Dravet Syndrome Should be Remembered in Vaccine Related Seizures

Abstract

Dravet syndrome is an epileptic encephalopathy that accounts for approximately 1.4% of childhood epilepsies. The first seizure in Dravet syndrome typically occurs in most patients under one year of age, usually in a febrile episode (sometimes post-vaccination) and is also diagnosed as a complicated febrile convulsion. At the onset of the disease electroencephalography and cranial magnetic resonance imaging are expected to be normal. In the follow-up, febrile status epilepticus, afebrile seizures, regression in cognitive and motor development occurs. SCNA1A gene variants can be detected in 75-80% of patients clinically diagnosed with Dravet syndrome. SCN1A gene is a gene involved in the formation of Nav1.1 protein, which encodes sodium channels located in the cell membrane and plays a role in the regular functioning of the nervous system. A patient with recurrent febrile seizures, febrile status epilepticus, febrile seizure after the 3rd dose of mixed vaccine and diagnosed as Dravet syndrome at the age of 8 months was presented and the importance of early diagnosis of Dravet syndrome especially in post-vaccine seizures was emphasized.

Keywords

• Dravet Syndrome
• epilepsy
• SCN1A gene

AŞI İLİŞKİLİ NÖBETLERDE DRAVET SENDROMU AKLA GELMELİDİR

Abstract

Dravet sendromu çocukluk çağı epilepsilerinin yaklaşık %1.4‘ünü oluşturan bir epileptik ensefalopatidir. Dravet sendromunda tipik olarak ilk nöbet, hastaların büyük çoğunluğunda bir yaş altında, ateşli bir atakta-bazen aşılamadan sonra- görülür ve genellikle komplike febril konvülziyon olarak tanı alır. Hastalığın başlangıcında elektroensefalografi ve kraniyal manyetik rezonans görüntüleme normal olarak beklenir. Takipte febril status epileptikus, afebril nöbetler, bilişsel ve motor gelişimde gerileme meydana gelir. Klinik olarak Dravet sendromu tanısı alan hastaların %75-80’inde SCNA1A gen varyantları saptanabilir. SCN1A geni hücre zarında yer alan ve sinir sisteminin düzenli işleyişinde rol oynayan sodyum kanallarını kodlayan Nav1.1 proteininin oluşumunda görevli bir gendir. Tekrarlayan febril nöbetleri, febril status epileptikusu, 3.doz karma aşı sonrası febril nöbeti olan ve 8 aylık iken Dravet sendromu tanısı alan bir hasta sunularak özellikle aşı sonrası nöbetlerde Dravet sendromunun akla gelerek erken tanı konmasının önemi vurgulanmıştır.

Keywords

• Dravet sendromu
• epilepsi
• SCN1A geni

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