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Gerstmann-Sträussler-Scheinker disease is an autosomal dominant prion brain disease that begins during middle age.

GSS occurs worldwide and is similar to but 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.

Last full review/revision December 2009 by Michael Jacewicz, MD

Content last modified November 2005