[Schizophrenia-like symptoms in the Westphal-Strümpell variation of Wilson disease].
Wilson's disease is a rare, autosomal recessive disorder of copper metabolism due to low serum ceruloplasm, resulting in increased copper deposition, especially in the liver and basal ganglia in the brain. The pseudosclerotic type of Wilson's disease, also known as the Westphal-Strümpell form, is distinguished by positional tremor, ataxia and dysarthria as the main symptoms. We use the example of a 23-year-old patient whose neurological symptoms were preceded by a long history of a schizophrenic-like disorder. Clinical symptoms are presented. MRI, SPECT and PET images are illustrated. Therapy and outcome are discussed.
Müller
J
J
Universitätsnervenklinik Psychiatrie-Psychotherapie, Homburg/Saar.
Landgraf
F
F
Trabert
W
W
D000328:Adult; D014151:Anti-Anxiety Agents / Q000008:administration & dosage / Q000009:adverse effects; D014150:Antipsychotic Agents / Q000008:administration & dosage / Q000009:adverse effects; D001480:Basal Ganglia Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D001569:Benzodiazepines; D001712:Biperiden / Q000008:administration & dosage / Q000009:adverse effects; D001928:Brain Diseases, Metabolic / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D004359:Drug Therapy, Combination; D004401:Dysarthria / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D006527:Hepatolenticular Degeneration / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D019965:Neurocognitive Disorders / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D010396:Penicillamine / Q000008:administration & dosage; D012559:Schizophrenia / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D013132:Spinocerebellar Degenerations / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*
D014151:Anti-Anxiety Agents; D014150:Antipsychotic Agents; D001712:Biperiden; D001569:Benzodiazepines; D010396:Penicillamine