Internuclear ophthalmoplegia in the Chiari type II malformation.
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.
Arnold
A C
AC
Department of Ophthalmology, Jules Stein Eye Institute, Los Angeles, CA 90024-7005.
Baloh
R W
RW
Yee
R D
RD
Hepler
R S
RS
D000328:Adult; D001139:Arnold-Chiari Malformation / Q000503:physiopathology*; D002675:Child, Preschool; D005133:Eye Movements; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000601:surgery; D008297:Male; D008591:Meningomyelocele / Q000601:surgery; D009886:Ophthalmoplegia / Q000150:complications / Q000503:physiopathology*; D010264:Paraplegia / Q000150:complications; D012438:Saccades