Abstracts

Rationale: Steroids are used to treat a small percentage of pediatric seizures including West, Landau-Kleffner or Lennox-Gastaut syndromes, as well as Rasmussen’s Encephalitis. However, their use in patients where an inflammatory etiology is not identified or suspected has never been systematically studied. The question thus remains as to the therapeutic potential of an anti-inflammatory strategy in pediatric subjects affected by multiple drug resistant seizures. Methods: This retrospective study includes 29 patients, aged less than 18 years, who received at least one steroid course (methyl-prednisolone, ACTH, hydrocortisone or dexamethasone) for the treatment of drug-resistant epilepsy. Twenty patients (32 treatments) were treated for marked worsening of seizure frequency in the week before treatment or ongoing status epilepticus; 9 patients (13 treatments) were treated for epilepsia partialis continua(EPC). The etiology of epilepsy consisted of a broad range of causes spanning from metabolic disorders to cortical dysplasia. Patients’ selection intentionally excluded children affected by epileptic encephalopathies known to respond to steroids (i.e., West, Landau-Kleffner, CSWS, Lennox-Gastaut and related syndromes) as well as patients affected by documented inflammatory disease, such as Rasmussen’s Encephalitis. The treatment was judged as effective when SE ceased, or when seizure were reduced by at least 50%. Assessment of efficacy in EPC was based on motor improvement and EEG polymyographic recordings. Results: Overall, steroid treatments were effective at least once in 25/29 patients. Decreased frequency of seizures, greater than 50% or interruption of status epilepticus was observed after 32 out of 38 steroid treatments. In these patients the treatment at least reversed the clinical worsening that triggered steroid treatement. A clinically significant reduction of EPC resulting in motor functional improvement was observed after 10/13 treatments, and the positive effect persisted after withdrawal in half of the cases. Only in 5% of the patients steroids were withdrawn due to the severity of side effects. Conclusions: Our study demonstrates a dramatic efficacy of steroidal immunomodulators in the treatment of SE in children. Remarkably, this positive outcome appears to be largely independent from the underlying etiology and class of steroids used.