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Published on: November 11, 2014

Strychnine treatment attempted in newborn twins with severe nonketotic hyperglycinemia

B Steinmann, R Gitzelmann

    Helvetica Paediatrica Acta
    |January 1, 1979
    PubMed

    Insights

    Strychnine, a glycine antagonist, showed early benefits in nonketotic hyperglycinemia twins, improving tone and movement. However, the treatment was stopped due to insufficient glycine level changes and overall inadequate success.

    Area of Science:

    • Biochemistry
    • Neurology
    • Pediatrics

    Background:

    • Nonketotic hyperglycinemia (NKH) is a rare metabolic disorder.
    • Glycine accumulation in the central nervous system causes severe neurological symptoms.
    • Effective treatments for NKH remain limited.

    Observation:

    • Strychnine, a glycine receptor antagonist, was administered to NKH twins at 73 hours old.
    • Early positive effects on muscle tone, movement, and respiration were noted.
    • Concomitant administration of pyridoxine, N5-formyl-tetrahydrofolate, and lipoic acid did not impact glycine levels.

    Findings:

    • Strychnine demonstrated a rapid, albeit partial, improvement in neurological function in NKH patients.
    • Therapeutic intervention with strychnine did not normalize glycine concentrations in plasma or cerebrospinal fluid.
    • The treatment duration was limited to 2.5 days due to perceived inadequacy of the response.

    Implications:

    • Strychnine may offer symptomatic relief in nonketotic hyperglycinemia, warranting further investigation.
    • Targeting glycine receptors could be a potential therapeutic strategy for NKH.
    • The lack of glycine level normalization highlights the complexity of NKH pathophysiology and treatment.

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