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Logopenic primary progressive aphasia — SCE Neurology MCQ

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ModerateCognitive NeurologyLogopenic primary progressive aphasiaSCE Neurology

A 18-year-old man presented with word-finding pauses and impaired sentence repetition. On examination, grammar and motor speech were preserved. Initial investigations showed: MRI showed left temporoparietal atrophy; amyloid PET was positive. What is the most likely diagnosis?

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Correct answer: ALogopenic primary progressive aphasia

Logopenic primary progressive aphasia is the best answer because the vignette describes Logopenic primary progressive aphasia: impaired repetition with pauses suggests logopenic PPA, often Alzheimer pathology. Creutzfeldt-Jakob disease is plausible in a neighbouring presentation, but the chronology, examination or investigation pattern does not match the key discriminator here. Vascular dementia and Corticobasal degeneration cognitive syndrome are less appropriate because they would require different localisation, timing or test findings; Normal pressure hydrocephalus dementia would fit a different syndrome. Clinical pearl: nonfluent PPA has effortful agrammatic speech.

Reference: NICE NG97; consensus criteria for dementia syndromes