[Wilson's disease: a clinical case is presented with neuropsychiatric dominant form and probable physiopathology interpretation with magnetic resonance of the brain]

Rev Neurol. 2002 Apr;34(8):745-50.
[Article in Spanish]

Abstract

Case report: A clinical case of Wilson's disease is presented. She was a 26 years old woman who began to show psychological symptoms, and later developed neurological signs such as asymmetrical hand tremor, parkinsonism, dystonia and later dysphagia and mutism. The ophthalmological examination found a Kayser Fleischer ring in the Descemet membrane. There was disturbance of copper metabolism documented with reduction of serum ceruloplasmin and increase of the urinary excretion of copper. Cirrhosis was demonstrated through laparoscopy and liver biopsy.

Results: The brain magnetic resonance showed frontotemporal atrophy and a degenerative process at the basal ganglia, cerebellum and brain stem, data which could de used to suggest the probable neuropsychiatric physiopathology. The stenosis and intense cervical dysphagia, associated with crycopharyngeal membrane, has not been mentioned previously.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adult
  • Brain / pathology*
  • Copper / metabolism
  • Eye / pathology
  • Female
  • Hepatolenticular Degeneration / diagnosis*
  • Hepatolenticular Degeneration / pathology
  • Hepatolenticular Degeneration / physiopathology*
  • Humans
  • Magnetic Resonance Imaging
  • Neuropsychological Tests

Substances

  • Copper