An ataxic form of subacute presenile polioencephalopathy (Creutzfeldt-Jakob disease)

In 1955 Foley and Denny-Brown reported three cases of subacute progressive encephalopathy occurring in middle age, and leading to death within 13 months. The outstanding clinical features, in order of appearance, were: ataxia of cerebellar type, with dysarthria; involuntary rhythmic jerking move- ments; dementia; stupor, progressing to coma; and finally, a state of generalized muscular rigidity, in which the involuntary movements tended to dis- appear.