Sturge-Weber Syndrome
DOI:
https://doi.org/10.61788/njn.v2i15.19Keywords:
Sturge-Weber syndrome, skin angiomatosis, glaucoma, seizuresAbstract
Sturge-Weber syndrome (SWS) refers to phakomatoses. In the classic form of SWS there are the triad of symptoms such as skin angioma, pathology of the eye and neurological disorders. In our clinical case on one half of the face are observed reddish, bluish tint cutaneous nevi "port wine stains" - angiomatosis, on the same side in the gyrus of the occipital-temporal-parietal lobes calcifications vertically up to 60mm (on computer tomography), generalized seizures which resistant to the treatment and sharp-slowly wave complex on electroencephalography, headaches, eye disorders as impaired vision and pain in the eyeball. The diagnosis was based on cutaneous angiomatosis, calcifications in the brain and seizures. Such patients recommended medical supervision, in case of increase of clinical symptoms providing preventive measures with the use of surgical methods.
References
Comi AM. Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge-Weber syndrome. Neurologist 2011;17:179-84.
Y.U.Baraşnev., “Perinatalnaya nevrologiya” Moskva. “Triada”.2001.213:638-639.
Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge Weber syndrome: A review. Pediatr Neurol 2004;30:303-310.
L.O.Badalyan “Detskaya nevrologiya”.Moskva. “MEDpress-inform”2001.422 :602-607.
Q.A.Akimov., M.M.Odinak., “Differensialnaya diaqnostika nervnıx bolezney” Sank Peterburq.”Hippokrat”. 2001., 634:650-663.
Ж..Айкарди « Заболевания Нервной Системы у Детей» Москва. “Бином”, “Панфилова”. 2013, 1том, 121: 553.
Matthew D. Shirley, Ph.D., Hao Tang, Ph.D., et al.Carol J. Gallione, B.A., Joseph D. Baugher, Ph.D., Laurence P. Frelin, M.S., Bernard Cohen, M.D., Paula E. North, M.D., Ph.D., Douglas A. Marchuk, Ph.D., Anne M. Comi, M.D., and Jonathan Pevsner, Ph.D. Sturge–Weber Syndrome and Port-Wine Stains Caused by Somatic Mutation in GNAQ. N Engl J Med 2013; 368:1971-1979.
Lee JB, Kim M, Lee SC, et al. Granuloma pyogenicum arising in an arteriovenous haemangioma associated with a port-wine stain. Br J Dermatol 2000;143:669-71.
Б.В. Лебедев и др.«справочник по неврологии детского возраста» Москва. Медицина. 1995; 344:430-447;
Thiele E.A, Korf BR, Fakomatoses and allied conditions. In: Swayman KF, Aswal S, Ferriero DM (eds). Pediatric Neurology. Philadelphia: Mosby Elsevier 2006:771-796.
Sugama S, Yoshimura H,Ashimine K,et al. Enhanced magnetic resonance imaging of leptomeningial ahgiomatosis. Pediatr Neurol 1997;17:262-265.
Н.Н.Яхно и др. «Болезни нервной системы» Москва. Медицина. 2007. 35:494-510.
Zaki, Syed Ahmed; Vijay Lad (July 2011). "Sturge-Weber syndrome with bilateral facial nevus and early cerebral calcification". Journal of Pediatric Neurosciences 6 (2). doi:10.4103/1817-1745.92825. PMID 3296402.
Arzimanoglou AA, Andermann F, Aicardi J, et al. Sturge-Weber syndrome: indications and results of surgery in 20 patients. Neurology 2000;55:1472-9.
Scherer K, Lorenz S, Wimmershoff M, et al. Both the flashlamp-pumped dye laser and the long-pulsed tunable dye laser can improve results in port-wine stain therapy. Br J Dermatol 2001;145:79-84.
Hennedige AA, Quaba AA, Al-Nakib K. Sturge-Weber syndrome and dermatomal facial port-wine stains: incidence, association with glaucoma, and pulsed tunable dye laser treatment effectiveness. Plast Reconstr Surg 2008;121:1173-80.
Léauté-Labréze C, Boralevi F, Pedespan JM, et al. Pulsed dye laser for Sturge-Weber syndrome. Arch Dis Child 2002;87:434-5.
Zografos L, Bercher L, Egger E, et al. [Treatment of eye tumors by accelerated proton beams. 7 years experience]. Klin Monbl Augenheilkd 1992;200:431-5.
Zografos L, Bercher L, Chamot L, et al. Cobalt-60 treatment of choroidal hemangiomas. Am J Ophthalmol 1996;121:190-9.
Sagong M, Lee J, Chang W. Application of intravitreal bevacizumab for circumscribed choroidal hemangioma. Korean J Ophthalmol 2009;23:127-31.
Anand R. Photodynamic therapy for diffuse choroidal hemangioma associated with Sturge Weber syndrome. Am J Ophthalmol 2003;136:758-60.
Bains HS, Cirino AC, Ticho BH, et al. Photodynamic therapy using verteporfin for a diffuse choroidal hemangioma in Sturge-Weber syndrome. Retina 2004;24:152-5.
Kocyła-Karczmarewicz B, Klimczak-Slaczka D, Grałek M, et al. [Childhood glaucoma associated with Sturge-Weber syndrome --the efficacy of cyclofotocoagulation and other therapeutic methods]. Klin Oczna 2006;108:180-3.).
A.M.Pulatov, A.C.Nikifirov “Spravocnik po semiotike nervnıx bolezney” Taskent“Medisina”Uz.SSR 1983.,173-200:168.
JB B. Sturge-Weber Syndrome. In: Vascular Birthmarks of the Head and Neck, Hochman M (Ed), Facial Plastic Surgery Clinics of North America, 2001:569.
Maria B.L.,Hoang KBN., Robertson RL., et al. İmaging brainstrukture and function in Sturge Weber syndrome.In:Bodensteiner JB,Roach ES.(eds), Sturge Weber syndrome. Mt.Freedom, NJ:Sturge-Weber Foundation, 1999,p43.
Amirikia A, Scott IU, Murray TG. Bilateral diffuse choroidal hemangiomas with unilateral facial nevus flammeus in Sturge-Weber syndrome. Am J Ophthalmol 2000;130:362-4.
Van Emelen C, Goethals M, Dralands L, et al. Treatment of glaucoma in children with Sturge-Weber syndrome. J Pediatr Ophthalmol Strabismus 2000;37:29-34.
Purkait R, Samanta T, Sinhamahapatra T, et al. Overlap of sturge-weber syndrome and klippel-trenaunay syndrome. Indian J Dermatol 2011;56:755-7.
Mammadova M.N., Kazımova G.K., Badalova S.T .,Aliyev R.R., Quliyeva A.İ., et al. “Klippel- Trenaunay-Weber Sendromu olgu sunumu” 17 Ulusal Çocuk Nörolojisi Kongresi Özeti İzmir. 2015.PS 231., 168-174
Rahman M, Rahman S, Akhter S, et al. Overlapping of Sturge Weber syndrome and Klippel Trenaunay Weber syndrome. Mymensingh Med J 2008;17:78-81.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2026 National Association of Neurologists

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.



