A case of progressive subcortical gliosis presenting clinically as Steele-Richardson-Olszewski syndrome

A patient presenting with the characteristic clinical features of Steele-Richardson-Olszewski syndrome is described, in whom neuropathological examination revealed atypical features, including extensive cortical and subcortical gliosis. The clinical and pathological features are discussed with particular reference to Creutzfeldt-Jacob disease and it is proposed that the case should be classified as progressive subcortical gliosis.