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ABBREVIATIONS FAODFatty acid b-oxidation disorders GSDI Glycogen storage disease type I HIns Hyperinsulinism ILAE International League against Epilepsy AIM The aim of the study was to characterize seizures and epilepsy related to hypoglycaemia.METHOD 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 b-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. INTERPRETATION Brief neonatal hyperinsulinaemic hypoglycaemic seizures havecharacteristics 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.Seizures are a common expression of hypoglycaemia that is particularly frequent in the neonatal period and infancy, although they are also occasionally reported in adolescence and adulthood. 1 Because of many confounding factors, mechanisms of brain damage are difficult to determine. Using inborn errors of metabolism as a model, we recently showed that the main determining factors for neurological sequelae are comorbidities (i.e. fever and cardiac/respiratory failure) and prolonged convulsions, independently of the aetiology of hypoglycaemia.2 The type of brain injury is determined not by the aetiology or severity of hypoglycaemia but by age at occurrence. 3In addition to neurological deficits and cognitive troubles, epilepsy is one of the sequelae, and it is most often related to hypoglycaemic brain damage. 4 The characteristics of hypoglycaemic seizures and subsequent epilepsy remain poorly described. The aim of this article is, on the basis of the previously studied cohort, to determine characteristics of first and recurrent seizures, and those of epilepsy following hypoglycaemia. METHODWe analyzed retrospectively the files of the 170 consecutive patients referred to Necker-Enfants Malades Hospital in Paris between 1971 and 2009 for hypoglycaemia caused by metabolic diseases. We selected for the present stu...

SUMMARYPurpose: To determine what epilepsy types occur after herpetic encephalitis and what are the determinant factors for subsequent infantile spasms. Methods: We analyzed retrospectively the clinical history of 22 patients, referred to Necker and Saint Vincent de Paul Hospitals (Paris) through the French pediatric epilepsy network from March 1986 to April 2010 and who developed epilepsy some months after herpetic encephalitis. We focused on seizure semiology with video-electroencephalography (EEG) recording, and on neuroradiology and epilepsy follow-up. Key Findings: Fourteen patients developed pharmacoresistant spasms, and eight developed focal epilepsy, but none had both. The patients who developed spasms were more frequently younger than 30 months at age of onset of epilepsy and had herpetic encephalitis earlier (mean 10.6 months of age) than those who developed focal epilepsy (mean 59.7 and 39.6 months, respectively). Epilepsy follow-up was similar in both groups (8.5 and 11 years, respectively). We found 26 affected cerebral areas; none alone was related to the development of epileptic spasms. Significance: Risk factors to develop epileptic spasms were to have had herpetic encephalitis early (mean 10 months); to be significantly younger at onset of epilepsy (mean 22.1 months); and to have cerebral lesions involving the insula, the hippocampus, and the temporal pole.

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