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Creutzfeldt-Jakob disease — SCE Neurology MCQ

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HardCognitive NeurologyCreutzfeldt-Jakob diseaseSCE Neurology

A patient with rapidly progressive cognitive decline and startle myoclonus has a positive CSF RT-QuIC but no family history. What further action is important after communicating the suspected diagnosis?

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Correct answer: B — Refer to the national prion surveillance and specialist service for confirmation

The best answer is “Refer to the national prion surveillance and specialist service for confirmation”. National specialist involvement supports diagnostic certainty, surveillance, family care and appropriate handling of potentially infectious tissues. “Start acetylcholinesterase inhibitor as disease-modifying therapy” can be reasonable in another presentation, but it does not account for the defining feature here. “Offer brain radiotherapy” can be reasonable in another presentation, but it does not account for the defining feature here. “Reassure that a positive RT-QuIC is a benign ageing marker” can be reasonable in another presentation, but it does not account for the defining feature here. “Begin long-term immunosuppression without reviewing mimics” can be reasonable in another presentation, but it does not account for the defining feature here.

Reference: National CJD Research & Surveillance Unit diagnostic criteria: https://www.cjd.ed.ac.uk/sites/default/files/criteria_0.pdf