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Sporadic Creutzfeldt-Jakob Disease — SCE Neurology MCQ

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ModerateNeurodegenerative DiseaseSporadic Creutzfeldt-Jakob DiseaseSCE Neurology

A 58-year-old woman presents with rapidly progressive dementia, myoclonus, visual disturbance, and ataxia over 4 months. EEG shows periodic sharp wave complexes. MRI brain shows cortical ribboning with high signal on DWI and FLAIR in the caudate and putamen bilaterally. CSF 14-3-3 protein is positive and RT-QuIC is positive. What is the most likely diagnosis?

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Correct answer: D — Sporadic Creutzfeldt-Jakob disease

Rapidly progressive dementia with myoclonus, cerebellar ataxia, visual symptoms, characteristic EEG (periodic sharp wave complexes), MRI findings (cortical ribboning on DWI, caudate/putamen high signal), positive CSF 14-3-3 protein and positive RT-QuIC assay is diagnostic of sporadic CJD. RT-QuIC has >90% sensitivity and >99% specificity for prion disease. A: AD progresses much more slowly. B: Autoimmune encephalitis can cause RPD but would have antibodies and respond to treatment. D: Hashimoto encephalopathy would show thyroid antibodies and respond to steroids. E: Neurosyphilis has different CSF and imaging findings.

Reference: WHO CJD Diagnostic Criteria; EAN RPD Guidelines (2019)