Mehmet CANPOLAT, Alper ÖZCAN, Hatice Gamze POYRAZOĞLU, Sefer KUMANDAŞ, Hüseyin PER, Hakan GÜMÜŞ, Abdulhakim ÇOŞKUN, Ali YIKILMAZ

Sturge-Weber sendromu; klinik ve radyolojik değerlendirme

Bu çalışmada Sturge-Weber sendromu (SWS) tanısı ile izlenen olguların klinik ve radyolojik olarak değerlendirilmesi amaçlandı. SWS tanısı konulan 11 olgunun klinik özellikleri, nörolojik ve göz muayene bulguları, elektroensefalografi ve kranial görüntüleme bulguları hasta takip dosyalarından geriye dönük olarak incelendi. Çalışma için Erciyes Üniversitesi Tıp Fakültesi etik kurul onayı (07.08.2012/486) alındı. Çalışmaya ortalama yaşları 61.82 ± 39.73 arasında değişen (16-132 ay) 11 olgu alındı. Olguların başvuruda en sık gözlenen yakınmaları, konvülsiyon ve yüzde leke idi. Porto şarabı lekesi tüm olgularda gözlendi. Epilepsi, psikomotor gerilik, hemiparezi ve glokom en sık gözlenen bulgulardı. Beyin Tomografisinde (BT) 6 olguda kalsifikasyon saptandı. Kranial manyetik rezonans görüntülemede (MRG), 9 olguda serebral atrofi, 7 olguda leptomeningeal anjiyoma, 5er olguda kalvaryal kalınlaşma ve genişlemiş koroid pleksus, 3er olguda da koroid pleksus kisti ve venöz anormaliler saptandı. Porto şarabı lekesi, epilepsi, hemiparezi, psikomotor gerilik, glokom, kortikal kalsifikasyon, serebral atrofi ve leptomeningeal anjiyoma en sık saptanan bulgulardı.

Sturge-Weber syndrome: clinical and radiological evaluation

In this study,we aimed to evaluate the clinical and neuroimaging features in children with Sturge-Weber syndrome (SWS). Eleven patients with SWS were included in this study. Chart analysis, clinical evaluation, neurological and ophthalmological examinations, electroencephalographic and neuroimaging studies were evaluated retrospectively. The study approved by the Erciyes University Faculty of Medicine Ethics committee (07.08.2012/486). The mean age was 61.82 ± 39.73 months (range from 16 to 132 months ). The most common symptoms were convulsion and facial angioma. Port-wine stains was observed in all cases. Epilepsy, hemiparesis, psychomotor retardation and glaucoma were the most common issues. Cortical calcifications on cranial tomography (CT) scan was present in 6 cases. On magnetic resonance imaging (MRI) of cranial, there were cerebral atropy in 9 cases, leptomeningeal angioma in 7 cases, diploic proimence in 5 cases, enlargement of the choroid plexus in 5 cases, choroid plexus cyst in 3 cases and venous anomalies in 3 cases. Port-wine stains, epilepsy, hemiparesis, psychomotor retardation, glaucoma, cortical calcifications on CT, cerebral atrophy and leptomeningeal angiomatosis on MRI were the most frequent features of patients with Sturge-Weber syndrome in this series.

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