ABNORMAL EYE-MOVEMENTS IN CREUTZFELDT-JAKOB-DISEASE

We report 3 patients with autopsy-proven Creutzfeldt-Jakob disease who , early in their course, developed abnormal eye movements that include d periodic alternating nystagmus and slow vertical saccades. These fin dings suggested involvement of the cerebellar nodulus and uvula, and t he brainstem reticular formation, respectively. Cerebellar ataxia was also an early manifestation and, in 1 patient, a frontal lobe brain bi opsy was normal at a time when ocular motor and cerebellar signs were conspicuous. As the disease progressed, all saccades and quick phases of nystagmus were lost, but periodic alternating gaze deviation persis ted. At autopsy, 2 of the 3 patients had pronounced involvement of the cerebellum, especially of the midline structures. Creutzfeldt-Jakob d isease should be considered in patients with subacute progressive neur ological disease when cognitive changes are overshadowed by ocular mot or findings or ataxia.