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Gerstmann-Sträussler-Scheinker disease is an autosomal dominant prion brain disease that typically begins during middle age.
GSS occurs worldwide and is about 100-fold less common than CJD. It develops at an earlier age (40 vs 60 yr), and average life expectancy is longer (5 yr vs 6 mo).
Patients have cerebellar dysfunction with unsteady gait, dysarthria, and nystagmus. Gaze palsies, deafness, dementia, parkinsonism, hyporeflexia, and extensor plantar responses are also common. Myoclonus is much less common than in CJD.
GSS should be considered in patients with characteristic symptoms and signs and a family history, particularly if they are ≤ 45 yr. Genetic testing can confirm the diagnosis.
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