Creutzfeldt-Jakob disease — SCE Neurology MCQ

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HardCognitive NeurologyCreutzfeldt-Jakob diseaseSCE Neurology

A 18-year-old man presented with rapid dementia over weeks with myoclonus and ataxia. On examination, there were pyramidal signs and startle-sensitive jerks. Initial investigations showed: MRI showed cortical ribboning; CSF RT-QuIC was positive. What is the most likely diagnosis?

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Correct answer: D — Creutzfeldt-Jakob disease

The best answer is “Creutzfeldt-Jakob disease”. UK stroke guidance supports urgent vascular imaging and reperfusion selection, short-course dual antiplatelet therapy for eligible minor stroke or high-risk TIA, decompression for life-threatening swelling, and secondary prevention matched to mechanism. The alternatives “Wernicke encephalopathy”, “Logopenic primary progressive aphasia”, “Alzheimer's disease”, “Behavioural variant FTD” are clinically adjacent possibilities, but they do not match the defining chronology, localisation, physiology, investigation result or UK management sequence in this stem.

Reference: National Clinical Guideline for Stroke: acute care: https://www.strokeguideline.org/chapter/acute-care/