Frontotemporal dementia (FTD), also known as frontotemporal lobar degeneration (FTLD), or less commonly, Pick's disease, is one of the most common causes of dementia in adults younger than 60 years. FTD is actually an umbrella clinical term that encompasses a group of neurodegenerative diseases (behavioural-variant frontotemporal dementia, non-fluent variant primary progressive aphasia, and semantic-variant primary progressive aphasia). Individuals with behavioural variant may have disinhibited behavior, hyperorality, lack of empathy, impaired executive function, and lack of sympathy; Loss of insight is one of the core features of the frontal/behavioural variant frontotemporal dementia (FTD). Those with primary progressive aphasia will have progressive deficits in speech, grammar, word output, semantic knowledge, or naming.
The criteria are met for major or mild neurocognitive disorder.
The disturbance has insidious onset and gradual progression.
Either (1) or (2);
3
or more of the following behavioural symptoms:Relative sparing of learning and memory and perceptual-motor function.
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:
Possible frontotemporal neurocognitive disorder is diagnosed if there is no evidence of a genetic mutation, and neuroimaging has not been performed.
Individuals with behavioral-variant frontotemporal dementia may present with apathy or disinhibition. Patients typically lose interest in socialization, self care, and personal responsibilities, or display socially inappropriate behaviours, such as sexual disinhibition. Insight is usually impaired as well. Cognitive impairment is much less prominent during the early stages, and few deficits are found on cognitive testing.
BEACH
can be used to remember the core symptoms of behavioural variant of FTD
B
- Behavioural disinhibitionE
- Empathy decreaseA
- ApathyC
- CompulsionsH
- HyperoralityThe International Behavioural Variant FTD Criteria Consortium (FTDC) developed revised guidelines for the diagnosis of bvFTD in 2011 that are more comprehensive than the DSM-5.[4]
The following symptom must be present to meet criteria for bvFTD:
3
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.
1
of the following symptoms (A1–A3) must be present]:1
of the following symptoms (B1–B2) must be present]:1
of the following symptoms (C1–C2) must be present]:1
of the following symptoms (D1–D3) must be present]:1
of the following symptoms (E1–E3) must be present]:all
of the following symptoms (F1–F3) must be present]:
All
of the following symptoms (A–C) must be present to meet criteria.
1
of the following (C1–C2) must be present]:Criterion A and either criterion B or C must be present to meet criteria.
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.
Frontotemporal dementia can be classified into different clinical variants or subtypes:[5]
A CT head or MRI head can show patterns of atrophy of the frontal lobes that can aid in the clinical diagnosis. Neuroimaging is also helpful to exclude other differential diagnoses such as mass lesions and hydrocephalus.
Variant | Neuroimaging Findings |
---|---|
Behavioral variant | Both frontal lobes (in particular the medial frontal lobes) and anterior temporal lobes have atrophy |
Semantic variant | Middle, inferior, and anterior temporal lobes are atrophic bilaterally but asymmetrically, with the left side usually being more affected |
Logopenic variant | Associated with predominantly left posterior perisylvian or parietal atrophy |
Nonfluent variant | Associated with predominantly left posterior frontal-insular atrophy |
Guideline | Location | Year | Website | |
---|---|---|---|---|
Canadian Consensus Conference on the Diagnosis and Treatment of Dementia (CCCDTD) - Diagnosis and Treatment | Canada | 2020 | - | Link |
CCCDTD - Pharmacological Recommendations for Symptomatic Treatment of Dementia | Canada | 2012 | - | Link |
Deprescribing.org (Bruyère Research Institute) and University of Sydney Deprescribing Guidelines | International | 2018 | - | Link |
National Institute for Health and Care Excellence (NICE) | UK | 2018 | - | Link |
American Psychiatric Association (APA) | USA | 2007, 2014 | - | • Guideline (2007) • Guideline Watch (2014) • Quick Reference |
Guideline | Location | Year | Website | |
---|---|---|---|---|
British Columbia Best Practice Guideline for Accommodating and Managing BPSD | Canada | 2012 | Link | Link |
Canadian Consensus Conference on the Diagnosis and Treatment of Dementia (CCCDTD) | Canada | 2020 | - | Link |
Canadian Coalition for Seniors' Mental Health (CCSMH) Assessment and Treatment of Mental Health Issues in Long-Term Care | Canada | 2006, 2014 | • 2006 Guideline • 2014 Update | Link |
National Institute for Health and Care Excellence (NICE) | UK | 2018 | - | Link |
Treatment of Inappropriate Sexual Behavior in Dementia | UK | 2016 | - | Link |
American Psychiatric Association (APA) | USA | 2016 | - | Link |
Deprescribing.org (Bruyère Research Institute) and University of Sydney Deprescribing Guidelines | International | 2018 | - | Link |