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NEUROLOGY 1990;40:1850
© 1990 American Academy of Neurology

Internuclear ophthalmoplegia in the Chiari type II malformation

Anthony C. Arnold, MD, Robert W. Baloh, MD, Robert D. Yee, MD and Robert S. Hepler, MD

Department of Ophthalmology (Drs. Arnold and Hepler), Jules Stein Eye Institute, and the Department of Neurology (Dr. Baloh), Reed Neurological Research Center, University of California, Los Angeles, CA; and the Department of Ophthalmology (Dr. Yee), Indiana University School of Medicine, Indianapolis, IN.

We describe 3 cases of Chiari type II malformation presenting with bilateral internuclear ophthalmoplegia (INO). Although prominent, the INO was not an isolated sign in any of the patients; superimposed abduction paresis was present in 2, and deficits in smooth pursuit, optokinetic nystagmus, and vestibulo-ocular responses were present in 3. Two had hydrocephalus: 1 was clinically unchanged without therapy after 5 years; the other did not improve with shunting. Findings in our 3 patients, along with the 4 previously reported, confirm that INO is 1 manifestation of widespread brainstem or cerebellar dysfunction. Its origin is probably multifactorial, related to hydrocephalus, vascular compromise, direct neuronal distortion, or congenital neural malformation.

Address correspondence and reprint requests to Dr. Anthony C. Arnold, Jules Stein Eye Institute, 100 Stein Plaza, UCLA, Los Angeles, CA 90024-7005.

Supported in part by a development grant from Research to Prevent Blindness, Inc., New York, NY (Dr. Yee).

Presented to the Clinical Eye Movement Society, Neuro-Ophthalmology Congress, Vancouver, BC, Canada, May 7, 1988.

Received February 26, 1990. Accepted for publication in final form May 18, 1990.






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