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Ocular motility and Wilson’s disease: A study on 34 patients
  1. I Ingster-Moati (isabelle.moati{at}wanadoo.fr)
  1. Université Paris 7, Hôpital Necker, Assistance Publique-Hôpitaux de Paris, France
    1. E Bui Quoc
    1. Hôpital Necker, Assistance -Publique Hôpitaux de Paris, France
      1. M Pless
      1. Massachusetts General Hospital 55 Fruit Street Boston MA 02114, United States
        1. R Djomby
        1. Hôpital Lariboisière, Assistance Publique- Hôpitaux de Paris 2, France
          1. C Orssaud
          1. Hôpital Necker, Assistance -Publique Hôpitaux de Paris, France
            1. J P Guichard
            1. Hôpital Lariboisière, Assistance -Publique Hôpitaux de Paris, France
              1. F Woimant
              1. Assistance Publique - Hôpitaux de Paris, France

                Abstract

                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.

                • EOG
                • ocular motility
                • smooth vertical pursuit
                • wilson's disease

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