Non-pharmacological treatment options of drug-resistant epilepsy in subcortical band heterotopia: systematic review and illustrative case

Childs Nerv Syst. 2023 Feb;39(2):451-462. doi: 10.1007/s00381-022-05638-w. Epub 2022 Aug 6.


Objective: Subcortical band heterotopia is a rare X-linked neuronal migration disorder primarily in females often associated with drug-resistant epilepsy. The aim of this study is to review the literature for non-pharmacological treatment options of drug-resistant epilepsy in subcortical band heterotopia.

Material and methods: In accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, we performed a systematic review. Entering the keywords "double cortex," "subcortical band heterotopia," and "subcortical laminar heterotopia," we searched Scopus and PubMed databases. We paid particular attention to type of invasive and non-invasive treatment, radiological presentation, and outcome. We also describe a related case report, managed at Alder Hey Children's Hospital, Liverpool.

Results: The systematic literature review yielded 25 patients with subcortical band heterotopia and drug-resistant epilepsy who underwent non-pharmacological treatment. Including our patient, 26 patients were reported. The patients' mean age at seizure onset was 6.5 years (range 0.2-23) with a female sex predilection (5.25:1). The patients' mean age at invasive or non-invasive treatment was 21.5 years (range 6.5-51). The 26 patients underwent 29 non-pharmacological treatments. Ten patients underwent corpus callosotomy; 8 patients had a formal temporal lobectomy. Three patients had focal cortical resection. Two patients respectively had multiple subpial transections, insertion of a vagal nerve stimulator, or deep brain stimulation of the bilateral anterior nuclei of the thalamus. One patient underwent responsive focal neurostimulation. Another patient had transcutaneous stimulation of the vagal nerve. Sixteen patients reported a reduction or the disappearance of the seizures; 1 patient had no improvement. The outcome of 2 patients was classified class I, of 1 patient class II, of 1 patient class III, and of 5 patients class IV according to the Engel Epilepsy Surgery Outcome Scale.

Conclusion: Mainly corpus callosotomy and formal temporal lobectomy have been performed as non-pharmacological treatment with few cases published overall. Several other invasive procedures and one non-invasive technique are based on case reports. The small number of reported cases prevents drawing a firm conclusion as to which non-pharmacological treatment is the best treatment option for refractive epilepsy in patients with subcortical band heterotopia.

Keywords: Double cortex; Epilepsy; Seizure; Subcortical band heterotopia; Subcortical laminar heterotopia; Treatment.

Publication types

  • Systematic Review
  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Classical Lissencephalies and Subcortical Band Heterotopias*
  • Drug Resistant Epilepsy*
  • Epilepsy* / surgery
  • Female
  • Humans
  • Infant
  • Middle Aged
  • Seizures
  • Thalamus
  • Treatment Outcome
  • Young Adult