Atypical benign partial epilepsy: recognition can prevent pseudocatastrophe

To characterize and distinguish atypical benign partial epilepsy of childhood among various epileptic syndromes, we conducted a clinical and electroencephalogram study. Seventeen children with atypical benign partial epilepsy of childhood were followed at our hospital. They all underwent a video/polygraphic study of characteristic daily seizures, facilitating a diagnosis of atypical benign partial epilepsy of childhood. Their clinical and electroencephalogram features were retrospectively analyzed. A video/polygraphic study indicated negative motor seizures including epileptic negative myoclonus, atonic absence seizures, or atonic seizures corresponding to spike-and-wave complexes arising from centro-parieto-temporal regions. Early in the clinical course, these seizures appeared every 4 ± 2 months, and lasted 1-3 months. Interictal sleep electroencephalograms, initially localizing in the centro-parieto-temporal regions, became widespread and displayed continuous, diffuse, spike-and-wave complexes, although the spike-wave index did not exceed 85%. Negative motor seizures responded to ethosuximide, corticotropin, and high-dose steroid, whereas other antiepileptic drugs were much less effective. All patients ultimately entered remission before age 12 years. Patients with atypical benign partial epilepsy of childhood exhibited a characteristic clinical course, and responded favorably to anti-absence treatment. Atypical benign partial epilepsy of childhood should be recognized as a discrete epileptic syndrome. Its early diagnosis leads to the prevention of pseudocatastrophe.