[A nation-wide survey for neurologic and hepato-neurologic type of Wilson disease: clinical features and hepatic copper content].
A nation-wide survey for Wilson disease was performed from 1990 to 1991. We studied clinical features and copper content in liver for the neurologic and hepato-neurologic types. A questionnaire was sent to more than five thousand hospitals in Japan. Thirty-three percent of physicians completed the questionnaire. Four hundred and twenty-five cases were studied for the onset age, primary symptoms, prognosis and hepatic copper content. The onset age of neurologic and hepato-neurologic type of Wilson disease was usually 6 years or older. The most common initial symptom was dysarthria. Gait disturbance, flapping tremor and Kayser-Fleischer rings were also very common symptoms. We conclude that in patients with dysarthria and/ or extra pyramidal symptoms over 6 years of age, Wilson disease should be considered. The prognosis quod vitam of patients with neurologic and hepato-neurologic Wilson disease is not always fatal. However, many patients required prolonged treatment at either a hospital, sanatorium or at home due to irreversibility of their severe neurological defects. This result shows that early detection is the most important factor for a promising prognosis. Copper content in liver was examined for each type of Wilson disease. Neurologic type of Wilson disease had the highest copper content, followed by hepato-neurologic type. Hepatic type had the lowest copper level out of these three forms of the disease. The mechanism of onset for each type of Wilson disease should be studied using these results.
Shimizu
N
N
Second Department of Pediatrics, Toho University School of Medicine.
Suzuki
M
M
Yamaguchi
Y
Y
Aoki
T
T
Matsuda
I
I
Arima
M
M
D000293:Adolescent; D000328:Adult; D000743:Anemia, Hemolytic / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003300:Copper / Q000032:analysis*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000378:metabolism; D006801:Humans; D008099:Liver / Q000737:chemistry*; D008107:Liver Diseases / Q000209:etiology; D008297:Male; D009422:Nervous System Diseases / Q000209:etiology; D011379:Prognosis; D011795:Surveys and Questionnaires
D003300:Copper