Lennox Gastaut Sendromu
Özet
Lennox Gastaut Sendromu (LGS) dirençli nöbetler, ciddi nörolojik sekel ile seyreden morbiditesi ve mortalitesi yüksek bir epileptik ensefalopatidir. Prognozda infantil spazm öyküsü, semptomatik etiyoloji, şiddetli bilişsel işlev bozukluğu ve 3 yaşından önce nöbet başlangıcı önemlidir. Hipoksik doğum en önemli önlenebilir faktördür. Başlangıçta tüm kriterler var olmadığından erken tanı zor olabilir. Nöbetlerin gelişmekte olan beyinde hasarı artırdığı da göz önüne alındığında LGS'nin erken tanı ve tedavisi ile prognoza olumlu katkı sağlanabilir. LGS'de nöbetler dirençli seyrettiğinden tedavi yaklaşımı; hastalığın evresi, bilişsel durum ve kliniğine göre belirlenebilir.
The Lennox Gastaut syndrome (LGS) is an epileptic encephalopathy with high morbidity and mortality, which progresses with refractory seizures and severe neurological sequels. History of infantile spasm, symptomatic etiology, severe cognitive dysfunction and age <3 years at onset of seizures are important factors in the prognosis. Hypoxic delivery is the most important, preventable factor in LGS. Early diagnosis can be difficult since all criteria may not be present at onset of disease. Given that seizures increase damage in developing brain, early diagnosis and appropriate treatment may have positive effect on prognosis in LGS. As seizures are refractory in LGS, management should be determined according to disease stage, cognitive state of patient and clinical presentation.
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