

    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

    Datentyp

    boolean

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

    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

    Datentyp

    boolean

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

    normal finding FAQ

    Datentyp

    boolean

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

    amnesic MCI single domain

    Datentyp

    boolean

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

    amnesic MCI multi domain

    Datentyp

    boolean

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

    non-amnesic MCI single domain

    Datentyp

    boolean

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

    non-amnesic MCI multi domain

    Datentyp

    boolean

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

    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)?
    Beschreibung

    NINCDS/ADRDA-Criteria AD diagnosis

    Datentyp

    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
    Beschreibung

    Clinical probable AD

    Datentyp

    boolean

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

    cognitive deficits

    Datentyp

    boolean

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

    progressive disturbance of memory

    Datentyp

    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
    Beschreibung

    no consciousness disturbance

    Datentyp

    boolean

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

    age at onset

    Datentyp

    boolean

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

    other system- or brain diseases

    Datentyp

    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)
    Beschreibung

    progressive deterioration of specific cognitive functions

    Datentyp

    boolean

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

    impairment of everyday activities and behavioral change

    Datentyp

    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)
    Beschreibung

    family history of similar disorders

    Datentyp

    boolean

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

    cerebral atrophy

    Datentyp

    boolean

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

    normal finding or unspecific changes in EEG

    Datentyp

    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
    Beschreibung

    normal finding of cerebrospinal fluid

    Datentyp

    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
    Beschreibung

    diagnosis of Alzheimers disease

    Datentyp

    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
    Beschreibung

    attendant symptoms

    Datentyp

    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
    Beschreibung

    neurological distinctive feature

    Datentyp

    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)
    Beschreibung

    epileptic seizure

    Datentyp

    boolean

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

    CT commensurate with age

    Datentyp

    boolean

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

    sudden apoplectic onset

    Datentyp

    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
    Beschreibung

    focal neurological signs

    Datentyp

    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
    Beschreibung

    epileptic seizures or gait disturbance

    Datentyp

    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
    Beschreibung

    dementia syndrome with atypical symptomatic

    Datentyp

    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
    Beschreibung

    second systemic or brain disease

    Datentyp

    boolean

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

    progressive severe cognitive deficit

    Datentyp

    boolean

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

    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?
    Beschreibung

    PCA diagnosis

    Datentyp

    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
    Beschreibung

    insidious onset and gradual progression

    Datentyp

    boolean

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

    visual disorders

    Datentyp

    boolean

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

    prominent complex visual deficits

    Datentyp

    boolean

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

    Balint syndrome

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0270706
    Visual agnosia
    Beschreibung

    visual agnosia

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0234502
    Dressing apraxis
    Beschreibung

    dressing apraxia

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0234529
    Topographic disorientation
    Beschreibung

    topographic disorientation

    Datentyp

    boolean

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

    relatively small deficits of memory and word fluency

    Datentyp

    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
    Beschreibung

    relatively good awareness of disability

    Datentyp

    boolean

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

    presenile onset

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0750901
    B. Alexia
    Beschreibung

    alexia

    Datentyp

    boolean

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

    Gerstmann syndrome

    Datentyp

    boolean

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

    ideomotor apraxia

    Datentyp

    boolean

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

    physical examination

    Datentyp

    boolean

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

    impairment of perceptual functions

    Datentyp

    boolean

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

    occipitoparietal abnormality

    Datentyp

    boolean

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

    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?)
    Beschreibung

    VD diagnoses

    Datentyp

    boolean

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

    cognitive impairment

    Datentyp

    boolean

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

    and at least two of the following abilities

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0029266
    Attention
    Beschreibung

    attention

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0004268
    Speech
    Beschreibung

    speech

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0037817
    Spatial visualization ability
    Beschreibung

    spatial visualization ability

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0679024
    Ability to judge
    Beschreibung

    ability to judge

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0022423
    Action ability
    Beschreibung

    action ability

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C1820715
    Abstraction ability
    Beschreibung

    abstraction ability

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0679195
    Motor control
    Beschreibung

    motor control

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0517961
    Praxia
    Beschreibung

    praxia

    Datentyp

    boolean

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

    everyday activities

    Datentyp

    boolean

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

    disturbed consciousness

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0549249
    Delirium
    Beschreibung

    delirium

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0011206
    Psychosis
    Beschreibung

    psychosis

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0033975
    Severe aphasia
    Beschreibung

    severe aphasia

    Datentyp

    boolean

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

    sensomotoric disorder

    Datentyp

    boolean

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

    systemic or other brain diseases

    Datentyp

    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
    Beschreibung

    cerebrovascular disease

    Datentyp

    boolean

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

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

    Datentyp

    boolean

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

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

    Datentyp

    boolean

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

    parietotemporal or temporoparietal association complex

    Datentyp

    boolean

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

    watershed infarction

    Datentyp

    boolean

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

    basal ganglia and frontal lacuna of white matter

    Datentyp

    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
    Beschreibung

    periventricular white matter lesions

    Datentyp

    boolean

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

    bilateral thalamus lesions

    Datentyp

    boolean

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

    great vessel lesions

    Datentyp

    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
    Beschreibung

    bilateral lesions of great vessel

    Datentyp

    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
    Beschreibung

    leukoencephalopathy

    Datentyp

    boolean

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

    onset of dementia

    Datentyp

    boolean

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

    abrupt degeneration of cognitive functions

    Datentyp

    boolean

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

    progression of cognitive deficits

    Datentyp

    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
    Beschreibung

    gait disturbance

    Datentyp

    boolean

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

    motoric instability and frequent support

    Datentyp

    boolean

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

    bladder problem

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0579152
    Pseudobulbar paralysis
    Beschreibung

    pseudobulbar paralysis

    Datentyp

    boolean

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

    personality disorder or mood alteration

    Datentyp

    boolean

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

    abulia

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0919974
    Depression
    Beschreibung

    depression

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0011581
    Emotional incontinence
    Beschreibung

    emotional incontinence

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C1142234
    Other subcortical deficits
    Beschreibung

    other subcortical deficits

    Datentyp

    boolean

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

    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?
    Beschreibung

    bvFTD diagnosis

    Datentyp

    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
    Beschreibung

    slowly progressing disturbance of behavior or of cognition

    Datentyp

    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
    Beschreibung

    socially inappropriate behavior

    Datentyp

    boolean

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

    loss of manners and decency

    Datentyp

    boolean

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

    impulsive or reckless behavior

    Datentyp

    boolean

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

    apathy

    Datentyp

    boolean

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

    inactivity

    Datentyp

    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
    Beschreibung

    reduced sympathy

    Datentyp

    boolean

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

    reduced interest in social contacts or human affection

    Datentyp

    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
    Beschreibung

    repetitive movements

    Datentyp

    boolean

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

    compulsive or ritualized behaviour patterns

    Datentyp

    boolean

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

    stereotypes in language

    Datentyp

    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
    Beschreibung

    disturbed preference in choice of food

    Datentyp

    boolean

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

    eating orgy or increased consumption of alcohol or cigarettes

    Datentyp

    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
    Beschreibung

    hyperorality

    Datentyp

    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
    Beschreibung

    disturbance in executive functions

    Datentyp

    boolean

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

    performance of episodic memory

    Datentyp

    boolean

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

    visuocontructive functions

    Datentyp

    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
    Beschreibung

    FTD diagnosis

    Datentyp

    boolean

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

    everyday functioning

    Datentyp

    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
    Beschreibung

    anterior temporal atrophy

    Datentyp

    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
    Beschreibung

    frontal or temporal hypo-perfusion

    Datentyp

    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
    Beschreibung

    FTD criteria fulfilled

    Datentyp

    boolean

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

    histopathological evidence

    Datentyp

    boolean

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

    mutation causing the behavioural variant of FTD

    Datentyp

    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
    Beschreibung

    disease pattern better explained by another disease

    Datentyp

    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
    Beschreibung

    behavioral disturbances explained by a psychiatric disease

    Datentyp

    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
    Beschreibung

    biomarkers emphasize Alzheimers disease or another neurodegenarative disease

    Datentyp

    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)
    Beschreibung

    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?
    Beschreibung

    PPA diagnosis

    Datentyp

    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.
    Beschreibung

    impairment of speech characteristics

    Datentyp

    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.
    Beschreibung

    restrictions in everylife due to speech impairment

    Datentyp

    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).
    Beschreibung

    intact premobid function of speech

    Datentyp

    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.
    Beschreibung

    symtoms missing in first two years

    Datentyp

    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.
    Beschreibung

    dyscalculia, ideomotoric apraxia and problems tracing

    Datentyp

    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.
    Beschreibung

    other cognitive functions

    Datentyp

    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.
    Beschreibung

    other reasons for aphasia excluded

    Datentyp

    boolean

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

    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?
    Beschreibung

    PNFA diagnosis

    Datentyp

    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
    Beschreibung

    agrammatism

    Datentyp

    boolean

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

    speech apraxia

    Datentyp

    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
    Beschreibung

    speech intelligibility disturbances

    Datentyp

    boolean

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

    speech intelligibility

    Datentyp

    boolean

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

    object knowledge

    Datentyp

    boolean

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

    clinical diagnosis of PNFA

    Datentyp

    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
    Beschreibung

    atrophy in posterior part of frontal lobe in MRI

    Datentyp

    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
    Beschreibung

    hypoperfusion and hypometabolism in posterior part of frontal lobe

    Datentyp

    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
    Beschreibung

    clinical diagnosis PNFA

    Datentyp

    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)
    Beschreibung

    neurodegenarative pathology

    Datentyp

    boolean

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

    mutation

    Datentyp

    boolean

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

    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?
    Beschreibung

    diagnosis of LPA

    Datentyp

    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
    Beschreibung

    disturbed sponteneous speech

    Datentyp

    boolean

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

    disturbed repetition of sentences

    Datentyp

    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
    Beschreibung

    phonologic paraphrasia

    Datentyp

    boolean

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

    speech intelligibility and object knowledge

    Datentyp

    boolean

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

    motor speech

    Datentyp

    boolean

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

    no distinctive agrammatism

    Datentyp

    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
    Beschreibung

    clinical diagnosis of LPA

    Datentyp

    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
    Beschreibung

    atrophy in posterior sections of perisylvian area or parietal atrophy

    Datentyp

    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
    Beschreibung

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

    Datentyp

    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
    Beschreibung

    clinical diagnosis LPA

    Datentyp

    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)
    Beschreibung

    histopathological evidence of a specific neurodegenerative pathology

    Datentyp

    boolean

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

    evidence of mutation

    Datentyp

    boolean

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

    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?
    Beschreibung

    diagnosis of SemD

    Datentyp

    boolean

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

    anomia

    Datentyp

    boolean

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

    disturbed speech intelligibility

    Datentyp

    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
    Beschreibung

    disturbed object knowledge

    Datentyp

    boolean

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

    dyslexia or agraphia

    Datentyp

    boolean

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

    undisturbed repetition

    Datentyp

    boolean

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

    fluent production of speech

    Datentyp

    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
    Beschreibung

    clinical diagnosis of SemD

    Datentyp

    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
    Beschreibung

    atrophy in anterior part of frontal lobe

    Datentyp

    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
    Beschreibung

    hypoperfusion and hypometabolism in anterior part of frontal lope

    Datentyp

    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
    Beschreibung

    clinical diagnosis SemD

    Datentyp

    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)
    Beschreibung

    neurodegenarative pathology

    Datentyp

    boolean

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

    mutation

    Datentyp

    boolean

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

    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)?
    Beschreibung

    IPS diagnosis

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0030567
    Hypo-bradykinesia
    Beschreibung

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

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C3176307
    Resting tremor
    Beschreibung

    resting tremor

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0234379
    Rigor
    Beschreibung

    rigor

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0026837
    Postural instability
    Beschreibung

    postural instability

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C1843921
    Unilateral onset
    Beschreibung

    Supportive symptoms (at least 3 'Yes' answers)

    Datentyp

    boolean

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

    resting tremor manifest

    Datentyp

    boolean

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

    progressive course of disease

    Datentyp

    boolean

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

    laterality

    Datentyp

    boolean

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

    response to L-Dopa

    Datentyp

    boolean

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

    L-Dopa induced chorea

    Datentyp

    boolean

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

    response to L-Dopa over 5 years

    Datentyp

    boolean

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

    response to L-Dopa over 10 years

    Datentyp

    boolean

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

    Exclusion criteria (No symptom is allowed to be fulfilled)

    Datentyp

    boolean

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

    medical history of encephalitis

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0014038
    Oculogyric crisis
    Beschreibung

    oculogyric crisis

    Datentyp

    boolean

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

    neuroleptic drugs at onset of disease

    Datentyp

    boolean

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

    remission of disease

    Datentyp

    boolean

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

    unilateral motor impairment

    Datentyp

    boolean

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

    supranuclear palsy

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C1720037
    Cerebellar signs
    Beschreibung

    cerebellar signs

    Datentyp

    boolean

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

    severe autonomous dysfunction

    Datentyp

    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
    Beschreibung

    early development of dementia

    Datentyp

    boolean

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

    babinski sign

    Datentyp

    boolean

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

    poor response to L-Dopa

    Datentyp

    boolean

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

    MTPT exposition

    Datentyp

    boolean

    Alias
    UMLS CUI [1]
    C0046312

    Ähnliche Modelle

    CRF Diagnostic Criteria

    Name
    Typ
    Description | Question | Decode (Coded Value)
    Datentyp
    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
    C0270706 (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])

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