title: Seizures and epilepsy in hypoglycaemia caused by inborn errors of metabolism.
abstract: OBJECTIVE
  The aim of the study was to characterize seizures and epilepsy related to hypoglycaemia.
  
  
  METHODS
  We analyzed the files of 170 consecutive patients referred for hypoglycaemia (onset 1h to 4y) caused by inborn errors of metabolism (glycogen storage disease type I, fatty acid β-oxidation disorders, and hyperinsulinism).
  
  
  RESULTS
  Ninety patients (42 males and 48 females; 38 neonates and 52 infants/children) had brief hypoglycaemic seizures (68%) or status epilepticus (32%). Status epilepticus occurred earlier (mean 1.4d) than brief neonatal seizures (4.3d, p=0.02). Recurrent status epilepticus followed initial status epilepticus and was often triggered by fever. Epilepsy developed in 21 patients. In 18 patients, epilepsy followed hypoglycaemic status epilepticus and began with shorter delay when associated with grey matter lesions (1.9mo, standard error of the mean [SEM] 1mo) than with white matter damage (3.3y [SEM 1y], p=0.003). Three patients with hyperinsulinism developed idiopathic epilepsy following brief neonatal seizures.
  
  
  CONCLUSIONS
  Brief neonatal hyperinsulinaemic hypoglycaemic seizures have characteristics of idiopathic neonatal seizures. Neonatal status epilepticus should be prevented by the systematic measurement of glucose blood level. Recurrent seizures never consist of status epilepticus when following brief initial seizures. Epilepsy is symptomatic of brain damage with shorter delay in the case of grey rather than white matter lesions, except in a few idiopathic cases in which epilepsy and hyperinsulinism may share a common genetic background.
authors.affiliation: Inserm U1129, Paris, France; CEA, Gif-sur-Yvette, France; Paris Descartes University, Paris, France.
authors.forename: Svetlana
authors.initials: S
authors.lastname: Gataullina
authors.forename: Pascale
authors.initials: P
authors.lastname: Delonlay
authors.forename: Eric
authors.initials: E
authors.lastname: Lemaire
authors.forename: Nathalie
authors.initials: N
authors.lastname: Boddaert
authors.forename: Christine
authors.initials: C
authors.lastname: Bulteau
authors.forename: Christine
authors.initials: C
authors.lastname: Soufflet
authors.forename: Gemma Aznar
authors.initials: GA
authors.lastname: Laín
authors.forename: Rima
authors.initials: R
authors.lastname: Nabbout
authors.forename: Catherine
authors.initials: C
authors.lastname: Chiron
authors.forename: Olivier
authors.initials: O
authors.lastname: Dulac
mesh_terms: D000293:Adolescent; D001786:Blood Glucose; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004827:Epilepsy / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications* / Q000453:epidemiology; D006801:Humans; D006946:Hyperinsulinism / Q000150:complications* / Q000453:epidemiology; D007003:Hypoglycemia / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008661:Metabolism, Inborn Errors / Q000150:complications* / Q000453:epidemiology; D012189:Retrospective Studies; D012640:Seizures / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D013226:Status Epilepticus / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D013997:Time Factors
chemical_list: D001786:Blood Glucose