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    Primary progressive aphasia remains a diagnostic challenge despite (or even because of) the increasing availability of ancillary tests and biomarkers. We present a 67-year-old man with apparently sporadic logopenic aphasia and positive Alzheimer biomarkers who was subsequently found also to have a pathogenic mutation in the progranulin gene. This was signalled by early atypical features (mild expressive agrammatism and behavioural change, rapid clinical deterioration) around the core logopenic aphasia syndrome. Each of the canonical progressive aphasia syndromes has a 'halo' of less typical variants that may herald alternative or additional pathologies. The accurate diagnosis of primary progressive aphasia depends on careful clinical analysis to direct investigations appropriately. © Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.


    Anthipa Chokesuwattanaskul, Charles R Marshall, Natasja van Harskamp, Henry Houlden, Jonathan D Rohrer, Chris Jd Hardy, Jason D Warren. Primary progressive aphasia: ReADing the clinical GRANularity. Practical neurology. 2022 Dec;22(6):509-514

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    PMID: 35710752

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