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  Vol. 134 No. 3, March 1980 TABLE OF CONTENTS
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Nonketotic Hyperglycinemia

Effects of Therapy With Strychnine

David Warburton, BSc, MB, MRCP; Robert J. Boyle, MD; John P. Keats, MD; Betty Vohr, MD; Siegfried Peuschel, MD; William Oh, MD

Am J Dis Child. 1980;134(3):273-275.


Abstract

• Nonketotic hyperglycinemia was diagnosed in identical twins with lethargy and respiratory failure in the neonatal period. Therapy with strychnine (0.32 mg/kg/day) resulted in great reductions in CSF and plasma glycine levels and improvement in muscle tone, respiration, and ability to suck. Myoclonic seizures were partially controlled by therapy with clonazepam. Higher dosages of strychnine (up to 2.0 mg/kg/day) were needed to counteract the increased lethargy following administration of clonazepam. At 5 months of age, the twins' developmental performance remained below the 1-month level despite adequate somatic growth. The twins died suddenly of status epilepticus at 61/2 months of age.

(Am J Dis Child 134:273-275, 1980)



Author Affiliations

From the Department of Pediatrics, Women and Infants Hospital of Rhode Island (Drs Warburton, Boyle, Keats, Vohr, and Oh), and Rhode Island Hospital (Dr Peuschel), Brown University Program in Medicine, Providence, RI.


Footnotes

Reprint requests to 50 Maude St, Providence, RI 02908 (Dr Oh).



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