Frontotemporal Dementia-Pick's Disease

The International Behavioural Variant FTD Criteria Consortium has developed revised guidelines for the diagnosis of behavioural variant FTD. These criteria show encouragingly high sensitivity and specificity when applied to patients with early-onset dementia and provide a useful tool both for specialist researchers and for general clinicians.
I. Neurodegenerative disease
The following symptom must be present to meet criteria for bvFTD:

• A. Shows progressive deterioration of behaviour and/or cognition by observation or history (as provided by a knowledgeable informant).
  

II. Possible bvFTD
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. Socially inappropriate behaviour
    
  • A.2. Loss of manners or decorum
    
  • A.3. Impulsive, rash, or careless actions
    
• 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 sympathy or empathy (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 perseverative, 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. Hyper-orality 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 memory and visuospatial functions (all of the following symptoms [F.1-F.3] must be present):
  
  • F.1. Deficits in executive tasks
    
  • F.2. Relative sparing of episodic memory
    
  • F.3. Relative sparing of visuospatial skills.
    

III. Probable bvFTD
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. Frontal and/or anterior temporal atrophy on MRI or CT
    
  • C.2. Frontal and/or anterior temporal 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 evidence of FTLD on biopsy or at post-mortem
  
• C. Presence of a known pathogenic mutation.
  

V. Exclusionary criteria for bvFTD
Criteria A and B must be answered negatively for any bvFTD diagnosis. Criterion C can be positive for possible bvFTD but must be negative for probable bvFTD.

• 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.