Creutzfeldt-Jakob's disease presenting with ataxia and a movement disorder

We describe a 62-year-old patient who presented with ataxia and dystonia with mild dementia and was shown at autopsy to have Creutzfeldt-Jakob's disease. Movement disorders may be the presenting feature of Creutzfeldt-Jakob's disease, and this entity should be considered in the differential diagnosis of an otherwise obscure movement disorder even when the typical electroencephalographic pattern is absent.