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

  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 -
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

Date of visit
Descrizione

Date of visit

Alias
UMLS CUI-1
C1320303
Date of visit
Descrizione

date of visit

Tipo di dati

date

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

study id

Tipo di dati

text

Alias
UMLS CUI [1]
C2826693
Visit:
Descrizione

visit name

Tipo di dati

text

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

Mild cognitive impairment (MCI)

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

current MCI

Tipo di dati

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...
Descrizione

cognitive degration

Tipo di dati

boolean

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

psychometric test proving cognitive degradation

Tipo di dati

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
Descrizione

everyday functioning

Tipo di dati

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).
Descrizione

signs of disturbed consciousness

Tipo di dati

boolean

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

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

Tipo di dati

boolean

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

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

Tipo di dati

boolean

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

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

Tipo di dati

boolean

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

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

Tipo di dati

boolean

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

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

Tipo di dati

boolean

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

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

Tipo di dati

boolean

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

normal finding FAQ

Tipo di dati

boolean

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

amnesic MCI single domain

Tipo di dati

boolean

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

amnesic MCI multi domain

Tipo di dati

boolean

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

non-amnesic MCI single domain

Tipo di dati

boolean

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

non-amnesic MCI multi domain

Tipo di dati

boolean

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

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)?
Descrizione

NINCDS/ADRDA-Criteria AD diagnosis

Tipo di dati

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
Descrizione

Clinical probable AD

Tipo di dati

boolean

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

cognitive deficits

Tipo di dati

boolean

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

progressive disturbance of memory

Tipo di dati

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
Descrizione

no consciousness disturbance

Tipo di dati

boolean

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

age at onset

Tipo di dati

boolean

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

other system- or brain diseases

Tipo di dati

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)
Descrizione

progressive deterioration of specific cognitive functions

Tipo di dati

boolean

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

impairment of everyday activities and behavioral change

Tipo di dati

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)
Descrizione

family history of similar disorders

Tipo di dati

boolean

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

cerebral atrophy

Tipo di dati

boolean

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

normal finding or unspecific changes in EEG

Tipo di dati

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
Descrizione

normal finding of cerebrospinal fluid

Tipo di dati

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
Descrizione

diagnosis of Alzheimers disease

Tipo di dati

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
Descrizione

attendant symptoms

Tipo di dati

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
Descrizione

neurological distinctive feature

Tipo di dati

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)
Descrizione

epileptic seizure

Tipo di dati

boolean

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

CT commensurate with age

Tipo di dati

boolean

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

sudden apoplectic onset

Tipo di dati

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
Descrizione

focal neurological signs

Tipo di dati

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
Descrizione

epileptic seizures or gait disturbance

Tipo di dati

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
Descrizione

dementia syndrome with atypical symptomatic

Tipo di dati

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
Descrizione

second systemic or brain disease

Tipo di dati

boolean

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

progressive severe cognitive deficit

Tipo di dati

boolean

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

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?
Descrizione

PCA diagnosis

Tipo di dati

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
Descrizione

insidious onset and gradual progression

Tipo di dati

boolean

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

visual disorders

Tipo di dati

boolean

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

prominent complex visual deficits

Tipo di dati

boolean

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

Balint syndrome

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0270706
Visual agnosia
Descrizione

visual agnosia

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0234502
Dressing apraxis
Descrizione

dressing apraxia

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0234529
Topographic disorientation
Descrizione

topographic disorientation

Tipo di dati

boolean

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

relatively small deficits of memory and word fluency

Tipo di dati

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
Descrizione

relatively good awareness of disability

Tipo di dati

boolean

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

presenile onset

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0750901
B. Alexia
Descrizione

alexia

Tipo di dati

boolean

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

Gerstmann syndrome

Tipo di dati

boolean

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

ideomotor apraxia

Tipo di dati

boolean

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

physical examination

Tipo di dati

boolean

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

impairment of perceptual functions

Tipo di dati

boolean

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

occipitoparietal abnormality

Tipo di dati

boolean

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

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?)
Descrizione

VD diagnoses

Tipo di dati

boolean

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

cognitive impairment

Tipo di dati

boolean

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

and at least two of the following abilities

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0029266
Attention
Descrizione

attention

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0004268
Speech
Descrizione

speech

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0037817
Spatial visualization ability
Descrizione

spatial visualization ability

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0679024
Ability to judge
Descrizione

ability to judge

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0022423
Action ability
Descrizione

action ability

Tipo di dati

boolean

Alias
UMLS CUI [1]
C1820715
Abstraction ability
Descrizione

abstraction ability

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0679195
Motor control
Descrizione

motor control

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0517961
Praxia
Descrizione

praxia

Tipo di dati

boolean

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

everyday activities

Tipo di dati

boolean

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

disturbed consciousness

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0549249
Delirium
Descrizione

delirium

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0011206
Psychosis
Descrizione

psychosis

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0033975
Severe aphasia
Descrizione

severe aphasia

Tipo di dati

boolean

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

sensomotoric disorder

Tipo di dati

boolean

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

systemic or other brain diseases

Tipo di dati

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
Descrizione

cerebrovascular disease

Tipo di dati

boolean

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

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

Tipo di dati

boolean

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

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

Tipo di dati

boolean

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

parietotemporal or temporoparietal association complex

Tipo di dati

boolean

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

watershed infarction

Tipo di dati

boolean

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

basal ganglia and frontal lacuna of white matter

Tipo di dati

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
Descrizione

periventricular white matter lesions

Tipo di dati

boolean

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

bilateral thalamus lesions

Tipo di dati

boolean

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

great vessel lesions

Tipo di dati

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
Descrizione

bilateral lesions of great vessel

Tipo di dati

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
Descrizione

leukoencephalopathy

Tipo di dati

boolean

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

onset of dementia

Tipo di dati

boolean

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

abrupt degeneration of cognitive functions

Tipo di dati

boolean

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

progression of cognitive deficits

Tipo di dati

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
Descrizione

gait disturbance

Tipo di dati

boolean

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

motoric instability and frequent support

Tipo di dati

boolean

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

bladder problem

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0579152
Pseudobulbar paralysis
Descrizione

pseudobulbar paralysis

Tipo di dati

boolean

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

personality disorder or mood alteration

Tipo di dati

boolean

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

abulia

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0919974
Depression
Descrizione

depression

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0011581
Emotional incontinence
Descrizione

emotional incontinence

Tipo di dati

boolean

Alias
UMLS CUI [1]
C1142234
Other subcortical deficits
Descrizione

other subcortical deficits

Tipo di dati

boolean

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

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?
Descrizione

bvFTD diagnosis

Tipo di dati

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
Descrizione

slowly progressing disturbance of behavior or of cognition

Tipo di dati

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
Descrizione

socially inappropriate behavior

Tipo di dati

boolean

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

loss of manners and decency

Tipo di dati

boolean

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

impulsive or reckless behavior

Tipo di dati

boolean

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

apathy

Tipo di dati

boolean

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

inactivity

Tipo di dati

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
Descrizione

reduced sympathy

Tipo di dati

boolean

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

reduced interest in social contacts or human affection

Tipo di dati

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
Descrizione

repetitive movements

Tipo di dati

boolean

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

compulsive or ritualized behaviour patterns

Tipo di dati

boolean

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

stereotypes in language

Tipo di dati

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
Descrizione

disturbed preference in choice of food

Tipo di dati

boolean

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

eating orgy or increased consumption of alcohol or cigarettes

Tipo di dati

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
Descrizione

hyperorality

Tipo di dati

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
Descrizione

disturbance in executive functions

Tipo di dati

boolean

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

performance of episodic memory

Tipo di dati

boolean

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

visuocontructive functions

Tipo di dati

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
Descrizione

FTD diagnosis

Tipo di dati

boolean

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

everyday functioning

Tipo di dati

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
Descrizione

anterior temporal atrophy

Tipo di dati

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
Descrizione

frontal or temporal hypo-perfusion

Tipo di dati

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
Descrizione

FTD criteria fulfilled

Tipo di dati

boolean

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

histopathologic evidence

Tipo di dati

boolean

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

mutation causing the behavioural variant of FTD

Tipo di dati

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
Descrizione

disease pattern better explained by another disease

Tipo di dati

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
Descrizione

behavioral disturbances explained by a psychiatric disease

Tipo di dati

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
Descrizione

biomarkers emphasize Alzheimers disease or another neurodegenarative disease

Tipo di dati

boolean

Alias
UMLS CUI [1]
C0005516
UMLS CUI [2,1]
C0002395
UMLS CUI [2,2]
C0524851
UMLS CUI [2,3]
C0205394

Similar models

CRF Diagnostic Criteria

Name
genere
Description | Question | Decode (Coded Value)
Tipo di dati
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])
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])
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])

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