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Frontotemporal Dementia — MRCPsych Paper B MCQ

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ModerateOld Age PsychiatryFrontotemporal DementiaMRCPsych Paper B

A 60-year-old woman has an 18-month history of progressive personality and behavioural change. Her husband describes tactless comments, impulsive spending, reduced empathy, loss of initiative and increasingly rigid daily routines. She can no longer manage the accounts at work. Neuropsychological assessment shows marked executive dysfunction, with relatively preserved episodic memory and visuospatial function. MRI shows disproportionate bilateral frontal and anterior temporal lobe atrophy without significant cerebrovascular disease. Which diagnosis is most likely?

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Correct answer: A — Frontotemporal dementia (behavioural variant)

The diagnosis is behavioural-variant frontotemporal dementia. The early dominant syndrome comprises progressive disinhibition, apathy, loss of empathy, stereotyped or compulsive behaviour and executive dysfunction, with relative preservation of episodic memory and visuospatial function. Functional decline plus characteristic frontal and anterior temporal atrophy supports probable bvFTD. Typical Alzheimer's disease is usually dominated initially by episodic memory impairment. Vascular dementia would be supported by cerebrovascular disease, focal neurological features or a temporal relationship to vascular events. Dementia with Lewy bodies more typically causes cognitive fluctuations, recurrent visual hallucinations, REM-sleep behaviour disorder and spontaneous parkinsonism. Normal pressure hydrocephalus is associated with gait disturbance, urinary dysfunction and ventriculomegaly rather than focal frontotemporal atrophy.

Reference: Rascovsky K et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011;134:2456-2477. https://pubmed.ncbi.nlm.nih.gov/21810890/