SavedAdd your journal

Journals / European Journal of Therapeutics / 2013 / Cilt: 19 Sayı: 1

Sturge-Weber syndrome: clinical and radiological evaluation

Sturge-Weber sendromu; klinik ve radyolojik değerlendirme

Mehmet CANPOLAT
Hüseyin PER
Ali YIKILMAZ
Hakan GÜMÜŞ
Alper ÖZCAN
Hatice Gamze POYRAZOĞLU
Abdulhakim ÇOŞKUN
Sefer KUMANDAŞ
Pages
30–34
Publication Date
2013-02-01
DOI
—
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

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.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:In this study,we aimed to evaluate the clinical and neuroimaging features in children with Sturge-Weber syndrome (SWS).
📊
Key Findings & Data:The study approved by the Erciyes University Faculty of Medicine Ethics committee (07.08.2012/486).
💡
Conclusion & Impact: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.
🔬Design:Research Article
📍Scope:2012 Verileri
⚙️Variables:Çocuk · manyetik rezonans görüntüleme · porto şarabı lekesi

Abstract

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.

Özet

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, 5'er olguda kalvaryal kalınlaşma ve genişlemiş koroid pleksus, 3'er 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.

🔖

Cite This Article

CANPOLAT, M., PER, H., YIKILMAZ, A., GÜMÜŞ, H., ÖZCAN, A., POYRAZOĞLU, H. G., ÇOŞKUN, A., KUMANDAŞ, S. (2013). Sturge-Weber syndrome: clinical and radiological evaluation. European Journal of Therapeutics, Cilt: 19 Sayı: 1, 30–34. https://ajindex.com/articles/976920

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (20)

  1. Di Rocco C, Tamburrini G. Sturge-Weber syndrome. Childs Nerv Syst 2006
  2. 22(8):909-21 2. Zhou J, Li NY, Zhou XJ, Wang JD, Ma HH, Zhang RS. Sturge- Weber syndrome: a case report and review of literatures. Chin Med J (Engl) 2010
  3. 21 3. Mıhçı E. Hamartomatous Syndromes. Türkiye Klinikleri Journal of Pediatric Sciences 2011
  4. 82 4. Baselga E. Sturge-Weber syndrome. Semin Cutan Med Surg 2004
  5. 98 5. Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: a review. Pediatr Neurol 2004
  6. 10 6. Comi AM. Pathophysiology of Sturge-Weber syndrome. J Child Neurol 2003
  7. 16 7. Comi AM. Update on Sturge-Weber syndrome: diagnosis, treatment, quantitative measures, and controversies. Lymphat Res Biol 2007
  8. 64 8. Pascual-Castroviejo I, Pascual-Pascual SI, Velazquez-Fragua R, Viaño J. Sturge-Weber syndrome: study of 55 patients. Can J Neurol Sci 2008
  9. 7 9. Sharan S, Swamy B, Taranath DA, Jamieson R, Yu T, Wargon O, Grigg JR. Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome. J AAPOS 2009
  10. 8 10. Sujansky E, Conradi S. Sturge-Weber syndrome: age of onset of seizures and glaucoma and the prognosis for affected children. J Child Neurol 1995
  11. 58 11. Sujansky E, Conradi S. Outcome of Sturge-Weber syndrome in 52 adults. Am J Med Genet 1995
  12. 45 12. Puttgen KB, Lin DD. Neurocutaneous vascular syndromes Childs Nerv Syst 2010
  13. 15 13. Comi AM. Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge-Weber syndrome. Neurologist 2011
  14. 84 14. Comi AM. Sturge-Weber syndrome and epilepsy: an argument for aggressive seizure management in these patients. Expert Rev Neurother 2007
  15. 6 15. Daniel RT, Thomas SG, Thomas M. Role of surgery in pediatric epilepsy. Indian Pediatr 2007
  16. 73 16. Slasky SE, Shinnar S, Bello JA. Sturge-Weber syndrome: deep venous occlusion and the radiologic spectrum. Pediatr Neurol 2006
  17. 7 17. Welch K, Naheedy MH, Abroms IF, Strand RD. Computed tomography of Sturge-Weber syndrome in infants. J Comput Assist Tomogr 1980
  18. 6 18. Martí-Bonmatí L, Menor F, Mulas F. The Sturge-Weber syndrome: correlation between the clinical status and radiological CT and MRI findings. Childs Nerv Syst 1993
  19. 9 19. Terdjman P, Aicardi J, Sainte-Rose C, Brunelle F Neuroradiological findings in Sturge-Weber syndrome (SWS) and isolated pial angiomatosis. Neuropediatrics 1991
  20. 20 20. Benedikt RA, Brown DC, Walker R, Ghaed VN, Mitchell M,