Case report: Successful anterior temporal lobectomy in drug-resistant temporal lobe epilepsy associated with Sotos syndrome

The Sotos syndrome is an autosomal dominant disorder characterized by haploinsufficiency of 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-sid...

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Bibliographic Details
Published in:Frontiers in neurology 2023-03, Vol.14, p.1126327
Main Authors: Favi Bocca, Leonardo, Pereira Rodrigues, Thiago, Bortholin, Thiago, Targas Yacubian, Elza Márcia, Carrete Júnior, Henrique, Guaranha, Mirian, Silva Centeno, Ricardo
Format: Article
Language:English
Subjects:
epilepsy surgery
hippocampal sclerosis
Neurology
outcome
Sotos syndrome
temporal lobe epilepsy
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Summary:The Sotos syndrome is an autosomal dominant disorder characterized by haploinsufficiency of 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.
ISSN:1664-2295
1664-2295
DOI:10.3389/fneur.2023.1126327