Epilepsy secondary to tuberous sclerosis: Lessons learned and current challenges

Romina Moavero, Caterina Cerminara, Paolo Curatolo

Research output: Contribution to journalArticle

Abstract

Background: In tuberous sclerosis complex (TSC), a substantially increased risk of developing epilepsy is present as a result of a disruption of a TSC gene expression in the brain and secondary abnormal cellular differentiation, migration, and proliferation. Dysregulated excitation probably has its roots in the disruption of GABAergic interneuron development. There is an age-dependent electroclinical expression of seizures, and epilepsy is often quite severe and unremitting. Discussion: The majority of patients (>60%) who are candidates for surgery remain seizure-free after tuberectomy. During the recent years technical advances in the localization of the epileptogenic zone during the recent years have lead to a 63% of Engel class I status after surgery compared with a previous 52%. In medically refractory patients not suitable for surgery, vagus nerve stimulation has proved efficacy in significantly reducing seizure frequency in more than 50% of cases. New evidence suggests that mTOR inhibitors may be helpful in the management of intractable epilepsy for individuals with TSC.

Original languageEnglish
Pages (from-to)1495-1504
Number of pages10
JournalChild's Nervous System
Volume26
Issue number11
DOIs
Publication statusPublished - Nov 2010

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Keywords

  • Antiepileptic drugs
  • Epilepsy
  • Neurological outcome
  • Surgery
  • Tuberous sclerosis

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

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