Failure of strychnine treatment during the neonatal period in three Finnish children with nonketotic hyperglycinemia

Abstract

Three Finnish infants with a severe neonatal-onset-type of nonketotic hyperglycinemia were treated with strychnine nitrate in a daily dosage of 0.2 to 0.9 mg/kg, given orally in four doses. In order to lower the plasma and CSF-glycine concentrations concomitant exchange transfusions (200 to 300 ml/kg of heparinized blood) were carried out in two of these infants. Although the strychnine therapy was started at ages 15, 40, and 62 hours, the strychnine produced no clinical effect, and the exchange transfusion caused only a transient decrease in the plasma glycine level. Despite treatment, the clinical course was the same as in the majority of children with the severe form of the disease--all died within the first ten days of life. Impressive effects of strychnine treatment initiated in two infants at ages 5 and 6 1/2 months, and given in addition to sodium benzoate and anticonvulsants, have been reported. These cases, however, probably represent a less severe type of nonketotic hyperglycinemia. Nevertheless, the therapeutic failure in the present cases probably indicates that strychnine treatment does not solve the therapeutic problems of severe forms of NKH.