Abstract
Acquired forms of gaze-holding nystagmus usually produce centrifugal nystagmus. The authors report about a 63-year-old-patient with a rapidly deteriorating syndrome of cerebellar signs, dementia and myoclonus suggesting Creutzfeldt-Jakob disease (CJD), who developed the unusual sign of bilateral horizontal and vertical centripetal nystagmus with sustained eccentric fixation early in the disease. To our knowledge, this is the first report of centripetal nystagmus in CJD.