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ID
13751
Descrizione
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
collegamento
http://www.dzne.de/forschung/forschungsbereiche/klinische-forschung/studien/describe-bn006.html
Keywords
versioni (14)
- 24/02/16 24/02/16 -
- 01/03/16 01/03/16 -
- 02/03/16 02/03/16 -
- 03/03/16 03/03/16 -
- 03/03/16 03/03/16 -
- 04/03/16 04/03/16 - Julian Varghese
- 07/03/16 07/03/16 -
- 09/03/16 09/03/16 -
- 09/03/16 09/03/16 -
- 09/03/16 09/03/16 -
- 10/03/16 10/03/16 -
- 10/03/16 10/03/16 -
- 13/04/21 13/04/21 - Dr. rer. medic Philipp Neuhaus
- 20/09/21 20/09/21 -
Caricato su
2 marzo 2016
DOI
Per favore, per richiedere un accesso.
Licenza
Creative Commons BY-NC 3.0
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DESCRIBE (DZNE – Clinical Register Study of neurodegenerative Disorders) CRF Diagnostic Criteria
CRF Diagnostic Criteria
- StudyEvent: ODM
Similar models
CRF Diagnostic Criteria
- StudyEvent: ODM
Name
genere
Description | Question | Decode (Coded Value)
Tipo di dati
Alias
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
C0205329 (UMLS CUI [1,2])
C0205082 (UMLS CUI [1,3])
Item Group
Posterior cortical atrophy (PCA)
C0235946 (UMLS CUI-1)
C0205095 (UMLS CUI-2)
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])
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])
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])
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])
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])
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])
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])
C1184145 (UMLS CUI [1,2])
C0442030 (UMLS CUI [1,3])
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])
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])
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])
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])
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])
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])
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])
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])
C0228157 (UMLS CUI [1,2])
bilateral thalamus lesions
Item
Bilateral thalamus lesions
boolean
C2026261 (UMLS CUI [1,1])
C0238767 (UMLS CUI [1,2])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
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])
C2266670 (UMLS CUI [2])
stereotypes in language
Item
D.3. Stereotypes in language
boolean
C0038271 (UMLS CUI [1,1])
C0037817 (UMLS CUI [1,2])
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])
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])
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])
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])
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])
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])
histopathologic evidence
Item
B. Histopathologic 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])
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])
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])
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])
C0002395 (UMLS CUI [2,1])
C0524851 (UMLS CUI [2,2])
C0205394 (UMLS CUI [2,3])
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