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Wilson disease: brain pathology.

Aurélia Poujois1, Jacqueline Mikol2, France Woimant1

  • 1French National Reference Centre for Wilson Disease, Neurology Department, Lariboisière Hospital, Paris, France.

Handbook of Clinical Neurology
|April 24, 2017
PubMed
Summary
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Wilson disease (WD) causes brain damage from copper deposition, primarily in the striatum. However, copper levels don't correlate with symptom severity, suggesting other factors contribute to WD neurological issues.

Area of Science:

  • Neurology
  • Pathology
  • Biochemistry

Background:

  • Wilson disease (WD) is characterized by copper deposition in the brain, leading to cellular damage.
  • Striatal lesions are common in neurologically symptomatic WD patients, but lesions can also affect other brain regions.
  • Characteristic cellular changes include proliferation of Alzheimer type II astrocytes and Opalski cells.

Purpose of the Study:

  • To review the neuropathologic findings in Wilson disease.
  • To explore the relationship between copper levels and neuropathologic/neuropsychiatric manifestations.

Main Methods:

  • Review of existing literature and case reports on Wilson disease neuropathology.
  • Analysis of clinicopathologic correlations and cerebral copper content.
Keywords:
ATP7AATP7BATPasesCSFWilson diseaseastrocytesblood–brain barriercopperhepatocerebral degenerationlenticular nucleusmetalneuropathology

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Main Results:

  • WD neuropathology involves lesions in various brain areas, including the pons, midbrain, thalamus, and dentate nucleus.
  • Increased cellularity with Alzheimer type II astrocytes and Opalski cells is observed in WD lesions.
  • Cerebral copper content does not correlate with the severity of neuropathologic abnormalities or neuropsychiatric symptoms.

Conclusions:

  • Neuropathologic damage in Wilson disease is complex and may involve factors beyond direct copper toxicity.
  • Further research is needed to elucidate the precise mechanisms underlying neurological disturbances in WD.