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Gerstmann-Sträussler-Scheinker Syndrome — SCE Neurology MCQ

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HardNeurodegenerative DiseaseGerstmann-Sträussler-Scheinker SyndromeSCE Neurology

A 55-year-old man presents with rapidly progressive dementia, ataxia, and behavioural change over 8 months. His father died similarly at 52. Genetic testing reveals a P102L PRNP mutation. MRI shows cortical atrophy without typical CJD pattern. What is the diagnosis?

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Correct answer: E — Gerstmann-Sträussler-Scheinker syndrome

GSS is a genetic prion disease (PRNP P102L mutation, autosomal dominant) with slower course than sCJD, prominent cerebellar ataxia and progressive dementia. A: sCJD is not hereditary. C: FFI has D178N-129M mutation with insomnia. D: vCJD is acquired. E: AD is slower with different biomarkers.

Reference: WHO Prion Disease Classification