Synopsis
Recognise behavioural and language-led frontotemporal degeneration, distinguish it from psychiatric and Alzheimer presentations, and coordinate genetic, communication, safeguarding and multidisciplinary care.
- Frontotemporal dementia often begins before 65 and presents with progressive personality, behaviour, executive or language change while episodic memory and visuospatial skills can be relatively preserved early.
- Behavioural-variant FTD features early disinhibition, apathy, loss of empathy, stereotyped or compulsive behaviour, hyperorality and executive dysfunction.
- Primary progressive aphasia may be semantic, with loss of word and object meaning, or non-fluent or agrammatic, with effortful speech and impaired grammar or speech planning.
- Obtain collateral history because reduced insight is common and a clinic conversation can underestimate home, financial, sexual, dietary or occupational consequences.
- MRI may show frontal or anterior temporal atrophy, often asymmetric, but diagnosis integrates progression, phenotype, cognitive testing and exclusion of mimics rather than a scan alone.
- Ask about motor neurone disease, parkinsonism and a three-generation family history; a substantial minority of FTD is genetic and counselling should precede predictive testing.
- NICE advises not offering acetylcholinesterase inhibitors or memantine for frontotemporal dementia because these Alzheimer medicines do not treat its core pathology.
- Non-drug care should adapt the environment, preserve routine, support communication, manage eating and risk, and protect carers from escalating burden.
- SSRIs or trazodone are sometimes used off label for selected behaviours, but evidence is limited; define a target, discuss uncertainty and stop when no meaningful benefit occurs.
Key red flags
Fasciculation, weakness, spasticity, dysphagia, parkinsonism, apraxia or impaired vertical eye movement may reveal overlap with motor neurone or atypical parkinsonian degeneration.
Hours-to-days change, impaired attention, altered consciousness, infection, medication toxicity or a new lesion indicates delirium or another emergency rather than uncomplicated neurodegeneration.
Investigation priorities
Establish progressive change in social cognition, language, executive function and everyday risk beyond the clinic snapshot.
Management branches
Personality, conduct or language has changed progressively in adult life.
- Obtain private and joint collateral examples of timing, progression, empathy, inhibition, routines, eating, language, finances, driving and occupational performance.
- Assess attention, mood, psychosis, substance use, sleep and medicines, separating gradual network decline from delirium, primary psychiatric illness and situational conflict.
- Examine cognition, language, eye movements, parkinsonism and upper and lower motor neurone signs, and arrange standard blood and structural imaging assessment.
- Refer to a cognitive-neurology or young-onset dementia service for neuropsychology, biomarker decisions and a formulation shared with the family.