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Behavioural variant frontotemporal dementia — SCE Neurology MCQ

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HardCognitive NeurologyBehavioural variant frontotemporal dementiaSCE Neurology

Over three years, a patient develops fluent but empty speech, severe anomia, loss of single-word comprehension and impaired knowledge of familiar objects. Repetition and grammar are preserved; MRI shows left anterior temporal atrophy. What is the syndrome?

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Correct answer: D — Semantic-variant primary progressive aphasia, in this clinical phenotype

The best answer is “Semantic-variant primary progressive aphasia, in this clinical phenotype”. Loss of word and object meaning with fluent grammatical speech and anterior temporal atrophy defines semantic-variant PPA. “Logopenic-variant primary progressive aphasia” is plausible in a different neurological context, but the decisive findings here argue against it. “Non-fluent agrammatic primary progressive aphasia” is plausible in a different neurological context, but the decisive findings here argue against it. “Typical amnestic Alzheimer disease” is plausible in a different neurological context, but the decisive findings here argue against it. “Behavioural-variant frontotemporal dementia” is plausible in a different neurological context, but the decisive findings here argue against it.

Reference: International consensus classification of primary progressive aphasia: https://pubmed.ncbi.nlm.nih.gov/21325651/