Temporoparietal Resection in a Patient With Landau-Kleffner Syndrome

Abstract

Landau-Kleffner syndrome (LKS) was originally described by Landau, a neurologist, and Kleffner, a speech pathologist, in 1957 as an epileptic disorder with an acquired aphasia. LKS is a childhood epileptic encephalopathy on the electroclinical spectrum of electrical status epilepticus in sleep (ESES). ESES is an electrographic pattern in which there is significant activation of spike wave discharges throughout slow wave sleep. LKS is an electroclinical syndrome presenting with language regression, seizures, and behavior abnormalities with the electroencephalography (EEG) findings of ESES. Other related syndromes include continuous spike and wave in slow wave sleep (CSWS) and benign childhood epilepsy with centrotemporal spikes (BECTS). In contrast to these other disorders, the primary clinical manifestation of LKS is language regression. Additional clinical findings seen in LKS include attention-deficit disorder, irritability, and autistic-like behaviors. Seizures are present in 70%-80% of children with LKS and typically occur infrequently. The findings on neuroimaging are typically normal in LKS. Although this epileptic encephalopathy resolves spontaneously in adolescence, there is often permanent neurologic sequela, especially if the epilepsy is pharmacoresistant. Numerous therapies have been used in LKS, including antiepileptics such as ethosuximide, valproic acid, and benzodiazepines; corticosteroids; adrenocorticotropic hormone; and immunotherapy. In cases of pharmacoresistance, surgical therapy such as multiple subpial transections has been performed. In children with pharmacoresistant ESES with lateralization, cortical resection has been previously reported but is not commonly used, even in those with lateralized seizure onset.