Congenital adrenal hyperplasia. Report of a case with neurological complications.

Hanefeld, F., Crome, L., France, N. E., and Jackson, A. D. M. (1973). Archives of Disease in Childhood, 48, 554. Congenital adrenal hyperplasia: report ofa case with neurological complications. A girl with congenital adrenal hyperplasia of the salt-losing type was treated with cortisone, desoxycorticosterone acetate (DOCA), and salt. She developed normally up to the age of 10 months, when she went through a short episode of hypernatraemia and hypoglycaemia. After that she presented severe brain damage and died at 14 months. The brain was reduced in weight and showed histologically severe atrophy of the cerebral and cerebellar cortex akin to that of Alpers' disease, with widespread loss of myelin and gliosis of the white matter indistinguishable from that of sudanophil leucodystrophy.

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