Elif Yüksel KARATOPRAK, Sema SALTIK, Bülent TAŞEL

Tüberoskleroz kompleksi tanılı hastalarda epilepsinin özellikleri ve klinik seyri

Amaç: Tüberoskleroz kompleksinde en sık görülen nörolojik sorun epilepsidir. Bu çalışma, epileptik tüberoskleroz kompleksi tanılı hastaların epilepsi özeliklerini ve klinik seyrini incelemek, epilepsi seyrini etkileyebilecek etmenleri araştırmak amacıyla planlandı. Gereç ve Yöntem: Çocuk Nörolojisi Kliniği’mizde epilepsi nedeniyle 9 ay-10 yıl izlenen, 1-14 (7,5±4,2 ) yaşları arasında 21 tüberoskleroz kompleksi tanılı hasta (11/10: E/K) çalışmaya alındı. Hastalara ait aşağıdaki bilgiler elde edildi: epileptik nöbet özellikleri, muayene bulguları, zeka veya bilişsel gelişim düzeyleri, antiepileptik tedavi, kraniyal manyetik rezonans görüntüleri ve elektroansefalogram bulguları. Epileptik nöbet seyri iyi ve kötü olarak gruplandırıldıktan sonra ilk nöbet yaşı, öyküde status, infantil spazm varlığı, ilk tedavi seçeneğinin vigabatrin olması, ilk elektroansefalogram bulguları, elektroansefalogramda epileptik aktivite yeri, hastalarda otizm veya zeka ve bilişsel gelişim geriliği varlığı, kortikal tüberlerin sayı ve yerleşimleri, astrositom varlığı açısından istatiksel olarak karşılaştırıldı. Bulgular: Hastaların nöbet başlama yaşı üç gün ile 2,5 yaş arasında olup 16 hastada (%76) bir yaş ve altındaydı. Hastalarımızın en sık nöbet tipi (%95) kısmi nöbet olup, sekizer hastada (%38) infantil spazm ve status epileptikus saptandı. İzlemleri sonucunda, nöbet seyri 11’inde kötü, 10’unda iyi olarak değerlendirildi. Nöbet başlangıç yaşı (bir yaş altı ve üstü), status veya infantil spazm öyküsü, ilk elektroansefalogram bulguları, ilk ilaç olarak vigabatrin kullanılması, hastada otizm veya zeka ve gelişim geriliği varlığı, kraniyal manyetik rezonans görüntülemede kortikal tüber sayısı, yerleşimi ve astrositom varlığının nöbet seyri ile ilişkisi saptanmadı (p>0,05). Çıkarımlar: Tüberoskleroz kompleksi tanılı hastalarda epilepsinin klinik seyrinin her hasta özelinde çeşitli etmenlere bağlı değişkenlik gösterebilmesi nedeniyle bu hastaların klinik ve elektroansefalogram ile yakın izlemi gereklidir.

Characteristics and the clinical course of epilepsy in patients with tuberous sclerosis complex

Aim: Epilepsy is the most common neurological problem in tuberous sclerosis complex (TSC). The aim of this study is to investigate the clinical features and prognosis of epilepsy associated with TSC and to determine the factors that may affect the course of epilepsy in TSC patients. Material and Method: Our study included 21 TSC patients (11/10: M/F) aged between 1-14 years (7.5±4.2 ) and followed up for 9 months-10 years because of epileptic seizures. After epileptic seizures were classified as seizures with good and poor prognosis, they were were statistically compared in terms of age at seizure onset, history of status and infantile spasms, initial treatment with vigabatrin, initial EEG findings, presence of autism or mental retardation or cognitive impairment, the number of cortical tubers and presence of astrocytoma. Results: The age at seizure onset ranged between 3 days and 2.5 years. The most common seizure type in our patients was partial seizure and eight patients had infantile spasms and status epilepticus. As a result of follow-up, the prognosis was evaluated as poor in 11 and good in 10 patients. No statistical relationship was found between seizure prognosis and age at seizure onset (younger and older than 1 age), the history of status and infantile spasms, initial EEG findings, use of vigabatrin as the first drug, the presence of autism or mental retardation or cognitive impairment, the number and localization of cortical tubers and the presence of astrocytoma (p>0.05). Conclusions: The prognosis of epilepsy in patients with TSC varies in each patient depending on several factors. For this reason close follow-up of these patients with clinical evaluation and EEG is required.

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