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The Efficacy of Vagus Nerve Stimulation in Intractable Epilepsy Associated With Nonketotic Hyperglycinemia in Two Children

Departments of Pediatric and Neurology, The Ohio State University, Columbus, Ohio

^* To whom correspondence should be addressed. E-mail: changyong.tsao{at}nationwidechildrens.org.

	Abstract

Nonketotic hyperglycinemia is an inborn error of glycine metabolism and these patients frequently suffer from intractable epilepsy despite treatment with sodium benzoate, dextromethophan, and multiple anticonvulsants. We encountered 2 infants with nonketotic hyperglycinemia whose intractable generalized convulsive seizures were difficult to control with sodium benzoate, dextromethophan, and multiple anticonvulsants. However, after the addition of vagus nerve stimulation, their intractable generalized seizures were >75% reduced in frequency, the numbers of multiple anticonvulsants were reduced, and the quality of life significantly improved. The efficacy in seizure reduction persists for at least 3 years in both children.

First published on October 19, 2009
Journal of Child Neurology 2009, doi:10.1177/0883073809336873


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