Wilson's disease with myoclonus and white matter lesions |
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| Authors: | Barbosa Egberto Reis Silveira-Moriyama Laura Machado Alexandre Costa Bacheschi Luiz Alberto Rosemberg Sérgio Scaff Milberto |
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| Affiliation: | 1. II Department of Neurology, Institute Psychiatry and Neurology, Warsaw, Poland;2. Department of Experimental and Clinical Pharmacology, Medical University, Warsaw, Poland;3. Department of Neuropathology, Institute Psychiatry and Neurology, Warsaw, Poland;4. Faculty of Chemistry, University of Warsaw, Warsaw, Poland;1. Department of Neurology, Sanjay Gandhi Post Graduate medical Sciences, Lucknow, India;2. Division of Endocrinology, CSIR-Central Drug Research Institute, Lucknow, India;1. II Department of Neurology, Institute Psychiatry and Neurology, Warsaw, Poland;2. Department of Radiology, Institute Psychiatry and Neurology, Warsaw, Poland;3. Department of Experimental and Clinical Pharmacology, Medical University, Warsaw, Poland |
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| Abstract: | White matter lesions (WML) and epilepsy have been occasionally seen in Wilson's disease. No cases of generalized myoclonus have been reported so far. We present a patient with psychiatric symptoms starting at age 16, followed by tremor, generalized dystonia and severe generalized myoclonus. In addition to classical findings, the MRI showed also extensive WML in temporal, parietal and frontal regions, preserving interhemispheric fibers. Necropsy revealed marked alterations of white matter, cortex and basal ganglia. We subsequently review the literature concerning WML and myoclonus in Wilson's disease. |
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