Ohtahara syndrome or early-onset West syndrome? A case with overlapping features and favorable response to vigabatrin

Eur J Paediatr Neurol. 2012 Nov;16(6):753-7. doi: 10.1016/j.ejpn.2012.05.009. Epub 2012 Jul 4.


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.

Aims: 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.

MeSH terms

  • Anticonvulsants / therapeutic use*
  • Carbamazepine / therapeutic use
  • Drug Resistance
  • Electroencephalography
  • Humans
  • Infant
  • Male
  • Neurologic Examination
  • Seizures / drug therapy
  • Seizures / etiology
  • Spasms, Infantile / drug therapy*
  • Syndrome
  • Treatment Outcome
  • Vigabatrin / therapeutic use*


  • Anticonvulsants
  • Carbamazepine
  • Vigabatrin