Vagus nerve stimulation in intractable epilepsy associated with SCN1A gene abnormalities

Stephen P. Fulton, Kate Van Poppel, Amy L. McGregor, Basanagoud Mudigoudar, James Wheless

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Mutations in the SCN1A gene cause a spectrum of epilepsy syndromes. There are 2 syndromes that are on the severe end of this spectrum. The classic severe form, Dravet syndrome, is an epileptic encephalopathy of childhood, causing cognitive decline as well as intractable seizures. Severe Myoclonic Epilepsy of Infancy-Borderline (SMEIB) is a term used to include cases with similar severities as those with Dravet syndrome, but lacking a single feature of classic severe myoclonic epilepsy of infancy. Vagus nerve stimulation is a nonpharmacologic treatment for intractable epilepsy. A retrospective review was conducted of patients with deleterious SCN1A mutations who had vagus nerve stimulation placement for treatment of their intractable epilepsy. These children had onset of their epilepsy between 3 and 29 months of age. Seizure control was assessed 6 months after implantation. Twenty patients are included in the study, with 12 implanted at our institution. Nine of the 12 patients implanted at our institution, who had confirmed pre- and post-implantation seizure assessments, showed improvement in seizure control, which was defined as >50% reduction in generalized tonic-clonic seizures, and 4 of those 12 reported improvement in cognitive or speech development. Seven of the 8 patients not implanted at our institution reported subjective benefit, with 4 relating "marked improvement" or seizure freedom. Vagus nerve stimulation appears to impart a benefit to children with deleterious SCN1A gene abnormalities associated with intractable epilepsy.

Original languageEnglish (US)
Pages (from-to)494-498
Number of pages5
JournalJournal of Child Neurology
Volume32
Issue number5
DOIs
StatePublished - Jan 1 2017

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Vagus Nerve Stimulation
Myoclonic Epilepsy
Seizures
Genes
Epilepsy
Mutation
Brain Diseases
Drug Resistant Epilepsy
Therapeutics

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Vagus nerve stimulation in intractable epilepsy associated with SCN1A gene abnormalities. / Fulton, Stephen P.; Van Poppel, Kate; McGregor, Amy L.; Mudigoudar, Basanagoud; Wheless, James.

In: Journal of Child Neurology, Vol. 32, No. 5, 01.01.2017, p. 494-498.

Research output: Contribution to journalArticle

Fulton, Stephen P. ; Van Poppel, Kate ; McGregor, Amy L. ; Mudigoudar, Basanagoud ; Wheless, James. / Vagus nerve stimulation in intractable epilepsy associated with SCN1A gene abnormalities. In: Journal of Child Neurology. 2017 ; Vol. 32, No. 5. pp. 494-498.
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