Abstract
Strychnine has been reported to be effective in the treatment of NKH (Pediat.Res. 11: 1016, 1977). We have also had some success in similarly treating an infant with NKH. This infant is the offspring of a consanguneous marriage and was born after an uneventful fullterm pregnancy. Within the first 48 hours of life he developed lethargy, poor cry, poor suck and hypotonia. At one week of age he became apneic but was resuscitated. Laboratory studies revealed hyperglycinemia with plasma glycine 148 μmoles/dl (nl 32±9), CSF glycine 21.7 μmoles/dl (nl 0.7±0.2) and plasma/CSF glycine 6.8 (nl 30-35). Blood gases, blood ammonia, serum short-chain fatty acids and urine organic acids were normal. Treatment was begun with strychnine sulfate 0.04mg/kg/day. Improvement in tone and activity was noted within 2 days. The dosage of strychnine sulfate was gradually increased to a maintenance of 0.2 mg/kg/day. Subsequent myoclonic seizures and increased tone necessitated reduction in the dosage. Following strychnine treatment plasma and CSF glycine concentrations were lowered to 77.8 μmoles/dl and 12.9 μmoles/dl respectively with continuation of the plasma/CSF glycine at 6.0. It would appear that strychnine may be at least somewhat effective in treating the neonatal form of NKH. The mechanism for this effectiveness could be in a lowering of the glycine accumulation.
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Garcia-Castro, J., Levy, H., Shih, V. et al. 853 NON-KETOTIC HYPERGLYCINEMIA (NKH) TREATED WITH STRYCHNINE. Pediatr Res 12 (Suppl 4), 506 (1978). https://doi.org/10.1203/00006450-197804001-00858
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DOI: https://doi.org/10.1203/00006450-197804001-00858
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