"I. Neurodegenerative disease "
"II. Possible bvFTD "
"A. Shows progressive deterioration of behaviour and/or cognition by observation or history (as provided by a knowledgeable informant)."
"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]:"
"B. Early apathy or inertia [one of the following symptoms (B.1–B.2) must be present]:"
"C. Early loss of sympathy or empathy [one of the following symptoms (C.1–C.2) must be present]:"
"D. Early perseverative, stereotyped or compulsive/ritualistic behaviour [one of the following symptoms (D.1–D.3) must be present]:"
"E. Hyperorality and dietary changes [one of the following symptoms (E.1–E.3) must be present]:"
"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]:"
"All of the following symptoms (A–C) must be present to meet criteria."
"C. Imaging results consistent with bvFTD [one of the following (C.1–C.2) must be present]:"
"B. Histopathological evidence of FTLD on biopsy or at post-mortem"
"I. Neurodegenerative disease"
"II. Possible bvFTD"
"III. Probable bvFTD"
"IV. Behavioural variant FTD with definite FTLD pathology"
"A. Shows progressive deterioration of behaviour and/or cognition by observation or history (as provided by a knowledgeable informant)."
"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"
"E. Hyperorality 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"
"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)"
"A. Meets criteria for possible or probable bvFTD"
"B. Histopathological evidence of FTLD on biopsy or at post-mortem"
"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"
"A. Early* behavioural disinhibition [one of the following symptoms (A.1–A.3) must be present]:"
"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. 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. 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. 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"
"D.2. Complex, compulsive or ritualistic behaviours D.3. Stereotypy of speech"
"E. Hyperorality and dietary changes [one of the following symptoms (E.1–E.3) must be present]:"
"E. Hyperorality 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. 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"
"F.2. Relative sparing of episodic memory F.3. Relative sparing of visuospatial skills"
"C. Imaging results consistent with bvFTD [one of the following (C.1–C.2) must be present]:"
"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"
Expected headings
"I. Neurodegenerative disease "
"II. Possible bvFTD "
"III. Probable bvFTD"
"IV. Behavioural variant FTD with definite FTLD pathology"
"V. Exclusion criteria for bvFTD"
"A.3. Impulsive, rash or careless actions"
"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.2. Complex, compulsive or ritualistic behaviours D.3. Stereotypy of speech"
"Patients with behavioural variant FTD typically present with a dysexecutive cognitive syndrome associated with changes in personality and social behaviour. These typically include disinhibition, apathy and loss of executive function."