title: Ohtahara syndrome or early-onset West syndrome? A case with overlapping features and favorable response to vigabatrin.
abstract: BACKGROUND
  The so called "severe neonatal epilepsies with suppression-burst pattern" include early infantile epileptic encephalopathy, and early myoclonic encephalopathy. Both syndromes are characterized by pharmacoresistant seizures that appear in the first weeks (up to the third month) of life, an electroencephalographic suppression-burst pattern, and a grim prognosis. Many patients later present with other forms of epileptic encephalopathies with difficult-to-treat seizures, such as West syndrome, and those who survive usually suffer from severe neurodevelopmental troubles. We here report the case of a patient who presented at our center with features consistent with a mixed form of these epileptic encephalopathies, and favorable neurodevelopmental evolution.
  
  
  OBJECTIVE
  To draw attention on the potentially favorable effect of vigabatrin in early-onset epileptic encephalopathies.
  
  
  METHODS
  Case study.
  
  
  RESULTS
  In our patient, seizures immediately stopped upon initiation of vigabatrin treatment, and his development and neurological examination at one year are normal.
  
  
  CONCLUSIONS
  Vigabatrin should be considered as an early treatment option in early-onset epileptic encephalopathies.
authors: Pediatric Neurology, Child and Adolescent Department, University Hospitals, Children's Hospital, 6 Rue Willy-Donzé, CH-1211 Geneva 14, Switzerland. christian.korff@hcuge.ch
authors: Christian M
authors: CM
authors: Korff
authors: Serge
authors: S
authors: Vulliemoz
authors: Fabienne
authors: F
authors: Picard
authors: Joel
authors: J
authors: Fluss
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use*; D002220:Carbamazepine / Q000627:therapeutic use; D004351:Drug Resistance; D004569:Electroencephalography; D006801:Humans; D007223:Infant; D008297:Male; D009460:Neurologic Examination; D012640:Seizures / Q000188:drug therapy / Q000209:etiology; D013036:Spasms, Infantile / Q000188:drug therapy*; D013577:Syndrome; D016896:Treatment Outcome; D020888:Vigabatrin / Q000627:therapeutic use*
chemical_list: D000927:Anticonvulsants; D002220:Carbamazepine; D020888:Vigabatrin