Suspected effective treatments
Based on open-label, uncontrolled studies
In addition to these medications, other treatments in this group include:
Roger and colleagues28 propose that prolonged corticosteroid therapy initiated at the onset of cryptogenic LGS can yield excellent results. Such therapy carries with it multiple potentially significant side effects, however, and relapse frequently occurs when the drugs are withdrawn.25,50,61–64
At least seven open-label trials have investigated the of adjunctive high-dose IVIG in patients with LGS, with very encouraging results.65,66,69–71 In one review, 30–92% of LGS patients receiving IVIG experienced at least a 50% seizure reduction during treatment.65 Dosing schedules varied between studies.
Two blinded, placebo-controlled studies have been published examining the efficacy of IVIG in children with LGS.72,73 The first study enrolled 10 children, ages 4 to 14 years, in an add-on, placebo- controlled, single-blind study design. Only 2 children showed a response to IVIG (42% and 100% decrease in seizure frequency); the remaining 8 children reportedly were unaffected.72
The second study involved 61 patients with various forms of refractory epilepsy (including LGS and West [infantile spasms]), who participated in a randomized, double-blind, placebo-controlled, dose-ranging (three different doses) trial of IVIG. In the IVIG group, 52.5% of patients had their seizures reduced by more than half, versus 27.8% of patients in the placebo group, but this difference did not reach statistical significance.73
Vigabatrin’s most common are generally related to the central nervous system. They include hyperactivity, agitation, weight gain, drowsiness, insomnia, facial edema, ataxia, stupor, and somnolence.76,79–81 It may exacerbate myoclonic seizures (and even absence seizures in some patients) and has been shown to cause irreversible visual field constriction.75,76,79,82,83
As of 2004, vigabatrin is not available in the United States.
The effectiveness of zonisamide in LGS has been investigated in three small studies. Between 26% and 50% of patients with LGS treated with zonisamide experienced at least a 50% reduction in seizure frequency (Dainippon Pharmaceuticals, Japan, data on file).68,84
No formal, published, open-label studies have investigated the effectiveness and safety of gabapentin, tiagabine, or oxcarbazepine in the treatment of seizures associated with LGS. Single reports have suggested that seizure frequency in patients with LGS may be reduced by:
A number of studies have shown the ketogenic diet to be useful for patients with LGS.61,62,88,89 Response to the diet usually is evident within 1 month of its initiation.50 In a study at the Johns Hopkins Hospital, the atonic or myoclonic seizures in 17 consecutively treated patients with LGS decreased by more than 50% immediately.90 The benefits of the diet can include fewer seizures, less drowsiness, better behavior, and fewer concomitant antiepileptic drugs.50
Among surgical procedures, corpus callosotomy is most often reported to be beneficial for patients with LGS. Focal resection is beneficial only rarely.50 Corpus callosotomy is effective in reducing drop attacks but typically does not appear to be helpful for other seizure types.25,91–93 In general, callosotomy is considered palliative rather than curative. Seizure freedom after this treatment is rare but can occur.51,94
Vagus nerve stimulation (VNS)
In several studies, VNS has been reported to be effective for patients with LGS.95–100 In three early published studies95–97 for which follow-up was as long as 5 years, a total of 13 of 18 (72%) patients with LGS experienced at least a 50% reduction in seizure frequency. In a larger cohort of LGS patients treated with VNS (n = 50), Frost et al.98 reported that the median reduction in total seizures was 42% at 1 month, 58.2% at 3 months, and 57.9% at 6 months.
Adapted from: Glauser TA and Morita DA. Lennox-Gastaut syndrome. In: Devinsky O and Westbrook LE, eds. Epilepsy and Developmental Disabilities. Boston: Butterworth-Heinemann; 2001;65–78.
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