Eye movement abnormalities in a family with cerebellar vermian atrophy.
We report the oculographic findings in a family whose members have a dominantly inherited, early onset, non-progressive syndrome which includes spontaneous upbeating nystagmus and mild cerebellar ataxia associated with cerebellar vermian atrophy seen on magnetic resonance scanning. Eye movements recorded with electro-oculography and a magnetic scleral search coil revealed severely impaired horizontal and vertical smooth pursuit, optokinetic nystagmus and visual-vestibular interaction, symmetrical horizontal but asymmetrical vertical vestibulo-ocular reflex, and normal saccades. The midline cerebellum appears to be essential for both horizontal and vertical visual tracking and visual modification of the vestibulo-ocular reflex in man.
D000328:Adult; D001284:Atrophy; D002524:Cerebellar Ataxia / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D002531:Cerebellum / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D004585:Electrooculography; D005133:Eye Movements; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D009759:Nystagmus, Pathologic / Q000235:genetics* / Q000503:physiopathology; D012021:Reflex, Abnormal / Q000503:physiopathology