Eriskinde Sturge Weber Sendromu Kranial MRG ve BT Bulguları İle Üç Olgu Sunumu

Sturge Weber Sendromu (SWS), yüzde Porto sarabı renginde anjiom, epilepsi ve glokom ile karakte- rize, ensefalotrigeminal anjiomatozis olarak da bilinen, nadir görülen bir nörokutanöz sendromdur. Mental retardasyon, bas ağrısı, davranıs bozuklukları ve görme alanı defektleri sık görülen semptomlar arasındadır. Görüntüleme yöntemleri, semptomlara neden olan patolojilerin saptanmasında fayda- lıdır. Bilgisayarlı Tomografi (BT) ve Manyetik Rezonans Görüntüleme (MRG) en sık kullanılan modalite- leridir. Manyetik Rezonans Görüntüleme, özellikle yakın zamandaki gelismeler ile leptomeningeal an- jiomatozis gibi nörolojik tutulumun değerlendirilmesinde en iyi yöntemdir. Bu yazıda SWS'lu üç yetiskin hastayı, BT ve MRG bulguları ile sunmayı amaçladık.

Adult Sturge Weber Syndrome : Cranial Mri and Ct Findings in Three Cases

Sturge Weber Syndrome (SWS), also known as encephalotrigeminal angiomatosis, is a rare neurocutaneous syndrome which is characterized by a classical clinical triad of Port-wine stain of the face, epilepsy and glaucoma. Other most common symptoms are mental retardation, headache, behavioral disorders and visual field defects. Neuroimaging provides important information for understanding the underlying pathology of symptoms. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) are commonly used imaging modalities. Among them, MRI is the best modality for assesment of neurologic involvement such as leptomeningeal angiomatosis. Furthermore, recent advances in MRI allow assesment of subtle microstructural brain changes that cannot be detected on conventional MRI images. We report three adult patients with SWS with their CT and MRI findings.

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1. Comi AM. Update on Sturge-Weber Syndrome: Diagnosis, Treatment, Quantitative Measures, and Controversies. Lymphat Res Biol 2007;5:257-264.
2. Comi AM. Pathophysiology of SturgeWeber syndrome. J Child Neurol 2003;18:509-516.
3. Phelps CD. The pathogenesis of glaucoma in Sturge-Weber syndrome. Ophthalmology 1978; 85:276-286.
4. Kossoff EH, Hatfield LA, Ball KL et al. Comorbidity of epilepsy and headache in patients with Sturge-Weber syndrome. J Child Neurol 2005;20:678-682.
5. Juhász C, Haacke EM, Hu J et al. Multimodality imaging of cortical and white matter abnormalities in Sturge-Weber syndrome. AJNR 2007 ;28:900-906.
6. Fogarasi A, Loddenkemper T, Mellado C et al. Sturge-Weber syndrome: clinical and radiological correlates in 86 patients. Ideggyogy Sz 2013; 66:53-57.
7. Arulrajah S, Ertan G, Comi AM et al. MRI with diffusion-weighted imaging in children and young adults with simultaneous supra and infratentorial manifestations of Sturge-Weber syndrome. J Neuroradiol 2010; 37:51-59.
8. Schneider JF, Il'yasov KA, Hennig J et al. Fast quantitative diffusiontensor imaging of cerebral white matter from the neonatal period to adolescence. Neuroradiology 2004;46:258.
9. Lin DDM, Barker PB, Kraut MA, Comi A. Early characteristics of sturge weber syndrome shown by perfusion MR imaging and proton MR spectroscopic imaging. AJNR 2003;24:1912-1915.
10. Moore GJ, Slovis TL, Chugani HT. Proton magnetic resonance spectroscopy in children with Sturge-Weber syndrome. J Child Neurol 1998;13:332-335