[Early infantile epileptic encephalopathy and glycine encephalopathy]

Rev Neurol. 1997 Dec;25(148):1916-8.
[Article in Spanish]

Abstract

Introduction: Early infantile epileptic encephalopathy (EIEE) with suppression burst activity in EEG (Ohtahara syndrome) is a rare type of epileptic encephalopathy in infancy and represents the earliest type of age-related symptomatic generalized epilepsy. The main etiologic factors associated to EIEE are cerebral dysgenesia and metabolopathies, principally nonketotic hyperglycinemia.

Clinical case: We report a neonate with EIEE secondary to glycine encephalopathy, diagnosed by increased of LCR/plasma glycine index.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Brain Diseases / etiology*
  • Brain Diseases / physiopathology*
  • Electroencephalography
  • Epilepsy, Generalized / diagnosis
  • Epilepsy, Generalized / etiology*
  • Epilepsy, Generalized / physiopathology*
  • Fatal Outcome
  • Female
  • Glycine / cerebrospinal fluid*
  • Humans
  • Infant, Newborn
  • Metabolism, Inborn Errors / cerebrospinal fluid*
  • Metabolism, Inborn Errors / complications*
  • Syndrome
  • Time Factors

Substances

  • Glycine