Cerebral infarction in a child with congenital adrenal hyperplasia presenting as acute encephalitis syndrome

Abstract

Congenital Adrenal Hyperplasia (CAH) is an inherited abnormality of steroid synthesis. It is usually diagnosed in the early neonatal period. Its association with white matter abnormalities in the developing brain has been reported. Cerebral infarction is one of the rarely associated complications of classical CAH. A 5-year-old child had presented with features of acute onset fever, refractory new-onset seizure, and altered sensorium. He was a known case of CAH, on regular exogenous steroid supplementation. Investigations revealed that he had extensive hemorrhagic cerebral infarction. No underlying infective or vascular cause could be detected to explain the etiology of cerebral infarction in this child. Hence, the effect of CAH on the developing brain and an inadequate escalation of steroid dose during stress have led to the cerebral infarction.