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  • Print publication year: 2011
  • Online publication date: March 2012

Chapter 26 - Sturge–Weber syndrome

from Section 3 - Symptomatic epilepsy


Aicardi J (1992) Diseases of the Nervous System in Childhood. London: McKeith Press.
Arzimanoglou A (1997) The surgical treatment of Sturge–Weber syndrome with respect to its clinical spectrum. In: Tuxhorn I, Holthausen H, Boenigk H (eds.) Pediatric Epilepsy Syndromes and Their Surgical Treatment. London: John Libbey, pp. 353–63.
Arzimanoglou A, Aicardi J (1992) The epilepsy of Sturge–Weber syndrome: clinical features and treatment in 23 patients. Acta Neurol Scand (Suppl.) 140:18–22.
Arzimanoglou AA, Andermann F, Aicardi J, et al. (2000) Sturge–Weber syndrome: indications and results of surgery in 20 patients. Neurology 55:1472–9.
Bebin EM, Gomez RM (1988) Sturge–Weber syndrome. In: Gomez RM (ed.) Neurocutaneous Diseases: A Practical Approach. London: Butterworth-Heinemann, pp. 356–67.
Benedikt RA, Brown DC, Walker R, et al. (1993) Sturge–Weber syndrome: cranial MR imaging with Gd-DTPA. AJNR Am J Neuroradiol 14:409–15
Bioxeda P, de Misa RF, Arrazola JM, et al. (1993) Facial angioma and the Sturge–Weber syndrome: a study of 121 cases. Med Clin (Barc) 101:1–4.
Boltshauser E, Wilson J, Hoare RD (1976) Sturge–Weber syndrome with bilateral intracranial calcification. J Neurol Neurosurg Psychiatry 39:429–35.
Bourgeois M, Crimmins DW, de Oliveira RS, et al. (2007) Surgical treatment of epilepsy in Sturge–Weber syndrome in children. J Neurosurg 106(1 Suppl):20–8.
Chevrie JJ, Specola N, Aicardi J (1988) Secondary bilateral synchrony in unilateral pial angiomatosis: successful surgical treatment. J Neurol Neurosurg Psychiatry 51:663–70.
Chiron C, Raynaud C, Tzourio N, et al. (1989) Regional cerebral blood flow by SPECT imaging in Sturge–Weber disease: an aid for diagnosis. J Neurol Neurosurg Psychiatry 52:1402–9.
Chugani HT, Mazziotta JC, Phelps ME (1989) Sturge–Weber syndrome: a study of cerebral glucose utilization with positron emission tomography. J Pediatr 2:244–53.
Cibis GW, Tripathi RC, Tripathi BJ (1984) Glaucoma in Sturge–Weber syndrome. Ophthalmology 91:1061–71.
Comi AM (2007) Sturge–Weber syndrome and epilepsy: an argument for aggressive seizure management in these patients. Expert Rev Neurother 7:951–6.
Di Rocco C, Tamburrini G (2006) Sturge–Weber syndrome. Childs Nerv Syst 22:909–21.
Enjolras O, Riche MC, Merland JJ (1985) Facial port-wine stains and Sturge–Weber syndrome. Pediatrics 76:48–51.
Erba G, Cavazzuti V (1990) Sturge–Weber syndrome: natural history and indications for surgery. J Epilepsy 3(Suppl.):287–91.
Etchevers HC, Vincent C, Le Douarin NM, Couly GF (2001) The cephalic neural crest provides pericytes and smooth muscle cells to all blood vessels of the face and forebrain. Development 128:1059–68.
Fukuyama Y, Tsuchiya S (1979) A study on Sturge–Weber syndrome: report of a case associated with infantile spasms and electroencephalographic evolution in five cases. Eur Neurol 18:194–209.
Greco F, Fiumara A, Sorge G, Pavone L (2008) Subgaleal hematoma in a child with Sturge–Weber syndrome: to prevent stroke-like episodes, is treatment with aspirin advisable? Childs Nerv Syst 24:1479–81.
Griffiths PD (1996) Sturge–Weber syndrome revisited: the role of neuroradiology. Neuropediatrics 27:284–94.
Happle R (1987) Lethal genes surviving by mosaicism: a possible explanation for sporadic birth defects involving the skin. J Am Acad Dermatol 16:899–906.
Hoffman HJ (1997) Benefits of early surgery in Sturge–Weber syndrome. In: Tuxhorn I, Holthausen H, Boenigk H (eds.) Pediatric Epilepsy Syndromes and Their Surgical Treatment. London: John Libbey, pp. 364–70.
Hoffman HJ, Hendrick EB, Dennis M, Armstrong D (1979) Hemispherectomy for Sturge–Weber syndrome. Childs Brain 5:233–48.
Ito M, Sato K, Ohnuki A, Uto A (1990) Sturge–Weber disease: operative indications and surgical results. Brain Dev 12:473–7.
Klapper J (1994) Headache in Sturge–Weber syndrome. Headache 34:521–2.
Kossoff EH, Buck C, Freeman JM (2002) Outcomes of 32 hemispherectomies for Sturge–Weber syndrome worldwide. Neurology 59:1735–8.
Kossoff EH, Hatfield LA, Ball KL, Comi AM (2005) Comorbidity of epilepsy and headache in patients with Sturge–Weber syndrome. J Child Neurol 20:678–82.
Kwan P, Arzimanoglou A, Berg AT, et al. (2010). Definition of drug resistant epilepsy: consensus proposal by the ad hoc Task Force of the ILAE Commission on Therapeutic Strategies. Epilepsia 51:1069–77.
Mallea MJ, Heras GI, Aguirre BP, et al. (1997) Sturge-Weber syndrome: experience with 14 cases. An Esp Pediatr. 46(2):138–42.
Maria BL, Neufeld JA, Rosainz LC, (1998) Central nervous system structure and function in Sturge–Weber syndrome: evidence of neurologic and radiologic progression. J Child Neurol 13:606–18.
Mazereeuw-Hautier J, Syed S, Harper JI (2006) Bilateral facial capillary malformation associated with eye and brain abnormalities. Arch Dermatol 142:994–8.
Oakes WJ (1992) The natural history of patients with the Sturge–Weber syndrome. Pediatr Neurosurg 18:287–90.
Ogunmekan AO, Hwang PA, Hoffman HJ (1989) Sturge–Weber–Dimitri disease: role of hemispherectomy in prognosis. Can J Neurol Sci 16:78–80.
Parsa CF (2008) Sturge–Weber syndrome: a unified pathophysiologic mechanism. Curr Treat Options Neurol 10:47–54.
Pascual-Castroviejo I, Díaz-Gonzalez C, García-Melian RM, Gonzalez-Casado I, Muñoz-Hiraldo E (1993) Sturge–Weber syndrome: study of 40 patients. Pediatr Neurol 9:283–8.
Pascual-Castroviejo I, Pascual-Pascual SI, Velazquez-Fragua R, Viaño J (2008) Sturge–Weber syndrome: study of 55 patients. Can J Neurol Sci 35:301–7.
Probst FP (1980) Vascular morphology and angiographic flow patterns in Sturge–Weber angiomatosis: facts, thoughts and suggestions. Neuroradiology 20:73–8.
Roach ES (1992) Neurocutaneous syndromes. Pediatr Clin N Am 39:591–620.
Rosen I, Salford L, Starck L (1984) Sturge–Weber disease: neurophysiological evaluation of a case with secondary epileptogenesis, successfully treated with lobe-ectomy. Neuropediatrics 15:95–8.
Sassower K, Duchowny P, Jayakar T, (1994) EEG evaluation of children with Sturge–Weber syndrome and epilepsy. J Epilepsy 7:285–9.
Sujansky E, Conradi S (1995a) Sturge–Weber syndrome: age of onset of seizures and glaucoma and the prognosis for affected children. J Child Neurol 10:49–58.
Sujansky E, Conradi S (1995b) Outcome of Sturge–Weber syndrome in 52 adults. Am J Med Genet 57:35–45.
Sullivan TJ, Clarke MP, Morin JD (1992) The ocular manifestations of the Sturge–Weber syndrome. J Pediatr Ophthalmol Strabismus 29:349–56.
Tallman B, Tan OT, Morelli JG, et al. (1991) Location of port-wine stains and the likelihood of ophthalmic and/or central nervous system complications. Pediatrics 87:323–7.
Uram M, Zubillaga C (1982) The cutaneous manifestations of Sturge–Weber syndrome. J Clin Neuroophthalmol 2:245–8.