Creutzfeldt-Jakob disease — MRCPsych Paper A MCQ

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EasyNeuropathologyCreutzfeldt-Jakob diseaseMRCPsych Paper A

A 64-year-old man develops rapidly progressive cognitive decline over several weeks, accompanied by myoclonus and gait ataxia. EEG and cerebrospinal fluid investigations support a diagnosis of sporadic Creutzfeldt-Jakob disease. Which neuropathological finding is most characteristic?

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Correct answer: C — Spongiform change with deposition of abnormal prion protein

Explanation lettering: E = shown as B · B = shown as E

C is correct. Sporadic CJD typically presents with rapidly progressive cognitive decline, myoclonus and cerebellar ataxia. Its hallmark neuropathology is spongiform vacuolation of grey matter, accompanied by neuronal loss, gliosis and deposition of misfolded abnormal prion protein. A describes perivenular inflammatory demyelination, associated with acute disseminated encephalomyelitis rather than CJD. B is characteristic of motor neurone disease and would principally cause lower motor neurone weakness. D is the classical Alzheimer disease pattern, which usually produces a much slower cognitive decline. E suggests dementia with Lewy bodies, where cognitive fluctuations, recurrent visual hallucinations and parkinsonism are more characteristic. The rapid course with myoclonus and ataxia strongly discriminates CJD.

Reference: Zerr I et al. Creutzfeldt-Jakob disease and other prion diseases. Nature Reviews Disease Primers. 2024. https://pubmed.ncbi.nlm.nih.gov/38424082/