Erken cerrahi tedaviden fayda gören bir Sturge - Weber sendromu olgusu

Sturge Weber sendromunda erken infantil dönemde nöbetler görüldüğünde, hemiparezinin erken dönemde geliştiği, nöbetlerin dirençli olabildiği ve mental motor retardasyonun ilerleyici olduğu bilinmektedir. Son zamanlarda bu olgula-ra erken cerrahi müdahale yapılması önerilmektedir. Bu yazıda kompleks parsiyel nöbetler şeklinde dirençli nöbetler-le gelen ve başarılı bir şekilde sol fonksiyonel hemisferektomi yapılan Sturge-Weber sendromlu bir olgu sunulmuştur. 21 aylık kız olgu 2.5 aylıkken başlayan vücudun sağ tarafına lokalize nöbetler nedeniyle hastanemize getirildi. Günde 10 defaya kadar olabilen bu nöbetler ateşli hastalıkları sırasında statusa neden oluyordu. Fizik muayenesinde sol yüz yarısı ve boyunda hemajiomu mevcut olan hastanın sağ hemiparezi ve mikrosefalisi mevcuttu. Beyin manyetik rezo-nans incelemesinde sol serebral hemisfer temporooksipital loblarda anjioma ve gliozis saptandı. Fenobarbital, karbamazepin ve valproat tedavileri maksimum dozlarda kullanılmasına rağmen 6 kez status tablosunda hastaneye yatırılan olguya beyin cerrahisi tarafından sol fonksiyonel hemisferektomi uygulandı. Operasyon sonrası antiepileptik tedavisi azaltılan hasta nöbetsiz olarak izlenmektedir.

A case of Sturge-Weber syndrome who benefit from early surgical intervention

In Sturge-Weber syndrom in case the seizures accompany in early infontile period, the occurrence of earlier hemiparesis, resistant hemiparesis and progressive motor mental retardation is well-established . Recently, surgical intervention has been proposed as a treatment option in such cases. This study presents a Sturge-Weber case who presented with resistant complex partial seizures and underwent a successful left functional hemispherectomy. A 21-month-old girl was presented to our hospital with right-localized seizures the onset of which was 2.5 months of age. The seizures which had occured as many as 10 times a day had caused status in the course of diseases accompanied by high fever. Physical exam revealed right hemiparesis, microcephaly, and a hemangioma located on left side of her face and on her neck. Angioma and gliosis were detected on left cerebral hemisphere temporooccipital lobe by brain magnetic resonance imaging. Although phenobarbital, carbamazepine and valproate had been given at maximal doses, these treatments did not help and the patient was hospitalized for 6 more times with status before she underwent left functional hemispherectomy. The patient has been under follow-up with lower doses of antiepileptics after the surgery and she has not had a seizure since then.

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