PEDIATRICS Vol. 63 No. 3 March 1979, pp. 369-373
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Strychnine Therapy in Nonketotic Hyperglycinemia

Dora Arneson Ph.D.1, Lawrence T. Ch'ien M.D.1, Philip Chance M.D.1, and R. Sidney Wilroy M.D.1

1 Child Development Center and the Department of Pediatrics, University of Tennessee Center for the Health Sciences, Memphis, Tennessee, and the Department of Pediatrics, University of Utah, Salt Lake City

Nonketotic hyperglycinemia is an inborn error of metabolism resulting from a defect in the glycine cleavage enzyme system. It is characterized biochemically by elevated concentrations of glycine in blood, spinal fluid, and urine. Previous therapies which have been directed toward reducing the glycine concentration in plasma and CSF have not been successful in preventing neurological deterioration, which may be the result of the role of glycine as an inhibitory neurotransmitter. Strychnine treatment was initiated because it is a specific antagonist of glycine at postsynaptic membranes. The patient reported here has shown clinical and EEG improvement while taking strychnine in conjunction with sodium benzoate.

Submitted on June 23, 1978
Accepted on September 11, 1978