What is the role of surgery in the treatment of seizures in Sturge-Weber syndrome (SWS)?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Answer

Surgical options are available for seizures refractory to medical treatment, especially for focal seizures. [105] Erba and Cavazzuti estimated that 40% of patients with SWS could become epilepsy surgery candidates, excluding those with either good seizure control or bilateral disease. [40]

Surgical procedures include focal cortical resection, hemispherectomy, corpus callosotomy, and vagal nerve stimulation (VNS). [4] SWS is considered one of the catastrophic epilepsies, which, according to Holmes, result in poor seizure control and developmental outcome if not controlled early. However, criteria for medical intractability should be fulfilled before considering surgery. [106]

Early surgery has been advocated specifically in SWS to improve outcome and prevent refractory seizures, developmental delay, and hemiparesis. In the era prior to modern neuroimaging, Alexander and Norman suggested exploratory craniotomy and lobectomy if the diagnosis was confirmed, even before seizures started, because they believed that early onset seizures were associated with mental retardation. [107]

Hoffman et al and Ogunmegan et al later advocated early hemispherectomy for seizures. [96, 108]

Erba and Cavazzuti recommended surgery when seizures, as well as other neurologic events, such as headaches or mild head trauma, are associated with functional neurologic deficits. The presence of such deficits indicates an impairment in cortical perfusion. [40]

Arzimanoglou and Aicardi preferred to treat seizures initially with antiepileptic drugs (AEDs), no matter what the age of onset, and recommended surgery when seizures are intractable or when evidence of progressive cortical damage is noted. The appropriate surgical procedure would be determined individually by clinical course, EEG, and neuroimaging. [94, 109]

Overall, while some variations exist in the criteria, most studies recommend early surgery with difficult-to-control seizures and a progressive clinical course, determined on an individual basis, as noted above.


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