Ophthalmologic Findings in Wilson's Hepatolenticular Degeneration: With Emphasis on Ocular Motility

Wilson's disease (hepatolenticular degeneration) is a familial disorder of copper metabolism which may be associated with hepatic cirrhosis. Progressive degeneration of the central nervous system characteristically results in muscular rigidity, tremor, dysarthria, dysphagia, hypertonicity and involuntary movements. The basal ganglia are particularly, but not exclusively, affected. The most common ophthalmologic finding in Wilson's disease is the Kayser-Fleischer ring.1-3Other, much less common physical signs include infrequent or absent blinking, jerky oscillations of the eyes, involuntary upward gaze, paresis of upward gaze, and various, nonspecific ophthalmoscopic abnormalities.2-8 Although widespread motor disturbances are characteristic of Wilson's disease, little systematic attention has been paid to the status of ocular motility in this disorder. Interestingly enough, four of Wilson's original six cases were thought to have normal ocular motility, although poor fixation was present in two instances (cases 1 and 3).9Walsh and Ford have noted that extraocular muscle palsies and