Criteria

A number of classification systems have been developed for the diagnosis of FTD. Widely used criteria are described in detail below; others are:

  • McKhann[82]

  • Lund-Manchester[6]

  • National Institute of Neurological Disorders and Stroke (NINDS).[82]

The Neary criteria for frontotemporal lobar degeneration[6]

The Neary criteria have been widely used for the diagnosis of FTD in practice and research, although most clinicians and researchers now use the international consensus criteria. The Neary criteria emphasise 3 clinical syndromes; within these 3 categories, symptoms defining the syndrome are specified:

  • Frontal dementia syndrome is a disorder of personality, social cognition, and social conduct, characterised by insidious decline in social and personal conduct, emotionality, and insight

  • Progressive aphasia syndrome is characterised by agrammatism, anomia, vacuous speech, and paraphasias, in some combination

  • Progressive associative agnosia syndrome is characterised by loss of face or object recognition.

The diagnosis is excluded by abrupt onset or evidence of cerebrovascular injury.

International consensus criteria for behavioural variant FTD[9]

The International Behavioural Variant FTD Criteria Consortium developed guidelines for the diagnosis of behavioural variant FTD (bvFTD). 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.[10][11][18]

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 carer 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 single-photon emission computerised tomography.

IV. Behavioural variant FTD with definite frontotemporal lobe degeneration (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.

*As a general guideline 'early' refers to symptom presentation within the first 3 years.

Diagnostic and statistical manual of mental disorders, 5th ed., text revision (DSM-5-TR) criteria for major or mild frontotemporal neurocognitive disorder[19][20]

A. The criteria are met for major or mild cognitive disorder.

  1. Major neurocognitive disorder: evidence of significant cognitive decline from a previous level of performance in one or more cognitive domains that interferes with independence in everyday activities. The cognitive deficits do not occur exclusively in the context of a delirium and are not better explained by another mental disorder.

    1. Specify severity:

      1. Mild: difficulties with instrumental activities of daily living (e.g., housework, managing money)

      2. Moderate: difficulties with basic activities of daily living (e.g., feeding, dressing)

      3. Severe: fully dependent.

    2. Specify:

      1. With agitation: If the cognitive disturbance is accompanied by clinically significant agitation

      2. With anxiety: If the cognitive disturbance is accompanied by clinically significant anxiety

      3. With mood symptoms: If the cognitive disturbance is accompanied by clinically significant mood symptoms (e.g., dysphoria, irritability, euphoria)

      4. With psychotic disturbance: If the cognitive disturbance is accompanied by delusions or hallucinations

      5. With other behavioural or psychological disturbance: If the cognitive disturbance is accompanied by other clinically significant behavioural or psychological disturbance (e.g., apathy, aggression, disinhibition, disruptive behaviours or vocalisations, sleep or appetite/eating disturbance)

      6. Without accompanying behavioural or psychological disturbance: If the cognitive disturbance is not accompanied by any clinically significant behavioural or psychological disturbance.

  2. Mild neurocognitive disorder: evidence of modest cognitive decline from a previous level of performance in one or more cognitive domains that does not interfere with independence in everyday activities. Greater effort, compensatory strategies, or accommodation may be required to preserve independence in complex instrumental activities of daily living (e.g., paying bills, managing medications). The cognitive deficits do not occur exclusively in the context of a delirium and are not better explained by another mental disorder.

    1. Specify:

      1. Without behavioural disturbance: If the cognitive disturbance is not accompanied by any clinically significant behavioural disturbance

      2. With behavioural disturbance (specify disturbance): If the cognitive disturbance is accompanied by a clinically significant behavioural disturbance (e.g., apathy, agitation, anxiety, mood symptoms, psychotic disturbance, or other behavioural symptoms).

B. The disturbance has an insidious onset and gradual progression.

C. Either (1) or (2):

  1. Behavioural variant:

    1. Three or more of the following behavioural symptoms:

      1. Behavioural disinhibition

      2. Apathy or inertia

      3. Loss of sympathy or empathy

      4. Perseverative, stereotyped or compulsive/ritualistic behaviour

      5. Hyperorality and dietary changes.

    2. Prominent decline in social cognition and/or executive abilities.

  2. Language variant:

    1. Prominent decline in language ability, in the form of speech production, word finding, object naming, grammar, or word comprehension.

D. Relative sparing of learning and memory and perceptual-motor function.

E. The disturbance is not better explained by cerebrovascular disease, another neurodegenerative disease, the effects of a substance, or another mental, neurological, or systemic disorder.

Probable frontotemporal neurocognitive disorder is diagnosed if either of the following is present; otherwise, possible frontotemporal neurocognitive disorder should be diagnosed:

  1. Evidence of a causative frontotemporal neurocognitive disorder genetic mutation, from either family history or genetic testing

  2. Evidence of disproportionate frontal and/or temporal lobe involvement from neuroimaging.

Possible frontotemporal neurocognitive disorder is diagnosed if there is no evidence of a genetic mutation, and neuroimaging has not been performed.

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