Evoked potentials in assessment and follow-up of patients with Wilson's disease

Lancet. 1990 Oct 20;336(8721):963-4. doi: 10.1016/0140-6736(90)92419-i.

Abstract

Treatment of 9 patients with Wilson's disease was prospectively studied with evoked potentials and magnetic resonance imaging (MRI). Oral penicillamine therapy led to a decrease in auditory brainstem (ABP) and somatosensory (SEP) conduction times in 6 and 4 neurologically symptomatic patients, respectively. ABP and SEP were normal in 3 other symptom-free patients. MRI showed cerebral lesions in 4 of 7 patients. Quantified indices of brain atrophy were unaffected by treatment. ABP and SEP may reveal a reversible component of the disease that cannot be detected by MRI, and may be a more sensitive measure of treatment efficacy.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Administration, Oral
  • Adult
  • Brain Chemistry
  • Child
  • Copper / analysis
  • Evaluation Studies as Topic
  • Evoked Potentials, Auditory, Brain Stem / drug effects*
  • Evoked Potentials, Somatosensory / drug effects*
  • Female
  • Follow-Up Studies
  • Hepatolenticular Degeneration / drug therapy
  • Hepatolenticular Degeneration / physiopathology*
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Monitoring, Physiologic
  • Penicillamine / administration & dosage
  • Penicillamine / therapeutic use
  • Prospective Studies
  • Time Factors

Substances

  • Copper
  • Penicillamine