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.