ID

13765

Beschrijving

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

Trefwoorden

  1. 24-02-16 24-02-16 -
  2. 01-03-16 01-03-16 -
  3. 02-03-16 02-03-16 -
  4. 03-03-16 03-03-16 -
  5. 03-03-16 03-03-16 -
  6. 04-03-16 04-03-16 - Julian Varghese
  7. 07-03-16 07-03-16 -
  8. 09-03-16 09-03-16 -
  9. 09-03-16 09-03-16 -
  10. 09-03-16 09-03-16 -
  11. 10-03-16 10-03-16 -
  12. 10-03-16 10-03-16 -
  13. 13-04-21 13-04-21 - Dr. rer. medic Philipp Neuhaus
  14. 20-09-21 20-09-21 -
Geüploaded op

4 maart 2016

DOI

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Licentie

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
Beschrijving

Date of visit

Alias
UMLS CUI-1
C1320303
Date of visit
Beschrijving

date of visit

Datatype

date

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

study id

Datatype

text

Alias
UMLS CUI [1]
C2826693
Visit:
Beschrijving

visit name

Datatype

text

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

Mild cognitive impairment (MCI)

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

current MCI

Datatype

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...
Beschrijving

cognitive degration

Datatype

boolean

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

psychometric test proving cognitive degradation

Datatype

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
Beschrijving

everyday functioning

Datatype

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).
Beschrijving

signs of disturbed consciousness

Datatype

boolean

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

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

Datatype

boolean

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

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

Datatype

boolean

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

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

Datatype

boolean

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

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

Datatype

boolean

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

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

Datatype

boolean

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

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

Datatype

boolean

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

normal finding FAQ

Datatype

boolean

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

amnesic MCI single domain

Datatype

boolean

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

amnesic MCI multi domain

Datatype

boolean

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

non-amnesic MCI single domain

Datatype

boolean

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

non-amnesic MCI multi domain

Datatype

boolean

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

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)?
Beschrijving

NINCDS/ADRDA-Criteria AD diagnosis

Datatype

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
Beschrijving

Clinical probable AD

Datatype

boolean

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

cognitive deficits

Datatype

boolean

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

progressive disturbance of memory

Datatype

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
Beschrijving

no consciousness disturbance

Datatype

boolean

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

age at onset

Datatype

boolean

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

other system- or brain diseases

Datatype

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)
Beschrijving

progressive deterioration of specific cognitive functions

Datatype

boolean

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

impairment of everyday activities and behavioral change

Datatype

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)
Beschrijving

family history of similar disorders

Datatype

boolean

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

cerebral atrophy

Datatype

boolean

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

normal finding or unspecific changes in EEG

Datatype

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
Beschrijving

normal finding of cerebrospinal fluid

Datatype

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
Beschrijving

diagnosis of Alzheimers disease

Datatype

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
Beschrijving

attendant symptoms

Datatype

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
Beschrijving

neurological distinctive feature

Datatype

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)
Beschrijving

epileptic seizure

Datatype

boolean

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

CT commensurate with age

Datatype

boolean

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

sudden apoplectic onset

Datatype

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
Beschrijving

focal neurological signs

Datatype

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
Beschrijving

epileptic seizures or gait disturbance

Datatype

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
Beschrijving

dementia syndrome with atypical symptomatic

Datatype

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
Beschrijving

second systemic or brain disease

Datatype

boolean

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

progressive severe cognitive deficit

Datatype

boolean

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

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?
Beschrijving

PCA diagnosis

Datatype

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
Beschrijving

insidious onset and gradual progression

Datatype

boolean

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

visual disorders

Datatype

boolean

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

prominent complex visual deficits

Datatype

boolean

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

Balint syndrome

Datatype

boolean

Alias
UMLS CUI [1]
C0270706
Visual agnosia
Beschrijving

visual agnosia

Datatype

boolean

Alias
UMLS CUI [1]
C0234502
Dressing apraxis
Beschrijving

dressing apraxia

Datatype

boolean

Alias
UMLS CUI [1]
C0234529
Topographic disorientation
Beschrijving

topographic disorientation

Datatype

boolean

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

relatively small deficits of memory and word fluency

Datatype

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
Beschrijving

relatively good awareness of disability

Datatype

boolean

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

presenile onset

Datatype

boolean

Alias
UMLS CUI [1]
C0750901
B. Alexia
Beschrijving

alexia

Datatype

boolean

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

Gerstmann syndrome

Datatype

boolean

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

ideomotor apraxia

Datatype

boolean

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

physical examination

Datatype

boolean

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

impairment of perceptual functions

Datatype

boolean

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

occipitoparietal abnormality

Datatype

boolean

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

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?)
Beschrijving

VD diagnoses

Datatype

boolean

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

cognitive impairment

Datatype

boolean

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

and at least two of the following abilities

Datatype

boolean

Alias
UMLS CUI [1]
C0029266
Attention
Beschrijving

attention

Datatype

boolean

Alias
UMLS CUI [1]
C0004268
Speech
Beschrijving

speech

Datatype

boolean

Alias
UMLS CUI [1]
C0037817
Spatial visualization ability
Beschrijving

spatial visualization ability

Datatype

boolean

Alias
UMLS CUI [1]
C0679024
Ability to judge
Beschrijving

ability to judge

Datatype

boolean

Alias
UMLS CUI [1]
C0022423
Action ability
Beschrijving

action ability

Datatype

boolean

Alias
UMLS CUI [1]
C1820715
Abstraction ability
Beschrijving

abstraction ability

Datatype

boolean

Alias
UMLS CUI [1]
C0679195
Motor control
Beschrijving

motor control

Datatype

boolean

Alias
UMLS CUI [1]
C0517961
Praxia
Beschrijving

praxia

Datatype

boolean

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

everyday activities

Datatype

boolean

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

disturbed consciousness

Datatype

boolean

Alias
UMLS CUI [1]
C0549249
Delirium
Beschrijving

delirium

Datatype

boolean

Alias
UMLS CUI [1]
C0011206
Psychosis
Beschrijving

psychosis

Datatype

boolean

Alias
UMLS CUI [1]
C0033975
Severe aphasia
Beschrijving

severe aphasia

Datatype

boolean

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

sensomotoric disorder

Datatype

boolean

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

systemic or other brain diseases

Datatype

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
Beschrijving

cerebrovascular disease

Datatype

boolean

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

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

Datatype

boolean

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

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

Datatype

boolean

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

parietotemporal or temporoparietal association complex

Datatype

boolean

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

watershed infarction

Datatype

boolean

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

basal ganglia and frontal lacuna of white matter

Datatype

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
Beschrijving

periventricular white matter lesions

Datatype

boolean

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

bilateral thalamus lesions

Datatype

boolean

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

great vessel lesions

Datatype

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
Beschrijving

bilateral lesions of great vessel

Datatype

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
Beschrijving

leukoencephalopathy

Datatype

boolean

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

onset of dementia

Datatype

boolean

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

abrupt degeneration of cognitive functions

Datatype

boolean

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

progression of cognitive deficits

Datatype

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
Beschrijving

gait disturbance

Datatype

boolean

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

motoric instability and frequent support

Datatype

boolean

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

bladder problem

Datatype

boolean

Alias
UMLS CUI [1]
C0579152
Pseudobulbar paralysis
Beschrijving

pseudobulbar paralysis

Datatype

boolean

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

personality disorder or mood alteration

Datatype

boolean

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

abulia

Datatype

boolean

Alias
UMLS CUI [1]
C0919974
Depression
Beschrijving

depression

Datatype

boolean

Alias
UMLS CUI [1]
C0011581
Emotional incontinence
Beschrijving

emotional incontinence

Datatype

boolean

Alias
UMLS CUI [1]
C1142234
Other subcortical deficits
Beschrijving

other subcortical deficits

Datatype

boolean

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

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?
Beschrijving

bvFTD diagnosis

Datatype

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
Beschrijving

slowly progressing disturbance of behavior or of cognition

Datatype

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
Beschrijving

socially inappropriate behavior

Datatype

boolean

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

loss of manners and decency

Datatype

boolean

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

impulsive or reckless behavior

Datatype

boolean

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

apathy

Datatype

boolean

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

inactivity

Datatype

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
Beschrijving

reduced sympathy

Datatype

boolean

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

reduced interest in social contacts or human affection

Datatype

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
Beschrijving

repetitive movements

Datatype

boolean

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

compulsive or ritualized behaviour patterns

Datatype

boolean

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

stereotypes in language

Datatype

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
Beschrijving

disturbed preference in choice of food

Datatype

boolean

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

eating orgy or increased consumption of alcohol or cigarettes

Datatype

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
Beschrijving

hyperorality

Datatype

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
Beschrijving

disturbance in executive functions

Datatype

boolean

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

performance of episodic memory

Datatype

boolean

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

visuocontructive functions

Datatype

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
Beschrijving

FTD diagnosis

Datatype

boolean

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

everyday functioning

Datatype

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
Beschrijving

anterior temporal atrophy

Datatype

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
Beschrijving

frontal or temporal hypo-perfusion

Datatype

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
Beschrijving

FTD criteria fulfilled

Datatype

boolean

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

histopathological evidence

Datatype

boolean

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

mutation causing the behavioural variant of FTD

Datatype

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
Beschrijving

disease pattern better explained by another disease

Datatype

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
Beschrijving

behavioral disturbances explained by a psychiatric disease

Datatype

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
Beschrijving

biomarkers emphasize Alzheimers disease or another neurodegenarative disease

Datatype

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)
Beschrijving

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?
Beschrijving

PPA diagnosis

Datatype

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.
Beschrijving

impairment of speech characteristics

Datatype

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.
Beschrijving

restrictions in everylife due to speech impairment

Datatype

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).
Beschrijving

intact premobid function of speech

Datatype

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.
Beschrijving

symtoms missing in first two years

Datatype

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.
Beschrijving

dyscalculia, ideomotoric apraxia and problems tracing

Datatype

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.
Beschrijving

other cognitive functions

Datatype

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.
Beschrijving

other reasons for aphasia excluded

Datatype

boolean

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

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?
Beschrijving

PNFA diagnosis

Datatype

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
Beschrijving

agrammatism

Datatype

boolean

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

speech apraxia

Datatype

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
Beschrijving

speech intelligibility disturbances

Datatype

boolean

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

speech intelligibility

Datatype

boolean

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

object knowledge

Datatype

boolean

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

clinical diagnosis of PNFA

Datatype

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
Beschrijving

atrophy in posterior part of frontal lobe in MRI

Datatype

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
Beschrijving

hypoperfusion and hypometabolism in posterior part of frontal lobe

Datatype

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
Beschrijving

clinical diagnosis PNFA

Datatype

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)
Beschrijving

neurodegenarative pathology

Datatype

boolean

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

mutation

Datatype

boolean

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

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?
Beschrijving

diagnosis of LPA

Datatype

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
Beschrijving

disturbed sponteneous speech

Datatype

boolean

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

disturbed repetition of sentences

Datatype

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
Beschrijving

phonologic paraphrasia

Datatype

boolean

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

speech intelligibility and object knowledge

Datatype

boolean

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

motor speech

Datatype

boolean

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

no distinctive agrammatism

Datatype

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
Beschrijving

clinical diagnosis of LPA

Datatype

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
Beschrijving

atrophy in posterior sections of perisylvian area or parietal atrophy

Datatype

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
Beschrijving

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

Datatype

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
Beschrijving

clinical diagnosis LPA

Datatype

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)
Beschrijving

histopathological evidence of a specific neurodegenerative pathology

Datatype

boolean

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

evidence of mutation

Datatype

boolean

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

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?
Beschrijving

diagnosis of SemD

Datatype

boolean

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

anomia

Datatype

boolean

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

disturbed speech intelligibility

Datatype

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
Beschrijving

disturbed object knowledge

Datatype

boolean

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

dyslexia or agraphia

Datatype

boolean

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

undisturbed repetition

Datatype

boolean

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

fluent production of speech

Datatype

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
Beschrijving

clinical diagnosis of SemD

Datatype

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
Beschrijving

atrophy in anterior part of frontal lobe

Datatype

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
Beschrijving

hypoperfusion and hypometabolism in anterior part of frontal lope

Datatype

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
Beschrijving

clinical diagnosis SemD

Datatype

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)
Beschrijving

neurodegenarative pathology

Datatype

boolean

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

mutation

Datatype

boolean

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

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)?
Beschrijving

IPS diagnosis

Datatype

boolean

Alias
UMLS CUI [1]
C0030567
Hypo-bradykinesia
Beschrijving

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

Datatype

boolean

Alias
UMLS CUI [1]
C3176307
Resting tremor
Beschrijving

resting tremor

Datatype

boolean

Alias
UMLS CUI [1]
C0234379
Rigor
Beschrijving

rigor

Datatype

boolean

Alias
UMLS CUI [1]
C0026837
Postural instability
Beschrijving

postural instability

Datatype

boolean

Alias
UMLS CUI [1]
C1843921
Unilateral onset
Beschrijving

Supportive symptoms (at least 3 'Yes' answers)

Datatype

boolean

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

resting tremor manifest

Datatype

boolean

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

progressive course of disease

Datatype

boolean

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

laterality

Datatype

boolean

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

response to L-Dopa

Datatype

boolean

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

L-Dopa induced chorea

Datatype

boolean

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

response to L-Dopa over 5 years

Datatype

boolean

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

response to L-Dopa over 10 years

Datatype

boolean

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

Exclusion criteria (No symptom is allowed to be fulfilled)

Datatype

boolean

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

medical history of encephalitis

Datatype

boolean

Alias
UMLS CUI [1]
C0014038
Oculogyric crisis
Beschrijving

oculogyric crisis

Datatype

boolean

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

neuroleptic drugs at onset of disease

Datatype

boolean

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

remission of disease

Datatype

boolean

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

unilateral motor impairment

Datatype

boolean

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

supranuclear palsy

Datatype

boolean

Alias
UMLS CUI [1]
C1720037
Cerebellar signs
Beschrijving

cerebellar signs

Datatype

boolean

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

severe autonomous dysfunction

Datatype

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
Beschrijving

early development of dementia

Datatype

boolean

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

babinski sign

Datatype

boolean

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

poor response to L-Dopa

Datatype

boolean

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

MTPT exposition

Datatype

boolean

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

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?
Beschrijving

PD-MCI diagnosis

Datatype

boolean

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

Main criteria of PD-MCI

Datatype

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
Beschrijving

insidious onset and progressive course of disease of cognitive dysfunction

Datatype

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
Beschrijving

loss of power

Datatype

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
Beschrijving

cognitive deficits do not interfere everyday activities

Datatype

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
Beschrijving

Exclusion criteria of PD-MCI

Datatype

boolean

Alias
UMLS CUI [1]
C0349081
Diseases that can explain cognitive impairment primarily (e.g. delir, stroke, traumatic brain injury, metabolic disorder, side effects of medication, severe depressive episode)
Beschrijving

diseases that can explain cognitive impairment

Datatype

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.
Beschrijving

comorbidities influence on test performance

Datatype

boolean

Alias
UMLS CUI [1,1]
C0009488
UMLS CUI [1,2]
C0521102
UMLS CUI [1,3]
C0679605

Similar models

CRF Diagnostic Criteria

Name
Type
Description | Question | Decode (Coded Value)
Datatype
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])
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
C0349081 (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])

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