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Space-Occupying Lesion – Differential Diagnosis — SCE Neurology MCQ

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HardCerebrovascular DiseaseSpace-Occupying Lesion – Differential DiagnosisSCE Neurology

Rapid cognitive decline, myoclonus and ataxia evolve over eight weeks. MRI shows cortical ribboning and caudate diffusion restriction; CSF RT-QuIC is positive. What is the diagnosis?

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Correct answer: D — Probable sporadic Creutzfeldt–Jakob disease, with these neuroanatomical findings

The best answer is “Probable sporadic Creutzfeldt–Jakob disease, with these neuroanatomical findings”. The rapidly progressive syndrome, characteristic diffusion pattern and positive prion seeding assay fulfil probable sporadic CJD criteria. “Autoimmune limbic encephalitis” can be reasonable in another presentation, but it does not account for the defining feature here. “Dementia with Lewy bodies” can be reasonable in another presentation, but it does not account for the defining feature here. “Wernicke encephalopathy” can be reasonable in another presentation, but it does not account for the defining feature here. “Alzheimer disease” 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