Abstracts

We report a young man followed in the Neurology Clinic at the Cincinnati Children[apos]s Hospital Medical Center (CCHMC) for partial epilepsy who developed Landau-Kleffner Syndrome at the age of 14 years. The patient was admitted to our hospital in July 2004 for observation following a seizure characterized by left hand clenching, head shaking and unresponsiveness for one minute. The patient was drowsy but at his mental status baseline following the episode. Further history revealed that the patient had had two other seizures in the 18 month period prior to admission. The patient[apos]s initial EEG revealed frequent high amplitude 2 Hz independent bitemporal spike and wave discharges with no clinical correlate. Neuroimaging was normal. The patient was discharged on valproic acid with close follow-up planned.
Six months later, the family reported a steady decline in school performance and behavior. The EEG showed electrographic seizures, generalized in onset, with a L[gt]R bitemporal predominance. Lamotrigine was added. Nine months after initial admission, specific deficits in receptive and expressive language were noted. An overnight EEG revealed abnormal activity originating in the left temporal region during wakefulness. Continuous spike-wave activity during stage III sleep was noted consistent with electrical status epilepticus of slow wave sleep.
Valium was added to the patient[apos]s regimen. Sixteen days later, the EEG normalized with clinical improvement in language function. Valium was tapered and a subsequent 24 hour EEG was normal. Two years after the initial diagnosis of epilepsy, the patient remains seizure-free on valproic acid and lamotrigine with some persistence of slow expressive speech. To our knowledge, this is the first case of Landau-Kleffner Syndrome in a teenager., , , ,