| Budget Amount *help |
¥1,800,000 (Direct Cost: ¥1,800,000)
Fiscal Year 1998: ¥800,000 (Direct Cost: ¥800,000)
Fiscal Year 1997: ¥1,000,000 (Direct Cost: ¥1,000,000)
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| Research Abstract |
Neurological manifestations in 43 patients of group A xeroderma pigmentosum were investigated. Nineteen of those patients had microcephaly with delay in head growth beginning in late infancy. After 6 years of age , the major neurological features of group A xeroderma pigmentosum developed gradually, including areflexia, sensorineural hearing impairment, and mental retardation. By 10 years of age, about one half of the patients with group A had developed spinocerebellar symptoms, such as nystagmus, ataxia, tremor, and choreoathetosis. The incidence of neurological manifestations increased with age, which is consistent with a chronic progressive degenerative disease of neurons.
Three children with group A xeroderma pigmentosum were investigated in prenary school. Two of them were educated in the special class for handicapped, and the other one in the ordinary school. This research project revealed that in the earlier infancy the patients started special program for this disease in integrated preschool, the better educational results they appreciate.
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