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Epilepsy in children with infantile thiamine deficiency

A Fattal-Valevski1, A Bloch-Mimouni, S Kivity

  • 1Pediatric Neurology Unit, Dana Children's Hospital, Sourasky Medical Center, 6 Weitzman St., Tel Aviv, 64239, Israel. afatal@post.tau.ac.il

Neurology
|July 3, 2009
PubMed

Insights

Severe infantile thiamine deficiency from soy formula caused epilepsy in 7 children. Follow-up shows persistent seizures, developmental delays, and brainstem dysfunction, indicating long-term neurological impact.

Area of Science:

  • Pediatric Neurology
  • Nutritional Neuroscience
  • Developmental Pediatrics

Background:

  • Infantile epilepsy is a significant concern with various etiologies.
  • Thiamine (vitamin B1) is crucial for neurological development.
  • Defective infant formulas can pose serious health risks.

Purpose of the Study:

  • To present follow-up findings in children with epilepsy linked to infantile thiamine deficiency.
  • To describe the long-term neurological outcomes after exposure to a defective soy-based formula.

Main Methods:

  • Retrospective review of medical records for 7 children aged 5-6 years.
  • Analysis of clinical data, electroencephalogram (EEG) tracings, and neuroimaging.
  • Assessment of seizure types, treatment response, and developmental status 5 years post-deficiency.

Main Results:

  • All 7 children experienced seizures (tonic, myoclonic, focal) during infancy.
  • Recurrent seizures, including myoclonic and complex partial, developed in all children.
  • EEG abnormalities evolved, with 3 cases showing hypsarrhythmia; seizures were often refractory to antiepileptic drugs.
  • All children exhibit mental retardation, motor disabilities, and brainstem dysfunction symptoms.

Conclusions:

  • Severe infantile thiamine deficiency is a potential cause of epilepsy.
  • This deficiency can lead to persistent, severe neurological impairment, including epilepsy and developmental deficits.
Abstract

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