Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: a review. Pediatr Neurol. 2004 May. 30 (5):303-10. [QxMD MEDLINE Link].
Sharan S, Swamy B, Taranath DA, et al. Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome. J AAPOS. 2009 Aug. 13(4):374-8. [QxMD MEDLINE Link].
Bruce DA. Neurosurgical aspects of Sturge-Weber syndrome. Bodensteiner JB, Roach ES, eds. Sturge-Weber Syndrome. Mt Freedom, NJ: Sturge Weber Foundation; 1999. 39-42.
Rappaport ZH. Corpus callosum section in the treatment of intractable seizures in the Sturge-Weber syndrome. Childs Nerv Syst. 1988 Aug. 4(4):231-2. [QxMD MEDLINE Link].
Kubicka-Trzaska A, Karska-Basta I, Oleksy P, Romanowska-Dixon B. Management of diffuse choroidal hemangioma in Sturge-Weber syndrome with Ruthenium-106 plaque radiotherapy. Graefes Arch Clin Exp Ophthalmol. 2015 May 26. [QxMD MEDLINE Link].
Aicardi J. Diseases of the Nervous System in Childhood. 2nd ed. London: Mac Keith Press. 1998.
Baselga E. Sturge-Weber syndrome. Semin Cutan Med Surg. 2004 Jun. 23(2):87-98. [QxMD MEDLINE Link].
Bodensteiner JB, Roach ES. Sturge-Weber Syndrome: Introduction and Overview. In: Bodensteiner JB, Roach ES, eds. Sturge-Weber Syndrome. Sturge Weber Foundation. Mt Freedom, New Jersey. 1999.
Boltshauser E, Wilson J, Hoare RD. Sturge-Weber syndrome with bilateral intracranial calcification. J Neurol Neurosurg Psychiatry. 1976 May. 39(5):429-35. [QxMD MEDLINE Link].
Bar-Sever Z, Connolly LP, Barnes PD. Technetium-99m-HMPAO SPECT in Sturge-Weber syndrome. J Nucl Med. 1996 Jan. 37(1):81-3. [QxMD MEDLINE Link].
[Guideline] American Association of Neuroscience Nurses. Care of the patient with seizures. 2nd ed. Glenview (IL): American Association of Neuroscience Nurses; 2007.
Roach ES. Neurocutaneous syndromes. Pediatr Clin North Am. 1992 Aug. 39(4):591-620. [QxMD MEDLINE Link].
Govori V, Gjikolli B, Ajvazi H, Morina N. Management of patient with Sturge-Weber syndrome: a case report. Cases J. 2009 Dec 23. 2:9394. [QxMD MEDLINE Link]. [Full Text].
Rochkind S, Hoffman HJ, Hendrick EB. Sturge-Weber Syndrome: Natural history and prognosis. J Epilepsy. 1990. 3(Suppl):293-304.
Comi AM. Advances in Sturge-Weber syndrome. Curr Opin Neurol. 2006 Apr. 19(2):124-8. [QxMD MEDLINE Link].
Comi AM. Pathophysiology of Sturge-Weber syndrome. J Child Neurol. 2003 Aug. 18(8):509-16. [QxMD MEDLINE Link].
Norman MG, Schoene WC. The ultrastructure of Sturge-Weber disease. Acta Neuropathol (Berl). 1977 Mar 31. 37(3):199-205. [QxMD MEDLINE Link].
Garcia JC, Roach ES, McLean WT. Recurrent thrombotic deterioration in the Sturge-Weber syndrome. Childs Brain. 1981. 8(6):427-33. [QxMD MEDLINE Link].
Gomez MR, Bebin EM. Sturge-Weber syndrome. Butterworths B. Neurocutaneous Diseases: A Practical Approach. 1987. 356-367.
Aylett SE, Neville BG, Cross JH. Sturge-Weber syndrome: cerebral haemodynamics during seizure activity. Dev Med Child Neurol. 1999 Jul. 41(7):480-5. [QxMD MEDLINE Link].
Okudaira Y, Arai H, Sato K. Hemodynamic compromise as a factor in clinical progression of Sturge- Weber syndrome. Childs Nerv Syst. 1997 Apr. 13(4):214-9. [QxMD MEDLINE Link].
Maria BL, Neufeld JA, Rosainz LC. Central nervous system structure and function in Sturge-Weber syndrome: evidence of neurologic and radiologic progression. J Child Neurol. 1998 Dec. 13(12):606-18. [QxMD MEDLINE Link].
Reid DE, Maria BL, Drane WE. Central nervous system perfusion and metabolism abnormalities in Sturge- Weber syndrome. J Child Neurol. 1997 Apr. 12(3):218-22. [QxMD MEDLINE Link].
Sujansky E, Conradi S. Sturge-Weber syndrome: age of onset of seizures and glaucoma and the prognosis for affected children. J Child Neurol. 1995 Jan. 10(1):49-58. [QxMD MEDLINE Link].
Udani V, Pujar S, Munot P, Maheshwari S, Mehta N. Natural history and magnetic resonance imaging follow-up in 9 Sturge-Weber Syndrome patients and clinical correlation. J Child Neurol. 2007 Apr. 22(4):479-83. [QxMD MEDLINE Link].
Huq AH, Chugani DC, Hukku B. Evidence of somatic mosaicism in Sturge-Weber syndrome. Neurology. 2002 Sep 10. 59(5):780-2. [QxMD MEDLINE Link].
Parsa CF. Sturge-weber syndrome: a unified pathophysiologic mechanism. Curr Treat Options Neurol. 2008 Jan. 10(1):47-54. [QxMD MEDLINE Link].
Shirley MD, Tang H, Gallione CJ, Baugher JD, Frelin LP, Cohen B, et al. Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med. 2013 May 23. 368 (21):1971-9. [QxMD MEDLINE Link].
Cunha e SÃ¡ M, Barroso CP, Caldas MC. Innervation pattern of malformative cortical vessels in Sturge-Weber disease: an histochemical, immunohistochemical, and ultrastructural study. Neurosurgery. 1997 Oct. 41(4):872-6; discussion 876-7. [QxMD MEDLINE Link].
Comi AM, Weisz CJ, Highet BH. Sturge-Weber syndrome: altered blood vessel fibronectin expression and morphology. J Child Neurol. 2005 Jul. 20(7):572-7. [QxMD MEDLINE Link].
Sturge Weber Syndrome. National Organization for Rare Disorders. Available at https://rarediseases.org/rare-diseases/sturge-weber-syndrome/. Accessed: January 4, 2018.
Enjolras O, Riche MC, Merland JJ. Facial port-wine stains and Sturge-Weber syndrome. Pediatrics. 1985 Jul. 76(1):48-51. [QxMD MEDLINE Link].
Sener RN. Sturge-Weber syndrome: a patient with a cervical port-wine nevus. Comput Med Imaging Graph. 1997 Nov-Dec. 21(6):359-60. [QxMD MEDLINE Link].
Bioxeda P, de Misa RF, Arrazola JM. [Facial angioma and the Sturge-Weber syndrome: a study of 121 cases]. Med Clin (Barc). 1993 May 29. 101(1):1-4. [QxMD MEDLINE Link].
Tallman B, Tan OT, Morelli JG. Location of port-wine stains and the likelihood of ophthalmic and/or central nervous system complications. Pediatrics. 1991 Mar. 87(3):323-7. [QxMD MEDLINE Link].
Williams DW 3d, Elster AD. Cranial CT and MR in the Klippel-Trenaunay-Weber syndrome. Am J Neuroradiol. 1992 Jan-Feb. 13(1):291-4. [QxMD MEDLINE Link].
Bebin EM, Gomez MR. Prognosis in Sturge-Weber disease: comparison of unihemispheric and bihemispheric involvement. J Child Neurol. 1988 Jul. 3(3):181-4. [QxMD MEDLINE Link].
Oakes WJ. The natural history of patients with the Sturge-Weber syndrome. Pediatr Neurosurg. 1992. 18(5-6):287-90. [QxMD MEDLINE Link].
Pascual-Castroviejo I, Diaz-Gonzalez C, Garcia-Melian RM. Sturge-Weber syndrome: study of 40 patients. Pediatr Neurol. 1993 Jul-Aug. 9(4):283-8. [QxMD MEDLINE Link].
Erba G, Cavazzuti V. Sturge-Weber Syndrome: A natural history. J Epilepsy. 1990. 3(Suppl):287-291.
Coley SC, Britton J, Clarke A. Status epilepticus and venous infarction in Sturge-Weber syndrome. Childs Nerv Syst. 1998 Dec. 14(12):693-6. [QxMD MEDLINE Link].
Sujansky E, Conradi S. Outcome of Sturge-Weber syndrome in 52 adults. Am J Med Genet. 1995 May 22. 57(1):35-45. [QxMD MEDLINE Link].
Jung A, Raman A, Rowland Hill C. Acute hemiparesis in Sturge-Weber syndrome. Pract Neurol. 2009 Jun. 9(3):169-71. [QxMD MEDLINE Link].
Uram M, Zubillaga C. The cutaneous manifestations of Sturge-Weber syndrome. J Clin Neuroophthalmol. 1982 Dec. 2(4):245-8. [QxMD MEDLINE Link].
Shimakawa S, Miyamoto R, Tanabe T, Tamai H. Prolonged left homonymous hemianopsia associated with migraine-like attacks in a child with Sturge-Weber syndrome. Brain Dev. 2009 Oct 1. [QxMD MEDLINE Link].
Maria BL, Neufeld JA, Rosainz LC. High prevalence of bihemispheric structural and functional defects in Sturge-Weber syndrome. J Child Neurol. 1998 Dec. 13(12):595-605. [QxMD MEDLINE Link].
Alkonyi B, Chugani HT, Karia S, Behen ME, Juhász C. Clinical outcomes in bilateral Sturge-Weber syndrome. Pediatr Neurol. 2011 Jun. 44(6):443-9. [QxMD MEDLINE Link]. [Full Text].
Klapper J. Headache in Sturge-Weber syndrome. Headache. 1994 Oct. 34(9):521-2. [QxMD MEDLINE Link].
Cheng KP. Ophthalmologic manifestations of Sturge-Weber syndrome. Bodensteiner JB, Roach ES, eds. Sturge-Weber Syndrome. Mt Freedom, New Jersey: Sturge Weber Foundation; 1999. 17-26.
Miles L, Eisenbaum AM, Biglan AW et al. Guidelines: Glaucoma and Sturge-Weber Syndrome, SWF Web Page.
Sullivan TJ, Clarke MP, Morin JD. The ocular manifestations of the Sturge-Weber syndrome. J Pediatr Ophthalmol Strabismus. 1992 Nov-Dec. 29(6):349-56. [QxMD MEDLINE Link].
Miller RS, Ball KL, Comi AM, Germain-Lee EL. Growth hormone deficiency in Sturge-Weber syndrome. Arch Dis Child. 2006 Apr. 91(4):340-1. [QxMD MEDLINE Link].
Comi AM, Bellamkonda S, Ferenc LM, Cohen BA, Germain-Lee EL. Central hypothyroidism and Sturge-Weber syndrome. Pediatr Neurol. 2008 Jul. 39(1):58-62. [QxMD MEDLINE Link].
Sagi E, Aram H, Peled IJ. Basal cell carcinoma developing in a nevus flammeus. Cutis. 1984 Mar. 33(3):311-2, 318. [QxMD MEDLINE Link].
Purkait R, Samanta T, Sinhamahapatra T, Chatterjee M. Overlap of sturge-weber syndrome and klippel-trenaunay syndrome. Indian J Dermatol. 2011 Nov. 56(6):755-7. [QxMD MEDLINE Link]. [Full Text].
Laufer L, Cohen A. Sturge-Weber syndrome associated with a large left hemispheric arteriovenous malformation. Pediatr Radiol. 1994. 24(4):272-3. [QxMD MEDLINE Link].
Gobbi G, Bouquet F, Greco L. Coeliac disease, epilepsy, and cerebral calcifications. The Italian Working Group on Coeliac Disease and Epilepsy. Lancet. 1992 Aug 22. 340(8817):439-43. [QxMD MEDLINE Link].
Siddique L, Sreenivasan A, Comi AM, Germain-Lee EL. Importance of utilizing a sensitive free thyroxine assay in Sturge-Weber syndrome. J Child Neurol. 2013 Feb. 28(2):269-74. [QxMD MEDLINE Link].
Wilms G, Van Wijck E, Demaerel P. Gyriform calcifications in tuberous sclerosis simulating the appearance of Sturge-Weber disease. Am J Neuroradiol. 1992 Jan-Feb. 13(1):295-7. [QxMD MEDLINE Link].
Borns PF, Rancier LF. Cerebral calcification in childhood leukemia mimicking Sturge-Weber syndrome. Report of two cases. Am J Roentgenol Radium Ther Nucl Med. 1974 Sep. 122(1):52-5. [QxMD MEDLINE Link].
Terdjman P, Aicardi J, Sainte-Rose C. Neuroradiological findings in Sturge-Weber syndrome (SWS) and isolated pial angiomatosis. Neuropediatrics. 1991 Aug. 22(3):115-20. [QxMD MEDLINE Link].
Marti-Bonmati L, Menor F, Mulas F. The Sturge-Weber syndrome: correlation between the clinical status and radiological CT and MRI findings. Childs Nerv Syst. 1993 Apr. 9(2):107-9. [QxMD MEDLINE Link].
Sugama S, Yoshimura H, Ashimine K. Enhanced magnetic resonance imaging of leptomeningeal angiomatosis. Pediatr Neurol. 1997 Oct. 17(3):262-5. [QxMD MEDLINE Link].
Fischbein NJ, Barkovich AJ, Wu Y. Sturge-Weber syndrome with no leptomeningeal enhancement on MRI. Neuroradiology. 1998 Mar. 40(3):177-80. [QxMD MEDLINE Link].
Hu J, Yu Y, Juhasz C, Kou Z, Xuan Y, Latif Z. MR susceptibility weighted imaging (SWI) complements conventional contrast enhanced T1 weighted MRI in characterizing brain abnormalities of Sturge-Weber Syndrome. J Magn Reson Imaging. 2008 Aug. 28(2):300-7. [QxMD MEDLINE Link].
Moore GJ, Slovis TL, Chugani HT. Proton magnetic resonance spectroscopy in children with Sturge-Weber syndrome. J Child Neurol. 1998 Jul. 13(7):332-5. [QxMD MEDLINE Link].
Cakirer S, Yagmurlu B, Savas MR. Sturge-Weber syndrome: diffusion magnetic resonance imaging and proton magnetic resonance spectroscopy findings. Acta Radiol. 2005 Jul. 46(4):407-10. [QxMD MEDLINE Link].
Jeong JW, Chugani HT, Juhász C. Localization of function-specific segments of the primary motor pathway in children with Sturge-Weber syndrome: A multimodal imaging analysis. J Magn Reson Imaging. 2013 Mar 5. [QxMD MEDLINE Link].
Mentzel HJ, Dieckmann A, Fitzek C. Early diagnosis of cerebral involvement in Sturge-Weber syndrome using high-resolution BOLD MR venography. Pediatr Radiol. 2005 Jan. 35(1):85-90. [QxMD MEDLINE Link].
Sivaswamy L, Rajamani K, Juhasz C, Maqbool M, Makki M, Chugani HT. The corticospinal tract in Sturge-Weber syndrome: a diffusion tensor tractography study. Brain Dev. 2008 Aug. 30(7):447-53. [QxMD MEDLINE Link].
Moritani T, Kim J, Sato Y, Bonthius D, Smoker WR. Abnormal hypermyelination in a neonate with Sturge-Weber syndrome demonstrated on diffusion-tensor imaging. J Magn Reson Imaging. 2008 Mar. 27(3):617-20. [QxMD MEDLINE Link].
Adamsbaum C, Pinton F, Rolland Y. Accelerated myelination in early Sturge-Weber syndrome: MRI-SPECT correlations. Pediatr Radiol. 1996 Nov. 26(11):759-62. [QxMD MEDLINE Link].
Griffiths PD, Blaser S, Boodram MB. Choroid plexus size in young children with Sturge-Weber syndrome. Am J Neuroradiol. 1996 Jan. 17(1):175-80. [QxMD MEDLINE Link].
Benedikt RA, Brown DC, Walker R. Sturge-Weber syndrome: cranial MR imaging with Gd-DTPA. AJNR Am J Neuroradiol. 1993 Mar-Apr. 14(2):409-15. [QxMD MEDLINE Link].
Juhasz C, Lai C, Behen ME, Muzik O, Helder EJ, Chugani DC, et al. White matter volume as a major predictor of cognitive function in Sturge-Weber syndrome. Arch Neurol. 2007 Aug. 64(8):1169-74. [QxMD MEDLINE Link].
Bernal B, Altman N. Visual functional magnetic resonance imaging in patients with Sturge-Weber syndrome. Pediatr Neurol. 2004 Jul. 31(1):9-15. [QxMD MEDLINE Link].
Lin DD, Barker PB, Kraut MA. Early characteristics of Sturge-Weber syndrome shown by perfusion MR imaging and proton MR spectroscopic imaging. AJNR Am J Neuroradiol. 2003 Oct. 24(9):1912-5. [QxMD MEDLINE Link].
Griffiths PD, Boodram MB, Blaser S. 99mTechnetium HMPAO imaging in children with the Sturge-Weber syndrome: a study of nine cases with CT and MRI correlation. Neuroradiology. 1997 Mar. 39(3):219-24. [QxMD MEDLINE Link].
Namer IJ, Battaglia F, Hirsch E. Subtraction ictal SPECT co-registered to MRI (SISCOM) in Sturge-Weber syndrome. Clin Nucl Med. 2005 Jan. 30(1):39-40. [QxMD MEDLINE Link].
Pinton F, Chiron C, Enjolras O. Early single photon emission computed tomography in Sturge-Weber syndrome. J Neurol Neurosurg Psychiatry. 1997 Nov. 63(5):616-21. [QxMD MEDLINE Link].
Chugani HT, Mazziotta JC, Phelps ME. Sturge-Weber syndrome: a study of cerebral glucose utilization with positron emission tomography. J Pediatr. 1989 Feb. 114(2):244-53. [QxMD MEDLINE Link].
Jordan LC, Wityk RJ, Dowling MM, DeJong MR, Comi AM. Transcranial Doppler ultrasound in children with Sturge-Weber syndrome. J Child Neurol. 2008 Feb. 23(2):137-43. [QxMD MEDLINE Link].
Riela AR, Stump DA, Roach ES. Regional cerebral blood flow characteristics of the Sturge-Weber syndrome. Pediatr Neurol. 1985 Mar-Apr. 1(2):85-90. [QxMD MEDLINE Link].
Juhász C, Haacke EM, Hu J, Xuan Y, Makki M, Behen ME, et al. Multimodality imaging of cortical and white matter abnormalities in Sturge-Weber syndrome. AJNR Am J Neuroradiol. 2007 May. 28(5):900-6. [QxMD MEDLINE Link].
Alkonyi B, Chugani HT, Behen M, Halverson S, Helder E, Makki MI, et al. The role of the thalamus in neuro-cognitive dysfunction in early unilateral hemispheric injury: a multimodality imaging study of children with Sturge-Weber syndrome. Eur J Paediatr Neurol. 2010 Sep. 14(5):425-33. [QxMD MEDLINE Link]. [Full Text].
Brenner RP, Sharbrough FW. Electroencephalographic evaluation in Sturge-Weber syndrome. Neurology. 1976 Jul. 26(7):629-32. [QxMD MEDLINE Link].
Sassower K, Duchowny M, Jayakar P. EEG evaluation of children with Sturge-Weber Syndrome and Epilepsy. J Epilepsy. 1994. 7:285-289.
Jansen FE, van Huffelen AC, Witkamp T. Diazepam-enhanced beta activity in Sturge Weber syndrome: its diagnostic significance in comparison with MRI. Clin Neurophysiol. 2002 Jul. 113(7):1025-9. [QxMD MEDLINE Link].
Di Trapani G, Di Rocco C, Abbamondi AL. Light microscopy and ultrastructural studies of Sturge-Weber disease. Childs Brain. 1982. 9(1):23-36. [QxMD MEDLINE Link].
Hoffman HJ. Benefits of early surgery in Sturge-Weber syndrome. Tuxhorn I, Holthausen H, Boenigk H, eds. Paediatric Epilepsy syndromes and their surgical treatment. London: John Libbey and Company; 1997. 364-370.
Simonati A, Colamaria V, Bricolo A. Microgyria associated with Sturge-Weber angiomatosis. Childs Nerv Syst. 1994 Aug. 10(6):392-5. [QxMD MEDLINE Link].
[Guideline] Patrianakos TD, Nagao K, Walton DS. Surgical management of glaucoma with the sturge weber syndrome. Int Ophthalmol Clin. 2008 Spring. 48(2):63-78. [QxMD MEDLINE Link].
Kossoff EH, Borsage JL, Comi AM. A pilot study of the modified Atkins diet for Sturge-Weber syndrome. Epilepsy Res. 2010 Dec. 92(2-3):240-3. [QxMD MEDLINE Link].
Arzimanoglou A. The surgical treatment of Sturge-Weber Syndrome with respect to its clinical spectrum. Tuxhorn I, Holthausen H, Boenigk H, eds. Paediatric Epilepsy Syndromes and Their Surgical Treatment. 1997. 353-363.
Gilly R, Lapras C, Tommasi M. [Sturge-Weber-Krabbe disease. Notes on 21 cases]. Pediatrie. 1977 Jan-Feb. 32(1):45-64. [QxMD MEDLINE Link].
Hoffman HJ, Hendrick EB, Dennis M. Hemispherectomy for Sturge-Weber syndrome. Childs Brain. 1979. 5(3):233-48. [QxMD MEDLINE Link].
Kossoff EH, Hatfield LA, Ball KL. Comorbidity of epilepsy and headache in patients with Sturge-Weber syndrome. J Child Neurol. 2005 Aug. 20(8):678-82. [QxMD MEDLINE Link].
Kossoff EH, Balasta M, Hatfield LM, Lehmann CU, Comi AM. Self-reported treatment patterns in patients with Sturge-Weber syndrome and migraines. J Child Neurol. 2007. 2007 Jun. 22(6):720-6. [QxMD MEDLINE Link].
Roach ES, Riela AR, McLean WT, et al. Aspirin therapy for Sturge-Weber Syndrome. Ann Neurol. 1985. 18:387.
Bay MJ, Kossoff EH, Lehmann CU, Zabel TA, Comi AM. Survey of aspirin use in Sturge-Weber syndrome. J Child Neurol. 2011 Jun. 26(6):692-702. [QxMD MEDLINE Link].
Lance EI, Sreenivasan AK, Zabel TA, Kossoff EH, Comi AM. Aspirin use in sturge-weber syndrome: side effects and clinical outcomes. J Child Neurol. 2013 Feb. 28(2):213-8. [QxMD MEDLINE Link].
Morelli JG. Port-wine stains and the Sturge-Weber syndrome. Bodensteiner JB, Roach ES, eds. Sturge Weber Syndrome. Mt Freedom, New Jersey: Sturge Weber Foundation; 1999. 11-16.
Morelli JG, Enjolras O, Goldberg G et al. Treatment of Port wine stains. SWF Web Page.
Troilius A, Wrangsjo B, Ljunggren B. Potential psychological benefits from early treatment of port-wine stains in children. Br J Dermatol. 1998 Jul. 139(1):59-65. [QxMD MEDLINE Link].
Roach ES, Riela AR, Chugani HT. Sturge-Weber syndrome: recommendations for surgery. J Child Neurol. 1994 Apr. 9(2):190-2. [QxMD MEDLINE Link].
Holmes GL. Surgery for intractable seizures in infancy and early childhood. Neurology. 1993 Nov. 43(11 Suppl 5):S28-37. [QxMD MEDLINE Link].
Alexander GL, Norman RM. Sturge-Weber syndrome. In: Vinken PJ, Bruyn GW, eds. Handbook of Clinical Neurology. 1972. 14: 223-240.
Ogunmekan AO, Hwang PA, Hoffman HJ. Sturge-Weber-Dimitri disease: role of hemispherectomy in prognosis. Can J Neurol Sci. 1989 Feb. 16(1):78-80. [QxMD MEDLINE Link].
Arzimanoglou A, Aicardi J. The epilepsy of Sturge-Weber syndrome: clinical features and treatment in 23 patients. Acta Neurol Scand Suppl. 1992. 140:18-22. [QxMD MEDLINE Link].
Kossoff EH, Buck C, Freeman JM. Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide. Neurology. 2002 Dec 10. 59(11):1735-8. [QxMD MEDLINE Link].
Audren F, Abitbol O, Dureau P, Hakiki S, Orssaud C, Bourgeois M, et al. Non-penetrating deep sclerectomy for glaucoma associated with Sturge-Weber syndrome. Acta Ophthalmol Scand. 2006 Oct. 84(5):656-60. [QxMD MEDLINE Link].
Eibschitz-Tsimhoni M, Lichter PR, Del Monte MA, Archer SM, Musch DC, Schertzer RM, et al. Assessing the need for posterior sclerotomy at the time of filtering surgery in patients with Sturge-Weber syndrome. Ophthalmology. 2003 Jul. 110(7):1361-3. [QxMD MEDLINE Link].
Kavanaugh B, Sreenivasan A, Bachur C, Papazoglou A, Comi A, Zabel TA. Intellectual and adaptive functioning in Sturge-Weber Syndrome. Child Neuropsychol. 2015 May 8. 1-14. [QxMD MEDLINE Link].

Media Gallery

A child with Sturge-Weber syndrome with bilateral facial port-wine stain.
Cranial CT scan showing calcifications.
MRI image in Sturge-Weber syndrome.
Single-photon emission computed tomographic scan in Sturge-Weber syndrome.
A child with Sturge-Weber syndrome that primarily affects the distribution of cranial nerve V2-3, with milder involvement of cranial nerve V1. Secondary glaucoma is evident. Ocular melanocytosis involving the sclera of both eyes is an associated finding. Image courtesy of Dr. Lamia Salah Elewa.
Close-up view of the left eye, showing the Ahmed valve implanted in the inferotemporal quadrant after multiple failed filtration procedures induced severe superior conjunctival scarring. Intraocular pressure (IOP) was controlled. Image courtesy of Dr. Lamia Salah Elewa.
T1-weighted, axial magnetic resonance imaging (MRI) scans demonstrate left cerebral hemiatrophy associated with leptomeningeal angiomatosis. Image courtesy of Dr. Lamia Salah Elewa.
Ocular ultrasonogram of the posterior segment demonstrating the diffuse choroidal thickening seen in a diffuse choroidal hemangioma with "tomato-catsup fundus." Image courtesy of Dr. Lamia Salah Elewa.
Choroidal hemangioma. Image courtesy of Thomas M. Aaberg, Jr, MD.
Choroidal hemangioma. Image courtesy of Thomas M. Aaberg, Jr, MD.
Circumscribed hemangioma. Image courtesy of F. Ryan Prall, MD.
Circumscribed hemangioma. Image courtesy of F. Ryan Prall, MD.
B-scan of a choroidal hemangioma showing medium to high internal reflectivity. This is a circumscribed choroidal hemangioma. The patient was not diagnosed with Sturge-Weber Syndrome. Image courtesy of Abdhish R Bhavsar, MD.

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Table 1. Clinical Manifestations of Sturge-Weber Syndrome

Clinical Manifestation	Incidence Rate
Risk of SWS with facial PWS	8%
SWS without facial nevus	13%
Bilateral cerebral involvement	15%
Seizures	72-93%
Hemiparesis	25-56%
Hemianopia	44%
Headaches	44-62%
Developmental delay and mental retardation	50-75%
Glaucoma	30-71%
Choroidal hemangioma	40%

Table 2. Developmental Morbidity Associated with Seizures in Adults with SWS

	With Seizures (%)	Without Seizures (%)
Developmental delay	45	0
Emotional/behavioral problems	85	58
Need for special education	71	0
Employability	46	78

Table 3. Summary of Work-up Findings in Sturge-Weber Syndrome

Procedure	Findings
CSF analysis	Elevated protein
Skull radiography	Tram-track calcifications
Angiography	Lack of superficial cortical veins Non-filling dural sinuses Abnormal, tortuous vessels
CT scanning	Calcifications, tram-track calcifications Cortical atrophy Abnormal draining veins Enlarged choroid plexus Blood-brain barrier breakdown (during seizures) Contrast enhancement
MRI	Gadolinium enhancement of leptomeningeal angiomas (LAs) Enlarged choroid plexus Sinovenous occlusion Cortical atrophy Accelerated myelination
SPECT scanning	Hyperperfusion, early Hypoperfusion, late
PET scanning	Hypometabolism
Electroencephalography (EEG)	Reduced background activity Polymorphic delta activity Epileptiform features

Table 4. Seizure Control in Sturge-Weber Syndrome

Study	Complete	Partial	Refractory/No Control
Gilly et al^[95]	NA*	NA	37%
Sujanski and Conradi^[42] (adults)	27%	49%	24%
Sujanski and Conradi^{[24, 42]}(all ages)	50%	39%	11%
Pascual-Castroviejo et al^[39]	47%	12%	28%
Oakes^[38]	10%	NA	83%
Sassower et al^[87]	NA	NA	43%
Arzimanoglou and Aicardi^[94]	NA	NA	39%
Erba and Cavazzuti^[40]	50%	NA	NA
Toronto^{[90, 96]}	NA	NA	32%
*NA = not available

Table 5. Surgical Results of Hemispherectomy and Limited Resection from 3 Centers

Center	Hemispherectomy	Seizure Free	Limited resection	Seizure Free	Improved
Toronto	12	11	11	8	2
Paris	5	5	15	7	8
Boston	9	8	6	3	0
Total	26	24	32	18	10
24 of 26 patients with hemispherectomy - Seizure free 28 of 32 patients with limited resection - Seizure free or improved

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Author

Masanori Takeoka, MD Assistant Professor, Department of Neurology, Harvard Medical School; Staff Physician, Department of Neurology, Division of Epilepsy and Clinical Neurophysiology, Boston Children's Hospital

Masanori Takeoka, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Medical Association, Child Neurology Society, Massachusetts Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

James J Riviello, Jr, MD George Peterkin Endowed Chair in Pediatrics, Professor of Pediatrics, Section of Neurology and Developmental Neuroscience, Professor of Neurology, Peter Kellaway Section of Neurophysiology, Baylor College of Medicine; Chief of Neurophysiology, Director of the Epilepsy and Neurophysiology Program, Texas Children's Hospital

James J Riviello, Jr, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Partner received royalty from Up To Date for section editor.

Chief Editor

George I Jallo, MD Professor of Neurosurgery, Pediatrics, and Oncology, Director, Clinical Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine

George I Jallo, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, American Society of Pediatric Neurosurgeons

Disclosure: Nothing to disclose.

Acknowledgements

Robert J Baumann, MD Professor of Neurology and Pediatrics, Department of Neurology, University of Kentucky College of Medicine

Robert J Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, and Child Neurology Society