Abstracts

Myoclonic-astatic epilepsy of Doose (MAE) is classified by the International League Against Epilepsy (ILAE) as a specific cryptogenic/symptomatic epilepsy syndrome. Nevertheless, this syndrome is often under recognized and confused with Lennox-Gastaut syndrome. Patients with MAE typically have normal development with an unremarkable examination at seizure onset. Seizures typically begin in the preschool age (one to five years of life). Seizure types include a mixture of generalized seizures including myoclonic-astatic, atonic-astatic, myoclonic, absence, generalized tonic-clonic, and tonic/vibratory seizures. The EEG shows irregular fast spike wave discharges. A monomorphic 4-7 Hz parasagittal rhythm is also seen. Seizures are often difficult to control and prognosis for seizure resolution and cognitive outcome is variable. However, Oguni et al in Neuropediatrics (2002, Jun;33(3):122-32) found that the ketogenic diet has marked efficacy in seizure resolution for patients with MAE. Twenty eight patients placed on the ketogenic diet in the past two years were retrospectively reviewed. Of these 28 patients, 10 had a clinical/electrographic diagnosis of MAE, 16 had a diagnosis of a symptomatic generalized epilepsy (including Lennox-Gastaut Syndrome, periodic spasms, and a diffuse encephalopathy with multifocal seizures), and 2 patients had a cryptogenic localization epilepsy. The patients were identified in the following groups: 1. Excellent outcome (seizure-free or [gt] 90% seizure reduction), 2. Good outcome ([gt] 50% seizure reduction), and 3. Poor outcome ([lt] 50% seizure reduction). The majority of the patients in the [quot]poor outcome[quot] group had no change in seizure frequency. For the 10 patients with MAE, 70% of the patients had an excellent outcome with initiation of the ketogenic diet with 30% having a good outcome. However, of the 16 patient with symptomatic generalized epilepsy, 81% of the patients had a poor outcome to the diet with only 6% with an excellent outcome and 13% with a good outcome. Both patients with cryptogenic localization-related epilepsy had a poor outcome with initiation of the ketogenic diet. Myoclonic-astatic epilepsy of Doose is a poorly recognized epilepsy syndrome with seizures that are often difficult to control. This retrospective review suggests that the ketogenic diet is particularly efficacious treatment in children diagnosed with this particular epilepsy syndrome. The ketogenic diet should be considered early in the course of therapy for children with myoclonic-astatic epilepsy.