Türk Nöroşirürji Dergisi 2016 , Vol 26 , Num 2
A Tuberous Sclerosis Case with Intractable Seizures: Case Report and Literature Review
Fatih YAKAR, Ümit EROĞLU, Onur ÖZGÜRAL, İhsan DOĞAN, Hasan UĞUR, Şükrü ÇAĞLAR
Ankara Üniversitesi, Tıp Fakültesi, Beyin ve Sinir Cerrahisi Anabilim Dalı, Ankara, Türkiye The Tuberous Sclerosis Complex (TSC) is a neurocutaneous hamartamatous syndrome with multiple system involvement. Genetic transmission is thought to occur with the gene loci on the 9th and 16th chromosomes. Adenoma sebaceum, mental retardation and epilepsy constitute the classical triad. TSC is the most common reason of genetically transduced epilepsy in the world. Although the first symptoms are often seizures in infants, these may also occur in the late ages. Epilepsy occurs in 90% and intractable seizures are present in 50-80% of the patients. The first step in the treatment of epilepsy is antiepileptic drugs, but surgery is an option for intractable patients.

In this paper, we present epilepsy surgery for an intractable Tuberous Sclerosis patient and review of the literature. Anahtar Kelimeler : Tuberous sclerosis, Epilepsy, Neurocutaneous syndrome