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Frontotemporal dementia pathology — MRCPsych Paper A MCQ

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EasyNeuropathologyFrontotemporal dementia pathologyMRCPsych Paper A

A 58-year-old woman develops progressive social disinhibition, loss of empathy and a marked preference for sweet foods. Episodic memory is relatively preserved early in the illness. Which neuropathological finding is most likely?

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Correct answer: E — Frontal and anterior temporal atrophy with neuronal loss and tau- or TDP-43-positive inclusions

The correct answer is E. Early disinhibition, loss of empathy, altered food preference and relative preservation of episodic memory indicate behavioural-variant frontotemporal dementia. Its pathological substrate is frontotemporal lobar degeneration, with frontal and anterior temporal neuronal loss, gliosis and atrophy. Most cases are classified by abnormal tau or TDP-43 inclusions; FUS pathology is less common. Medial temporal neurofibrillary tangles and amyloid plaques favour Alzheimer disease, in which early episodic memory impairment is more typical. Prion disease usually progresses rapidly and produces spongiform change. Hypoxic hippocampal sclerosis requires a relevant acute insult rather than progressive behavioural change. Multifocal demyelination would suggest an inflammatory demyelinating disorder and does not explain this characteristic dementia syndrome.

Reference: Mann DMA, Snowden JS. Frontotemporal lobar degeneration: Pathogenesis, pathology and pathways to phenotype. Brain Pathology. 2017;27(6):723–736. https://pubmed.ncbi.nlm.nih.gov/28100023/