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Abnormal Eye Movements in Creutzfeldt-Jakob DiseaseWe 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 one 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.
Document ID
19970032368
Document Type
Reprint (Version printed in journal)
External Source(s)
Authors
Grant, Michael P. (Case Western Reserve Univ. Cleveland, OH United States)
Cohen, Mark (Case Western Reserve Univ. Cleveland, OH United States)
Petersen, Robert B. (Case Western Reserve Univ. Cleveland, OH United States)
Halmagyi, G. Michael (Royal Prince Alfred Hospital Sydney, Australia)
McDougall, Alan (Royal Prince Alfred Hospital Sydney, Australia)
Tusa, Ronald J. (Johns Hopkins Medical Institutions Baltimore, MD United States)
Leigh, R. John (Case Western Reserve Univ. Cleveland, OH United States)
Date Acquired
August 17, 2013
Publication Date
August 1, 1993
Publication Information
Publication: Annals of Neurology
Volume: 34
Issue: 2
Subject Category
Aerospace Medicine
Report/Patent Number
NAS 1.26:204180
NASA-CR-204180
Funding Number(s)
CONTRACT_GRANT: NIH-EY-06717
CONTRACT_GRANT: NIH-AG-08992
CONTRACT_GRANT: NAG9-571
CONTRACT_GRANT: NIH-EY-09289
Distribution Limits
Public
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Public Use Permitted.
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