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Glutathione peroxidase deficiency and childhood seizures

G F Weber1, P Maertens, X Z Meng

  • 1Department of Biological Chemistry and Molecular Pharmacology, Harvard Medical School, Boston, MA 02115.

Lancet (London, England)
|June 15, 1991
PubMed

Insights

Children with intractable seizures and infections may have glutathione peroxidase deficiency. Selenium supplementation and stopping anticonvulsants improved their health, suggesting a link between selenium, enzyme activity, and neurological conditions.

Area of Science:

  • Biochemistry
  • Pediatric Neurology
  • Nutritional Science

Background:

  • Intractable seizures and recurrent infections in children can indicate underlying metabolic or nutritional disorders.
  • Glutathione peroxidase (GPx) is a critical antioxidant enzyme, and its deficiency can impair cellular defense mechanisms.
  • Anticonvulsant medications, while necessary for seizure control, can sometimes have complex interactions with metabolic pathways.

Purpose of the Study:

  • To investigate the role of glutathione peroxidase deficiency in pediatric patients presenting with intractable seizures, recurrent infections, and anticonvulsant intolerance.
  • To explore the relationship between intracellular and plasma glutathione peroxidase activity, selenium levels, and clinical outcomes.
  • To assess the efficacy of selenium substitution and discontinuation of anticonvulsant medication in managing these complex pediatric cases.

Main Methods:

  • Case study analysis of four children with severe neurological and infectious symptoms.
  • Biochemical assays to measure intracellular and plasma glutathione peroxidase activity.
  • Assessment of whole blood selenium concentrations.
  • Clinical monitoring of patient status before and after therapeutic interventions.

Main Results:

  • Four children exhibited evidence of glutathione peroxidase deficiency.
  • Two children showed low intracellular GPx activity with normal blood selenium and high plasma GPx.
  • The other two children had low intracellular GPx activity, low plasma GPx, and low selenium levels.
  • Clinical improvement, including resolution of seizures and infections, was observed after discontinuing anticonvulsants and initiating selenium therapy.

Conclusions:

  • Glutathione peroxidase deficiency, potentially linked to selenium status, may contribute to intractable seizures and recurrent infections in susceptible children.
  • Selenium substitution and careful management of anticonvulsant medication can lead to significant clinical improvement.
  • This highlights the importance of considering nutritional cofactor deficiencies in pediatric epilepsy management.

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