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Behavioural variant frontotemporal dementia — SCE Geriatric Medicine MCQ

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HardDementiaBehavioural variant frontotemporal dementiaSCE Geriatric Medicine

A 67-year-old develops rapidly progressive cognitive decline over 8 weeks, startle-sensitive myoclonus and gait ataxia. MRI shows cortical ribboning. Routine metabolic, infectious and autoimmune screening is unrevealing. What is the most appropriate next step?

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Correct answer: B — Discuss urgently with the National CJD Research and Surveillance Unit or National Prion Clinic for specialist diagnostic testing and surveillance

Explanation lettering: C = shown as A · D = shown as B · E = shown as C · A = shown as D · B = shown as E

D is correct: the time course, myoclonus, ataxia and cortical ribboning strongly suggest CJD and warrant prompt discussion with the national specialist service for coordinated diagnosis, surveillance and infection-control advice. A delays a time-critical pathway. B targets an unsuitable mechanism. C routes a rapidly progressive syndrome as ordinary dementia. E starts immunotherapy without the stated evidence and delays prion advice. Referral can occur at suspicion; certainty is not required first.

Reference: UKHSA: CJD guidance, data and analysis: https://www.gov.uk/government/collections/creutzfeldt-jakob-disease-cjd-guidance-data-and-analysis