| Summary: | Two distinct patterns of neurological disease have been identified among Cree Indian children in Chisasibi, Nouveau Quebec and among Native Indian infants in Northern Manitoba. Both affect the cerebral white matter. The fifteen patients in the first group may suffer from an autosomal recessive defect causing defective development of cerebral myelin. The eleven patients in the second group have systemic immune dysfunction and meningoencephalitis. Together, these diseases are responsible for over half the deaths of infants and young children in Chisasibi. Inbreeding, and the familial occurrence of cases, suggest a genetic cause in both groups, in whom no gestational or perinatal causes can be identified. The first disease pattern may be due to hypomyelination of the central nervous system. The etiology of the second group may be a genetic susceptibility to viral infection.
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