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Sturge-Weber syndrome: Continued vigilance is needed.

Saeed Hassan, Amir Babiker, Fahad A Bashiri, Hamdi H Hassan, Maha El Husseini, Mustafa A Salih.


Abstract
Sturge-Weber syndrome (SWS) is a non-hereditary congenital disorder due to somatic mosaic mutations in the GNAQ gene. The classical presentation relates to the brain lesion (cerebral angiomatous lesion of leptomeninges, which is responsible for epileptic seizures, hemiparesis and mental retardation), skin lesion (unilateral facial nevus), ocular and oral involvement. We present a 12-year-old boy who was referred to the Division of Pediatric Neurology, King Saud University Medical City, Riyadh, Saudi Arabia with left-sided hemiparesis. Physical examination showed a port wine stain involving the right side of the face, extending to the upper thorax, and enlargement of both the right eye globe and cornea (megalocornea), indicating the presence of glaucoma. Following urgent referral to ophthalmology service, his eye condition improved dramatically post surgery. Neuroradiological investigations, including cranial computed tomography (CT) and magnetic resonance angiography (MRI) revealed the classical brain lesions of SWS, as well as right leptomeningeal choroidal angioma. Ten months later, he developed focal-onset seizures which responded to treatment. His cognition is normal with good school performance. Continued vigilance is needed to identify and manage the complications of SWS.

Key words: Cerebral angiomatosis; Cerebral calcification; Choroidal haemangioma; Dental manifestations; Dyke-Davidoff-Masson syndrome; Glaucoma; Sturge-Weber syndrome


 
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How to Cite this Article
Pubmed Style

Hassan S, Babiker A, Bashiri FA, Hassan HH, Husseini ME, Salih MA. Sturge-Weber syndrome: Continued vigilance is needed.. Sudan J Paed. 2015; 15(2): 63-70.

Web Style

Hassan S, Babiker A, Bashiri FA, Hassan HH, Husseini ME, Salih MA. Sturge-Weber syndrome: Continued vigilance is needed.. https://sudanjp.com//?mno=280650 [Access: June 23, 2024].

AMA (American Medical Association) Style

Hassan S, Babiker A, Bashiri FA, Hassan HH, Husseini ME, Salih MA. Sturge-Weber syndrome: Continued vigilance is needed.. Sudan J Paed. 2015; 15(2): 63-70.


Vancouver/ICMJE Style

Hassan S, Babiker A, Bashiri FA, Hassan HH, Husseini ME, Salih MA. Sturge-Weber syndrome: Continued vigilance is needed.. Sudan J Paed. (2015), [cited June 23, 2024]; 15(2): 63-70.


Harvard Style

Hassan, S., Babiker, . A., Bashiri, . F. A., Hassan, . H. H., Husseini, . M. E. & Salih, . M. A. (2015) Sturge-Weber syndrome: Continued vigilance is needed.. Sudan J Paed, 15 (2), 63-70.


Turabian Style

Hassan, Saeed, Amir Babiker, Fahad A Bashiri, Hamdi H Hassan, Maha El Husseini, and Mustafa A Salih. 2015. Sturge-Weber syndrome: Continued vigilance is needed.. Sudanese Journal of Paediatrics, 15 (2), 63-70.


Chicago Style

Hassan, Saeed, Amir Babiker, Fahad A Bashiri, Hamdi H Hassan, Maha El Husseini, and Mustafa A Salih. "Sturge-Weber syndrome: Continued vigilance is needed.." Sudanese Journal of Paediatrics 15 (2015), 63-70.


MLA (The Modern Language Association) Style

Hassan, Saeed, Amir Babiker, Fahad A Bashiri, Hamdi H Hassan, Maha El Husseini, and Mustafa A Salih. "Sturge-Weber syndrome: Continued vigilance is needed.." Sudanese Journal of Paediatrics 15.2 (2015), 63-70. Print.


APA (American Psychological Association) Style

Hassan, S., Babiker, . A., Bashiri, . F. A., Hassan, . H. H., Husseini, . M. E. & Salih, . M. A. (2015) Sturge-Weber syndrome: Continued vigilance is needed.. Sudanese Journal of Paediatrics, 15 (2), 63-70.




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