Abstract

CASTANEDA, Marco A et al. Enfermedad de Wilson: forma nueropsiquiátrica dominante presentación de un caso y su interpretación fisiopatológica basada en resonancia magnética del encéfalo. Rev. gastroenterol. Perú [online]. 2002, vol.22, n.1, pp.74-80. ISSN 1022-5129.

This is a presentation of a clinical case of Wilson's disease. The patient is a 26 year old woman who began to evidence psychological symptoms, which were later accompanied by neurological manifestations such as asymmetrical hand tremor, parkinsonism, dystonia and later on, dysphagia and mutism. The ophtalmological examination found a Kayser - Fleischer ring in Descemet's 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. The brain magnetic resonance showed frontotemporal atrophy and a degenerative process at the basal ganglia, cerebellum and brain stem. This information could suggest probable neuropsychiatric physiopathology. The stenosis and intense cervical dysphagia, associated with the crycopharyngeal membrane, has not been mentioned previously.

Keywords : Wilson's Disease; Neuropsychiatric type Cirrhosis.