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Wilson's disease and epilepsy.

T R Dening1, G E Berrios, J M Walshe

  • 1Department of Psychiatry, University of Cambridge Clinical School.

Brain : a Journal of Neurology
|October 1, 1988
PubMed
Summary

Epilepsy is ten times more common in Wilson's disease patients than the general population. Direct copper deposition, not pyridoxine deficiency, likely causes seizures in Wilson's disease.

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Area of Science:

  • Neurology
  • Hepatology
  • Genetics

Background:

  • Wilson's disease is a genetic disorder of copper metabolism.
  • Epilepsy is a neurological disorder characterized by recurrent seizures.

Purpose of the Study:

  • To investigate the relationship between Wilson's disease and epilepsy.
  • To determine the prevalence and characteristics of epilepsy in Wilson's disease patients.

Main Methods:

  • Review of 44 literature cases and 14 personal cases of Wilson's disease with epilepsy.
  • Analysis of seizure prevalence in a Cambridge series of 200 Wilson's disease patients.

Main Results:

  • Epilepsy prevalence in the Cambridge series was 6.2%, approximately ten times higher than in the general population.
  • Seizures can occur at any stage of Wilson's disease, often starting after treatment initiation.
  • Seizure prognosis was comparable to idiopathic epilepsy, with 60% seizure-free for 5+ years and 75% for 2+ years.

Conclusions:

  • Epilepsy is significantly more prevalent in Wilson's disease.
  • Direct copper deposition is the likely primary cause of seizures in Wilson's disease.
  • Penicillamine-induced pyridoxine deficiency plays a minor role, if any.

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