Gabapentin can improve dystonia in confirmed Wilson disease.

نویسندگان

  • Margherita Lerose
  • Roberto Di Fabio
  • Mariano Serrao
  • Georgios Loudianos
  • Francesco Pierelli
  • Carlo Casali
چکیده

Wilson disease (WD) is an autosomal recessive disease of copper metabolism due to mutations in the ATP7B gene on chromosome 131. Patients may show a disabling movement disorder characterized by extrapyramidal signs with dystonia and choreoathetosis, associated with psychiatric signs and liver involvement, usually occurring during the first decades of life. Copper chelation by D-penicillamine represents the main therapy in WD. Those patients who experience severe sideeffects due to D-penicillamine may instead be commenced on another chelating agent, such as trientine hydrochloride. Zinc acetate, also accepted for the treatment of WD, especially in presymptomatic stages of the disease, stimulates the induction of the intestinal cell metallothionein, which eventually reduces copper absorption from the intestinal tract and its transport to the liver2. The symptomatic treatment of WD is commonly unsatisfactory, however1,2. We observed a case that is of particular interest, since a young patient with WD showed a striking improvement of axial and segmental dystonia on gabapentin at a low dosage.

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عنوان ژورنال:
  • The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques

دوره 41 2  شماره 

صفحات  -

تاریخ انتشار 2014