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.