This protocol covers the management of Lennox-Gastaut syndrome (LGS) that has progressed from a prior epilepsy syndrome — such as infantile epileptic spasms syndrome — in a patient who is not already receiving sodium valproate.
Many patients receive antiseizure therapy to control seizures before a formal LGS diagnosis is established, or develop LGS having progressed over time from another epilepsy syndrome, such as infantile epileptic spasms syndrome (IESS). When such a patient is not already being managed with valproate, a specific transitional pathway is required before the established LGS treatment algorithm is applied.
After appropriate foundational management is established, adjunctive antiseizure therapy becomes a key step when adequate seizure control has not been achieved. The complete protocol — including the specific agents involved, the sequencing of therapy adjustments, and how concurrent medications are managed — is set out in the structured evidence-based regimen below.
DOI: 10.1002/epi4.13075
Patients with LGS that has evolved from another type of epilepsy who are not already being treated with valproate should be transitioned to valproate and then managed using the same algorithm as for newly diagnosed LGS.
Many patients will either have been treated with ASM therapy to initially control seizures before LGS diagnosis or will have developed LGS having progressed over time from another epilepsy syndrome, such as IESS.
If VPA plus LTG does not provide adequate seizure control, RUF should be initiated as adjunctive therapy.
Once RUF has been initiated, attempts should be made to discontinue either VPA or LTG, and, if VPA is discontinued, the LTG dose should be increased.
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