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Abnormal eye movements in Creutzfeldt–Jakob disease
Author(s) -
Grant Michael P.,
Cohen Mark,
Petersen Robert B.,
Halmagyi G. Michael,
McDougall Alan,
Tusa Ronald J.,
Leigh R. John
Publication year - 1993
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410340215
Subject(s) - nystagmus , ataxia , cerebellum , brainstem , eye movement , reticular formation , cerebellar ataxia , medicine , autopsy , neurology , frontal lobe , psychology , neuroscience , pathology , audiology , central nervous system
We report 3 patients with autopsy‐proven Creutzfeldt–Jakob disease who, early in their course, developed abnormal eye movements that included periodic alternating nystagmus and slow vertical saccades. These findings suggested involvement of the cerebellar nodulus and uvula, and the brainstem reticular formation, respectively. Cerebellar ataxia was also an early manifestation and, in 1 patient, a frontal lobe brain biopsy 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 persisted. At autopsy, 2 of the 3 patients had pronounced involvement of the cerebellum, especially of the midline structures. Creutzfeldt‐Jakob disease should be considered in patients with subacute progressive neurological disease when cognitive changes are overshadowed by ocular motor findings or ataxia.