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Neuropediatrics 1987; 18(1): 11-12
DOI: 10.1055/s-2008-1052427
© Georg Thieme Verlag KG Stuttgart · New York

Neurological Abnormalities in Wilson's Disease Are Reversible

S.  Lingam , J.  Wilson , H.  Nazer1 , A. P. Mowat2
  • The Hospital for Sick Children, Great Ormond Street, London, WC1, England
  • 1Present address: Faculty of Medicine, University of Jordan, Amman, Jordan
  • 2King's College Hospital, Denmark Hill, London SE5, England
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Publikationsdatum:
19. März 2008 (online)

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Abstract

The therapeutic responses of seven children with Wilson's disease who presented with neurological disease were evaluated. Neurological abnormalities comprised intellectual deterioration in 7, conduct disorder in five, dystonia in three, choreoathetosis in three, seizures in one and hemiparesis in one. Lethargy and weight loss were present for several months in 6 children. Four children had clinically demonstrable liver disease which was fatal in two. Electroencephalography performed in two children was normal. Computed tomography (CT) of the brain in three children showed cerebral atrophy in all and areas of low attenuation in the basal ganglia which resolved on treatment in one. All patients were treated with penicillamine but, in four, methylene tetramine (TETA) was substituted because of adverse effects. Neurological abnormalities in these patients were reversible.

Key words

Wilson's disease - Neurology - Therapeutic response