1st Step: Neurodegenerative disease: Shows ❌ of behaviour and/or cognition by observation or history (as provided by a knowledgeable informant).
II. ❌ bvFTLD
Three of the following behavioural/cognitive symptoms (A–F) must be present to meet criteria. Ascertainment requires that symptoms be persistent or recurrent, rather than single or rare events.
A. Early* behavioural disinhibition [one of the following symptoms (A.1–A.3) must be present]:
A.1. ❌
A.2. ❌
A.3. ❌
B. Early apathy or inertia [one of the following symptoms (B.1–B.2) must be present]:
B.1. Apathy
B.2. Inertia
C. Early loss of ❌ [one of the following symptoms (C.1–C.2) must be present]:
C.1. Diminished response to other people’s needs and feelings
C.2. Diminished social interest, interrelatedness or personal warmth
D. Early ❌, stereotyped or compulsive/ritualistic behaviour [one of the following symptoms (D.1–D.3) must be present]:
D.1. Simple repetitive movements
D.2. Complex, compulsive or ritualistic behaviours
D.3. Stereotypy of speech
E. ❌ and dietary changes [one of the following symptoms (E.1–E.3) must be present]:
E.1. Altered food preferences
E.2. Binge eating, increased consumption of alcohol or cigarettes
E.3. Oral exploration or consumption of inedible objects
F. Neuropsychological profile: executive/generation deficits with relative sparing of ❌ [all of the following
symptoms (F.1–F.3) must be present]:
F.1. Deficits in executive tasks
F.2. ❌
F.3. Relative sparing of visuospatial skills
III. Probable bvFTLD
All of the following symptoms (A–C) must be present to meet criteria.
A. Meets criteria for possible bvFTD
B. Exhibits significant functional decline (by caregiver report or as evidenced by Clinical Dementia Rating Scale or Functional Activities Questionnaire scores)
C. Imaging results consistent with bvFTD [one of the following (C.1–C.2) must be present]:
C.1. ❌ on MRI or CT
C.2. ❌ hypoperfusion or hypometabolism on PET or SPECT
IV. Behavioural variant FTD with definite FTLD Pathology
Criterion A and either criterion B or C must be present to meet criteria.
A. Meets criteria for possible or probable bvFTD
B. Histopathological ❌
C. Presence of a known pathogenic mutation
V. ❌ for bvFTLD
Criteria A and B must be answered negatively for any bvFTLD diagnosis. Criterion C can be positive for possible bvFTLD but must be negative for probable bvFTLD
A. Pattern of deficits is better accounted for by other non-degenerative nervous system or medical disorders
B. Behavioural disturbance is better accounted for by a psychiatric diagnosis
C. Biomarkers strongly indicative of Alzheimer’s disease or other neurodegenerative process
Arraste e solte para completar o texto.
Exclusionary criteria
Exclusionary criteria
evidence of on biopsy or at post-mortem
evidence of on biopsy or at post-mortem
Frontal and/or anterior temporal atrophy
Frontal and/or anterior temporal atrophy
Frontal and/or anterior temporal
Frontal and/or anterior temporal
Socially inappropriate behaviour
Socially inappropriate behaviour
Loss of manners or decorum
Loss of manners or decorum
Impulsive, rash or careless actions
Impulsive, rash or careless actions
Hyperorality
Hyperorality
progressive deterioration
progressive deterioration
memory and visuospatial functions
memory and visuospatial functions
Relative sparing of episodic memory
Relative sparing of episodic memory
perseverative
perseverative
sympathy or empathy
sympathy or empathy