Treatment Of Symptomatic Generalized Epilepsy Lennox Gastaut Syndrome LGS

The classic triad of LGS is frequent seizures with multiple seizure types, interictal slow spike-wave and delayed mental development. The Food and Drug Administration (FDA) has approved felbamate (FBM), LTG and TPM for use in the treatment of LGS. TPM was shown to decrease drop attacks and convulsions when compared with placebo (33% of TPM patients had greater than 50% reduction in seizures, compared with 8% of placebo) [14]. Of patients treated with LTG vs. placebo, 33% vs. 16% had greater than 50% seizure reduction [15]. Patients treated with FBM had a 34% decrease in seizures, compared with a 16% decrease in placebo-treated patients [16].

Other AEDs often used in treatment of LGS, without data from controlled trials, include: VPA, vigabatrin (VGB), ZNS, PB, benzodiazepines and adrenocorticotropic hormone (ACTH) or prednisolone. Additional options include the ketogenic diet, vagus nerve stimulator or corpus callosotomy.

As with other epilepsy syndromes with multiple seizure types, targeting the most disabling seizures is important. In patients with LGS, tonic seizures cause injuries and are often the most disabling. Simple interventions, such as providing a helmet with face protection, can also be helpful.

Figure 5.2 Evaluation of a child with infantile spasms. CMV, cytomegalovirus; CSF, cerebrospinal fluid; IS infantile spasm; LFT, liver function test; MRI, magnetic resonance imaging; PKU, phenylketonuria.

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