strychnine and Diseases-in-Twins

Identical male twins with non-ketotic hyperglycinaemia did not exhibit any development progress while on strychnine treatment. Since other therapeutic attempts with single agents previously had failed in most instances, we tried a combination of various treatment modalities. The results indicated that the administration of glycine receptor antagonist (strychnine) together with a low-protein diet (1.5 gm/kg/day), augmentation of 1-C units (N(5)-formlytetrahydrofolate), and removal of glycine from extracellular fluid (sodium salicylate and sodium benzoate) yielded some clinical improvement and positive biochemical changes in out patients.

Topics: Adrenocorticotropic Hormone; Amino Acid Metabolism, Inborn Errors; Benzoates; Benzoic Acid; Clonazepam; Dietary Proteins; Diseases in Twins; Glycine; Humans; Infant; Infant, Newborn; Leucovorin; Male; Sodium Salicylate; Strychnine

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 6 1/2 months of age.

Topics: Amino Acid Metabolism, Inborn Errors; Clonazepam; Diseases in Twins; Epilepsies, Myoclonic; Female; Glycine; Humans; Infant, Newborn; Male; Pregnancy; Strychnine; Twins, Monozygotic

Strychnine, a potent antagonist of glycine was given to twins suffering from nonketotic hyperglycinemia at age 73 hours. Within hours of the onset of treatment favorable effects were observed such as improvements of muscle tone, movements, defense reactions, and probably breathing. Pyridoxine, N5-formyl-tetrahydrofolate and lipoic acid were given concomitantly with strychnine but failed to alter glycine levels in plasma and cerebrospinal fluid. The therapeutic trial was terminated after 2 1/2 days because success, though considerable, was judged inadequate.

Topics: Diseases in Twins; Electroencephalography; Female; Glycine; Humans; Infant, Newborn; Infant, Newborn, Diseases; Male; Strychnine