How are seizures in Sturge-Weber syndrome (SWS) treated?

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

Because seizures in SWS are typically focal, an antiepileptic medication with efficacy in focal seizures is preferable. Antiepileptic medications include the following:

  • Oxcarbazepine (Trileptal)

  • Carbamazepine (Tegretol)

  • Phenytoin (Dilantin)

  • Lamotrigine (Lamictal)

  • Levetiracetam (Keppra)

  • Valproic acid (Depakote, Depakene, Depacon)

  • Zonisamide (Zonegran)

  • Topiramate (Topamax)

  • Lorazepam (Ativan)

  • Phenobarbital (Luminal, Barbita)

  • Gabapentin (Neurontin)

  • Pregabalin (Lyrica)

  • Tiagabine (Gabitril)

  • Diazepam (Valium)

  • Felbamate (Felbatol)

  • Lacosamide (Vimpat)

  • Clonazepam (Klonopin)

The modified Atkins diet was reported as safe and effective in reducing seizure frequency in 5 children with SWS. [93]

The chance of achieving seizure control with medical therapy in SWS varies. Depending on the series, complete seizure control has been achieved in 10-50% of patients, and refractory seizures occur in 11-83% (see Table 4, below). Results vary by the patient population seen at different centers, with a higher incidence of medical failures reported by surgical centers. However, according to Arizmanoglou, even data from the surgical centers indicate that good seizure control is achieved in one third to one half of the patients seen at these centers. [94]

Table 4. Seizure Control in Sturge-Weber Syndrome (Open Table in a new window)

Study

Complete

Partial

Refractory/No Control

Gilly et al [95]

NA*

NA

37%

Sujanski and Conradi [42]

(adults)

27%

49%

24%

Sujanski and Conradi [24, 42] (all ages)

50%

39%

11%

Pascual-Castroviejo et al [39]

47%

12%

28%

Oakes [38]

10%

NA

83%

Sassower et al [87]

NA

NA

43%

Arzimanoglou and Aicardi [94]

NA

NA

39%

Erba and Cavazzuti [40]

50%

NA

NA

Toronto [90, 96]

NA

NA

32%

*NA = not available


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