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Brainstem auditory evoked potentials in Wilson's disease.

D Butinar1, J V Trontelj, A J Khuraibet

  • 1Institute of Clinical Neurophysiology, University Medical Center, Ljubljana, Yugoslavia.

Journal of the Neurological Sciences
|February 1, 1990
PubMed
Summary

Brainstem auditory evoked potential (BAEP) studies in Wilson's disease patients show abnormal findings, particularly prolonged NIII-NV intervals, correlating with neurological symptoms. These abnormal BAEPs are not typically an early indicator in Wilson's disease.

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Area of Science:

  • Neurology
  • Neurophysiology

Background:

  • Wilson's disease is a genetic disorder causing copper accumulation, often leading to neurological and hepatic manifestations.
  • Central nervous system involvement is common, necessitating sensitive diagnostic tools.

Purpose of the Study:

  • To evaluate the utility of brainstem auditory evoked potential (BAEP) studies in assessing neurological involvement in pediatric and young adult Wilson's disease patients.
  • To correlate BAEP findings with clinical, neuroradiological, and laboratory data.

Main Methods:

  • Brainstem auditory evoked potential (BAEP) studies were performed on twelve patients (aged 11-25 years) with Wilson's disease.
  • Results were correlated with clinical neurological examinations, CT scans, and copper metabolism tests.

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Main Results:

  • Two neurologically asymptomatic patients and one with mild symptoms had normal BAEPs.
  • Eight of ten patients with significant neurological symptoms showed abnormal BAEPs, characterized by prolonged NIII-NV interpeak intervals.
  • The NIII-NV interval prolongation correlated significantly with the neurological score (r = 0.64, P = 0.001).

Conclusions:

  • Abnormal BAEP findings, specifically prolonged NIII-NV interpeak intervals, are associated with neurological deficits in Wilson's disease.
  • BAEP abnormalities do not appear to be an early diagnostic marker for Wilson's disease.
  • BAEP can be a valuable tool for assessing the extent of central nervous system involvement in Wilson's disease.