Abstracts

Rationale: Cerebral cortical dysplasias constitute a group of disorders of cerebral cortical migration abnormalities that lead to impaired connectivity and an imbalance in the excitatory and inhibitory circuits leading to intractable epilepsy and a constellation of comorbidities such as motor and cognitive difficulties, speech delays, learning disabilities and neurocognitive/neuropsychiatric syndromes. Given the structural cerebral cortical abnormality, cortical dysplasias are usually refractory to medical or dietary treatment modalities and often necessitate epilepsy surgery. Conventional surgical technologies, however, are associated with multiple complications and morbidities. Recently, advent of minimally invasive laser-induced thermal ablation technology has been introduced to epilepsy surgery. Methods: A 15-year-old boy with history of autism spectrum disorder, neurocognitive, personal/social difficulties in the setting of intractable epilepsy due to a left temporal open-lip schizencephaly was investigated with long term video EEG monitoring that revealed partial seizures with confusion, photophobia and alteration of consciousness with involvement of left mid, posterior temporal, mid and posterior inferior temporal, and parietal T7, P7, T9, P9, P3 head regions with relative reduction in radiotracer uptake on PET-CT scan in left temporal regions corresponding to cortical dysplasia. Given the localization of seizures to the region of the cortical dysplasia, inside out sequential laser-induced thermal ablation of the entire grey matter lining the schizencephalic cortical dysplasia was performed from ventricular to pial end. Post procedure MRI confirmed adequate ablation of the entire dysplastic cortex and ensured disconnection of the lesion from the normal cortex. Results: Preoperatively the patient had at least one simple partial seizure weekly; characterized by cessation of activity, hand automatisms, photophobia, confusion and mild alteration of consciousness. He had autistic features, lack of good eye contact, ritualistic and repetitive behavior, obsessive-compulsive symptoms, neurocognitive delays, and learning disability. He returned to his baseline well being within hours after recovery from anesthesia. When he woke up, he was observed to have better eye contact and appropriate interaction with others. He was discharged home in stable condition on post-operative day 2. Despite no change in his anticonvulsant regimen, he has been seizure free over the six months after the laser-ablation procedure. A follow-up comprehensive neuropsychological assessment also revealed data to confirm the aforementioned observations when compared to preoperative assessment. Conclusions: Novel, minimally invasive, MRI-guided laser ablation technology may revolutionize epilepsy surgery with significant elimination of perioperative risks encountered with conventional surgical techniques in patients with cortical dysplasia.