3095 Primary non-fluent variant aphasia heralding progressive supranuclear palsy: a word on language-leading presentations of neurodegenerative conditions and diagnostic delay

• Published in: BMJ neurology open Vol. 6; no. Suppl 1; p. A32
• Main Authors: Georgiades, Matthew J, Ahmed, Rebekah
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd 01.08.2024; BMJ Publishing Group LTD; BMJ Publishing Group
• Subjects: Aphasia; Poster Abstracts
• ISSN: 2632-6140
• DOI: 10.1136/bmjno-2024-ANZAN.89

We report a 68 year-old right-handed retired chemical engineer with 12 months of progressive speech and language disturbance. She became increasingly frustrated by effortful pronunciation especially for multisyllabic words, and typographical errors when writing emails. She experienced no memory nor behavioural changes, no visual symptoms and no parasomnias. Her husband noted a new ‘blank stare’, but she did not report any motor symptoms.Examination [SEE VIDEO] revealed masked facies and restricted up-gaze with slow saccades. There was increased blink rate and left facial dystonia. There was subtle parkinsonism with mild left sided finger-tapping bradykinesia with decrement and cogwheeling emergent with co-activation. There was no tremor, no myoclonus and no alien-limb phenomenon. Gait was normal without freezing, and she recovered within 2–3 steps on retropulsion. There were no cerebellar signs. She had a non-fluent aphasia with slow, effortful, fragmented speech but no paraphrasic errors, no anomia and repetition was intact. There were no frontal release signs.MRI demonstrated frontal and temporal atrophy (mostly left). FDG-Neurological PET showed anterior neurodegenerative changes. CSF tau was elevated with normal amyloid. The diagnosis was felt most likely to be Progressive Supranuclear Palsy (PSP) presenting initially with a primary progressive non-fluent variant aphasia.This case adds to the growing recognition of language-leading presentations of neurodegenerative conditions including PSP [1] however the average delay to diagnosis in PSP is still unacceptably more than two years from symptom onset [2].References Peterson KA, et al. J Neurol 2021;268:796–809. Mamarabadi M, et al. Mov Disord Clin Pract 2018;5(6):603–606.