Glutaric aciduria type 1 (GA1) is an autosomal recessive disorder caused by deficiency of glutaryl CoA-dehydrogenase. The metabolism of lysine, hydroxylysine and tryptophan is blocked, leading to accumulation of glutaric acid (GA) and increased urinary concentrations of GA and 3-hydroxy GA. Onset usually involves an acute encephalopatbic episode in a macrocephalic infant, associated with fever. Unlike other organic acidemias, metabolic and lactic acidosis, hyperammonemia and hypogly-caemia are rarely present. Subsequently, there is gross motor delay with marked dystonia-dyskinesia. Subdural haematoma is a rare presenting feature of GA1. We report a case who presented with subdural haematoma and diagnosed as GA1.
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