Background: Wilson’s disease is an autosomal recessive genetic disorder resulting from an abnormality of copper metabolism. The excessive accumulation of copper in the brain induces an extra-pyramidal syndrome. Oculomotor abnormalities occur in most extra-pyramidal disorders but have rarely been studied in Wilson’s disease.
Objective: To evaluate ocular motility manifestations of Wilson’s disease.
Methods: A prospective study of 34 patients affected by Wilson’s disease who were recruited and whose ocular motility was recorded by electro-oculography (EOG).
Results: Vertical smooth pursuit was abnormal in 29 patients (85%). Vertical opto-kinetic nystagmus and horizontal smooth pursuit were impaired in 14 patients (41 %). No MRI abnormality was found in the lenticular nuclei of 7 patients who manifested ocular motility abnormalities.
Conclusion: Vertical eye movements, in particular vertical pursuits (85.3%), are impaired in Wilson’s disease, more often than vertical optokinetic nystagmus (38.2%) and vertical saccades (29.4%). EOG abnormalities can be found in patients who do not yet exhibit anatomical lesion by MRI.
- ocular motility
- smooth vertical pursuit
- wilson's disease
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