Abstract

Nonketotic hyperglycinemia (NKG) is a non-acidotic, non-hyperammonemic inborn error of metabolism presenting in the neonatal period. Often babies with NKG present in the first week of life with altered sensorium, seizures, apnea and respiratory failure (1, 2). Definite diagnosis is possible with CSF to glycine ratio and estimation of glycine cleaving enzyme levels in the liver. Here we report Opsoclonus in a baby with NKG.

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