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Confirming FTD (Diagnostic Criteria)

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bvFTD

An international consortium of experts agreed upon these diagnostic criteria for behavioral variant frontotemporal dementia (bvFTD) in 2010. These criteria employ three levels of diagnostic certainty: possible, probable and definite. The criteria must be met as defined without meeting any of the exclusion criteria.

Neurodegenerative Disease

  1. Shows progressive deterioration of behavior and/or cognition by observation or history (as provided by a knowledgeable informant)

Possible bvFTD

Three of the following behavioral/cognitive symptoms must be present to meet criteria. These symptoms should occur repeatedly, not just as a single instance.

  1. Early behavioral disinhibition (one of the following must be present)
    1. Socially inappropriate behavior: touching or kissing strangers, inappropriate sexual acts, public urination, criminal behavior, etc.
    2. Loss of manners or decorum: examples include
      1. crude or sexually explicit remarks, jokes or opinions which may be offensive to others;
      2. lack of response to social cues (e.g., will continue talking despite other’s attempts to end a conversation);
      3. physical behaviors such as flatulence, touching private body parts, belching or spitting;
      4. poor hygiene or grooming (malodorous, stained, torn or inappropriate clothing),
      5. loss of respect for interpersonal space
    3. Impulsive, rash or careless actions: impulsive behaviors which may or may not be performed in a social context (e.g., new- onset gambling, taking things, stealing (usually food or visually stimulating objects), buying or selling property without regard for consequences)
  2. Early apathy or inertia (one of the following must be present)
    1. Apathy: loss of interest, drive or motivation (e.g., passivity, lack of spontaneity, does not engage in previously rewarding activities)
    2. Inertia: decreased initiation of behavior (e.g., needs prompting to initiate or continue routine activities, less likely to initiate or sustain conversations)
  3. Early loss of sympathy or empathy (one of the following must be present)
    1. Diminished response to other people’s needs and feelings: positive rating of this feature should be based on specific examples that reflect a lack of understanding or indifference to other people’s feelings – e.g., hurtful comments, disregard for other’s pain or distress, making inappropriate jokes
    2. Diminished social interest, interrelatedness or personal warmth: general decrease in social engagement (coldness, lack of eye contact, etc.)
  4. Early perseverative, stereotyped or compulsive/ritualistic behavior (one of the following must be present)
    1. Simple repetitive movements: tapping, clapping, rubbing, scratching, picking at skin or clothing, pursing lips, lip smacking, etc.
    2. Complex, compulsive or ritualistic behaviors: counting and cleaning rituals, collecting, checking, repetitive trips to the bathroom, ordering objects, walking fixed routes, etc.
    3. Stereotypy of speech: repeating words or phrases that have no communicative value
  5. Hyperorality and dietary changes (one of the following must be present)
    1. Altered food preferences: carbohydrate craving (particularly sweets) or food fads
    2. Binge eating, increased consumption of alcohol or cigarettes: patient continues to eat despite satiety (weight gain ____ lb); compulsive smoking, chewing tobacco or gum; or alcohol ingestion
    3. Oral exploration or consumption of inedible objects
  6. Neuropsychological profile: executive/generation deficits with relative sparing of memory and visuospatial functions (all of the following must be present)
    1. Deficits in executive tasks: Trails-B, generation tasks (letter fluency/design fluency) or presence of errors (e.g., perseverations or rule violations)
    2. Relative sparing of episodic memory
    3. Relative sparing of visuospatial skills e.g., ability to copy simple line drawings

Probable bvFTD

  1. Meets criteria for possible bvFTD
  2. Exhibits significant functional decline (by caregiver report or as evidenced by CDR or FAQ scores)
  3. Imaging results consistent with bvFTD (one of the following must be present)
    1. Frontal and/or temporal atrophy on CT or MRI (Disproportionate atrophy in frontal and/or anterior temporal regions)
    2. Frontal hypoperfusion or hypometabolism on SPECT or PET (Disproportionate frontal or frontotemporal hypometabolism or hypoperfusion)

bvFTD with Definite FTLD Pathology

  1. Meets criteria for possible or probable bvFTD
  2. Meets one of the following
    1. Histopathological evidence of FTLD on biopsy or at post-mortem e.g., FTLD-tau, FTLD-TDP, FTLD-UPS, FTLD-IF, FTLD-ni, BIBD
    2. Presence of a known pathogenic mutation e.g., MAPT, PGRN, CHMP2B, VCP

Exclusion Criteria for bvFTD

Criteria 1 and 2 must both be answered negatively for any bvFTD diagnosis. Criterion 3 can be positive for possible bvFTD but must be negative for probable bvFTD.

  1. Pattern of deficits is better accounted for by other non-degenerative nervous system or medical disorders (excludes all bvFTD diagnoses) e.g., delirium, cerebrovascular disease, cerebellar disorder, systemic disorders (e.g., hypothyroidism) or substance-induced conditions
  2. Behavioral disturbance is better accounted for by a psychiatric diagnosis (excludes all bvFTD diagnoses) e.g., depression, bipolar disorder, schizophrenia, pre-existing personality disorder
  3. Biomarkers strongly indicative of Alzheimer’s disease or other neurodegenerative process (excludes diagnosis of probable bvFTD) e.g., genetic mutations, extensive PIB binding, CSF markers
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