Long-term neurological outcome in children with early-onset epilepsy associated with tuberous sclerosis

Raffaella Cusmai, Romina Moavero, Roberta Bombardieri, Federico Vigevano, Paolo Curatolo

Research output: Contribution to journalArticlepeer-review

Abstract

In tuberous sclerosis complex, early seizure onset is associated with high risk of intractable epilepsy and cognitive/behavioral impairment. We retrospectively evaluated the long-term outcome of 44 infants presenting with seizures in the first 12. months who received vigabatrin, and were followed up for at least 3.5. years. At the final evaluation 55% of patients were still having seizures, 80% had intellectual disability, and 30% had autism. Sixty-five percent of children who had been treated earlier with vigabatrin after seizure onset achieved seizure freedom, compared with 24% of subjects who received vigabatrin treatment later (P<0.01). Intellectual disability was present in 61% of the children treated early (group A) and in 100% of the children treated later (group B). Nine percent of group A and 52% of group B had autism (P≈ 0.001).A shorter gap between seizure onset and start of treatment could reduce the risk of epileptic encephalopathy, minimizing the deleterious effect of seizures, but is not able to completely reverse the tuberous sclerosis complex-associated cognitive impairment.

Original languageEnglish
Pages (from-to)735-739
Number of pages5
JournalEpilepsy and Behavior
Volume22
Issue number4
DOIs
Publication statusPublished - Dec 2011

Keywords

  • Epilepsy outcome
  • Intellectual disability
  • Seizures
  • Tuberous sclerosis
  • Vigabatrin

ASJC Scopus subject areas

  • Clinical Neurology
  • Behavioral Neuroscience
  • Neurology

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