Surgical treatment of autistic epileptiform regression

Abstract

Autistic epileptiform regression (AER), a subtype of epileptic regression syndrome (ERS), is a clinical syndrome characterized by regression of language functions, sociability, and nonverbal communication skills; decline of cognitive skills; the development of stereotyped, repetitive, self-stimulatory behaviors; and, epileptiform brain electrical activity. In this article the authors present the results of a small series of six patients with AER who were surgically treated and have follow-up of at least 15 months. Preoperative diagnostic studies included magnetic resonance imaging (MRI), positron emission tomography (PET) scans, magnetoencephalography (MEG), developmental psychologic evaluations, and video-electroencephalogram (EEG) monitoring with scalp and subdural electrodes. Epileptiform brain electrical activity generator sites were identified in both hemispheres, including in the margin of the Sylvian fissure, in all patients. Multiple cortical subpial transection (MST) was the primary operation for them; it was performed over all areas in which seizure foci were identified. Four patients also underwent minimal cortical resection on MST resistant foci. Follow-up interval is from 15–24 months. There were no complications from surgery. All six patients showed marked improvement in language functions, sociability, repetitive behaviors, and cognitive skills. In addition, of the four patients who had overt clinical seizures, two are seizure-free; one has rare seizures that are much shorter and less complicated than preoperatively; and one has sustained >90% reduction in seizure frequency. The results imply that patients with AER who are medication resistant show marked improvement after the surgical interventions described.