%0 Case Reports %T Case report: Successful anterior temporal lobectomy in drug-resistant temporal lobe epilepsy associated with Sotos syndrome. %A Favi Bocca L %A Pereira Rodrigues T %A Bortholin T %A Targas Yacubian EM %A Carrete JĂșnior H %A Guaranha M %A Silva Centeno R %J Front Neurol %V 14 %N 0 %D 2023 %M 36970544 %F 4.086 %R 10.3389/fneur.2023.1126327 %X The Sotos syndrome is an autosomal dominant disorder characterized by haploinsufficiency of NSD1 gene, with some individuals affected by epilepsy and, rarely, drug-resistant seizures. A 47-years-old female patient with Sotos syndrome was diagnosed with focal-onset seizures in left temporal lobe, left-side hippocampal atrophy, and neuropsychological testing with decreased performance in several cognitive domains. Patient was treated with left-side temporal lobe resection and developed complete awake seizure control in 3-years of follow-up, with marked improvement in quality-of-life. In selected, clinically concordant patients, resective surgeries may play a significant role in improving patient's quality of life and seizure control.