[Ataxic form of Jakob-Creutzfeldt syndrome]

The authors describe a man aged 43 years with a typical onset of the disease with cerebellar signs and progressive dementia prevailing during a 4 month course of the illness. Neuropathological examination demonstrated subacute spongiform encephalopathy with significant although not overwhelming damage to the cerebellar cortex. The case is an example of the ataxic form of this disease isolated in 1965. In the clinical diagnosis of Creutzfeldt-Jakob disease cerebellar signs may play a similarly important role as myoclonia and characteristic EEG curve.