Neuropediatrics 2006; 37 - THP65
DOI: 10.1055/s-2006-945888

TREATMENT OF INTRACTABLE EPILEPTIC ENCEPHALOPATHIES IN INFANCY

K Sugai 1, Y Fujikawa 1, H Komaki 1, E Nakagawa 1, M Sasaki 1, T Ohtsuki 1
  • 1National Center Hospital for Mental, Nervous and Muscular Disorders National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan

Objectives: Ohtahara syndrome (OS), early myoclonic encephalopathy (EME), migrating partial seizures in infancy (MPSI) and severe myoclonic epilepsy in infancy (SMEI) do not respond to conventional antiepileptic therapies. We studied the best way to treat these disorders.

Methods: Sixteen patients with OS, 6 with EME, 6 with MPSI and 6 with SMEI, with onset at 3 days and 8 months of age, were treated at 2 months and 4 years of age. The therapies which each patient best responded to were selected, and their short-term efficacy during 3 months and long-term efficacy after 1 year from the initiation of the particular therapy were evaluated by seizure reduction rate as follows: 100%(SF), >75%(ME), >50%(EF) and <50%(IE).

Results: In short-term efficacy, OS patients with hemimegalencephaly or large cortical dysplasia obtained SF in 5 cases, ME in 1 case and IE in 1 case by hemispherotomy. OS patients without cortical dysplasia got SF in 2 cases by high-dose PB (H-PB) and 2 cases by VPA+ZNS, and ME in 2 cases by H-PB, 2 cases by H-PB+bromide (Br), and 1 case by VPA+ZNS. EME patients gained SF in 1 case by H-PB and ME in 5 cases by H-PB+Br. MPSI patients reached SF in 3 cases by Br and 2 cases by H-PB+Br, and ME in 1 case by Br. SMEI patients showed SF in 1 case by H-PB, ME in 2 cases by H-PB+Br, 1 case by H-PB and 1 case by clorazepate (CLP), and EF in 1 case by CLP. Long-term efficacy was nearly similar to short-term efficacy except in MPSI patients, and some patients with SF showed developmental improvement.

Conclusion: Intractable epileptic encephalopathies in infancy can be successfully treated by modification of antiepileptic medications or surgery.