Severe Methylenetetrahydrofolate Reductase Deficiency, Methionine Synthase, and Nitrous Oxide — A Cautionary Tale

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Abstract
In this issue of the Journal, Selzer et al. (pages 45–50) describe the unexpected neurologic deterioration and death of an infant boy who had been anesthetized twice within a short time with the widely used anesthetic nitrous oxide. Postmortem studies of his cultured fibroblasts established a diagnosis of 5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency, an inherited defect in folate metabolism. The severe form of deficiency seen in this patient occurs only rarely. The authors mention that patients with mild abnormalities of the folate cycle as well as those with severe abnormalities are likely to be exposed to nitrous oxide during their lifetime, . . .

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