sturge weber syndrome


Summary: A non-inherited congenital condition with vascular and neurological abnormalities. It is characterized by facial vascular nevi (PORT-WINE STAIN), and capillary angiomatosis of intracranial membranes (MENINGES; CHOROID). Neurological features include EPILEPSY; cognitive deficits; GLAUCOMA; and visual defects.

Top Publications

  1. Vidaurri de la Cruz H, Tamayo Sánchez L, Duran McKinster C, Orozco Covarrubias M, Ruiz Maldonado R. Phakomatosis pigmentovascularis II A and II B: clinical findings in 24 patients. J Dermatol. 2003;30:381-8 pubmed
    ..II B in 7 male and 11 female patients, with melanosis bulbi in 9, glaucoma in 9, iris mammillations in 2, Sturge Weber syndrome in 6 female patients, and Klippel-Trenaunay syndrome in 2 males, hemifacial, hemicorporal, or limb ..
  2. Anand R. Photodynamic therapy for diffuse choroidal hemangioma associated with Sturge Weber syndrome. Am J Ophthalmol. 2003;136:758-60 pubmed
    ..To report successful treatment of a diffuse choroidal hemangioma associated with chronic retinal detachment...
  3. Sihota R, Gupta V, Agarwal H. Ultrasound biomicroscopic evaluation in Sturge Weber syndrome without glaucoma. Acta Ophthalmol Scand. 2003;81:408-9 pubmed
  4. Rahman M, Rahman S, Rahman M, Akhter S, Kawser C. Overlapping of Sturge Weber syndrome and Klippel Trenaunay Weber syndrome. Mymensingh Med J. 2008;17:78-81 pubmed
    ..right side of the body, glaucoma of both eyes, subcortical calcification which were consistent with the Sturge Weber Syndrome; on the other hand he had also hypertrophy of the right side of the including the face and limbs, ..
  5. Comi A. Advances in Sturge-Weber syndrome. Curr Opin Neurol. 2006;19:124-8 pubmed
    ..More research is needed to translate advances in molecular research and neuroimaging advances into new treatment strategies for the disease. ..
  6. Welty L. Sturge-Weber syndrome: A case study. Neonatal Netw. 2006;25:89-98 pubmed
    ..This article begins with a case presentation of an infant with SWS and then presents the etiology, embryology, pathophysiology, clinical presentation, management, and prognosis of SWS. ..
  7. Sijens P, Gieteling E, Meiners L, Sival D, Potze J, Irwan R, et al. Diffusion tensor imaging and magnetic resonance spectroscopy of the brain in a patient with Sturge-Weber syndrome. Acta Radiol. 2006;47:972-6 pubmed
    ..The N-acetylaspartate decreases and/or choline increases observed here and in eight previously described Sturge-Weber patients probably reflect neuronal loss or dysfunction and demyelination as a result of recurrent seizures. ..
  8. Oguz K, Senturk S, Ozturk A, Anlar B, Topcu M, Cila A. Impact of recent seizures on cerebral blood flow in patients with sturge-weber syndrome: study of 2 cases. J Child Neurol. 2007;22:617-20 pubmed
    ..These findings point to the variable results of functional studies in Sturge-Weber syndrome that might lead to miscalculations of the lesion area before surgery. ..
  9. Lorette G, Piram M, Herbreteau D, Lermusiaux P. [Sturge-Weber-Krabbe syndrome (or encephalo-trigeminal angiomatosis)]. Ann Dermatol Venereol. 2004;131:405-6 pubmed

More Information

Publications110 found, 100 shown here

  1. Mofidi R, Nagy J, Levison R, Griffiths G. Beware of varicose veins in a patient with sturge-weber syndrome. Ann Vasc Surg. 2007;21:640-4 pubmed
  2. Curatolo P, Lo Castro A, Pinci M, Moavero R, Bombardieri R. Neuroimaging findings of Sturge-Weber Syndrome in a child with Tuberous Sclerosis. Brain Dev. 2009;31:352-5 pubmed publisher
    ..The coexistence of signs of both diseases in the same individuals could be explained by common altered pathways that could lead to an anomalous angiogenesis. ..
  3. de Leon Casasola O, Lema M. Anesthesia for patients with Sturge-Weber disease and Klippel-Trenaunay syndrome. J Clin Anesth. 1991;3:409-13 pubmed
    ..The successful management of this case illustrates that adequate preparation to handle the possible complications may result in safe anesthetic management in patients with these problems. ..
  4. Lee C, Choi D, Oh Y, Yoon H, Kim J. An infantile case of Sturge-Weber syndrome in association with Klippel-Trenaunay-Weber syndrome and phakomatosis pigmentovascularis. J Korean Med Sci. 2005;20:1082-4 pubmed
    ..Very rare case combined with these three kinds of phakomatosis has been reported...
  5. Kelley T, Hatfield L, Lin D, Comi A. Quantitative analysis of cerebral cortical atrophy and correlation with clinical severity in unilateral Sturge-Weber syndrome. J Child Neurol. 2005;20:867-70 pubmed
    ..This method offers the advantages of relative simplicity, objectivity, and wide applicability to films from outside institutions, as would be encountered in clinical practice. ..
  6. Bhansali R, Yeltiwar R, Agrawal A. Periodontal management of gingival enlargement associated with Sturge-Weber syndrome. J Periodontol. 2008;79:549-55 pubmed
    ..Periodic systemic and oral examinations are recommended to identify and prevent any complications from the cranial and oral lesions. ..
  7. Giordano F, Spacca B, Barba C, Mari F, Pisano T, Guerrini R, et al. Vertical extraventricular functional hemispherotomy: a new variant for hemispheric disconnection. Technical notes and results in three patients. Childs Nerv Syst. 2015;31:2151-60 pubmed publisher
  8. Inan C, Marcus J. Sturge--Weber syndrome: report of an unusual cutaneous distribution. Brain Dev. 1999;21:68-70 pubmed
    ..This case was diagnosed as Sturge-Weber syndrome with unusual cutaneous manifestations. ..
  9. Widdess Walsh P, Friedman N. Left-sided facial nevus with contralateral leptomeningeal angiomatosis in a child with Sturge-Weber syndrome: case report. J Child Neurol. 2003;18:304-5 pubmed
    ..The literature regarding variants of Sturge-Weber syndrome and their prognosis is reviewed. The prognosis for this variant is likely similar to Sturge-Weber syndrome with an ipsilateral leptomeningeal angioma...
  10. Hatfield L, Crone N, Kossoff E, Ewen J, Pyzik P, Lin D, et al. Quantitative EEG asymmetry correlates with clinical severity in unilateral Sturge-Weber syndrome. Epilepsia. 2007;48:191-5 pubmed
    ..qEEG may potentially be a useful tool for early diagnosis and monitoring of disease progression in SWS. qEEG may prove useful, in severely affected individuals with SWS, for determining regions of brain dysfunction. ..
  11. Quagliano F, Fontana L, Parente G, Tassinari G. Choroidal effusion after diode laser cyclophotocoagulation in Sturge-Weber syndrome. J AAPOS. 2008;12:526-7 pubmed publisher
    ..Our case illustrates that significant but transient choroidal effusions can still occur after diode laser procedure. ..
  12. Comi A. Sturge-Weber syndrome. Handb Clin Neurol. 2015;132:157-68 pubmed publisher
    ..Future possible treatments based upon new knowledge of the somatic mutation and downstream pathways are currently being considered and studied. ..
  13. Abdolrahimzadeh S, Scavella V, Battaglia D, Recupero S. Spectral Domain Optical Coherence Tomography of Choroidal and Outer Retinal Layer Thickness in the Sturge Weber Syndrome. Curr Eye Res. 2016;41:1614-1617 pubmed
    To evaluate choroidal thickness and its effect on the outer retinal layers in patients with Sturge Weber syndrome (SWS)...
  14. Giridharan W, Belloso A, Pau H, McEwan J, Clarke R. Epistaxis in children with vascular malformations--commentary of two cases and literature review. Int J Pediatr Otorhinolaryngol. 2002;65:137-41 pubmed
    ..Both cases were managed conservatively since intervention in these vascular lesions carries a high risk of damaging adjacent structures. ..
  15. Madlom M, Hoggard N, Griffiths P, Chan J. Facial naevus flammeus with choroidal haemangioma and without intracranial involvement. Dev Med Child Neurol. 2003;45:139 pubmed
  16. Nathan N, Thaller S. Sturge-Weber syndrome and associated congenital vascular disorders: a review. J Craniofac Surg. 2006;17:724-8 pubmed
  17. Greco F, Fiumara A, Sorge G, Pavone L. Subgaleal hematoma in a child with Sturge-Weber syndrome: to prevent stroke-like episodes, is treatment with aspirin advisable?. Childs Nerv Syst. 2008;24:1479-81 pubmed publisher
    ..It is conceivable that the minor head trauma and chronic use of aspirin caused the SGH. Based on this event, the chronic use of aspirin in young patients with SWS, as suggested to prevent stroke-like episodes, is disputable. ..
  18. Obeid M, Wyllie E, Rahi A, Mikati M. Approach to pediatric epilepsy surgery: State of the art, Part II: Approach to specific epilepsy syndromes and etiologies. Eur J Paediatr Neurol. 2009;13:115-27 pubmed publisher
  19. Gajinov Z, Misic Pavkov G, Matic M, Duran V, Adić O, Duran B, et al. Persistent hepatic venous plexus in a patient with Sturge-Weber syndrome. Pediatr Dermatol. 2008;25:452-4 pubmed publisher
    ..To the best of our knowledge, this hepatic finding has not been previously described in association with Sturge-Weber syndrome. ..
  20. Pinto A, Chen L, Friedman R, Grant P, Poduri A, Takeoka M, et al. Sturge-Weber Syndrome: Brain Magnetic Resonance Imaging and Neuropathology Findings. Pediatr Neurol. 2016;58:25-30 pubmed publisher
  21. Pfund Z, Kagawa K, Juhasz C, Shen C, Lee J, Chugani D, et al. Quantitative analysis of gray- and white-matter volumes and glucose metabolism in Sturge-Weber syndrome. J Child Neurol. 2003;18:119-26 pubmed
    ..Extensive gray- and white-matter volume loss and hypometabolism ipsilateral to the angioma likely contribute to the frequently observed progressive cognitive dysfunction in these patients, regardless of the extent of the angioma. ..
  22. Lam S, Williams E. Practical considerations in the treatment of capillary vascular malformations, or port wine stains. Facial Plast Surg. 2004;20:71-6 pubmed
    ..Introductory remarks are made as to the nature of PWSs, related syndromes, and the evolution of treatment; practical management guidelines are then discussed. ..
  23. Elgin U, Simsek T, Batman A. Use of the ex-press miniature glaucoma implant in a child with Sturge-Weber syndrome. J Pediatr Ophthalmol Strabismus. 2007;44:248-50 pubmed
    ..The Ex-Press miniature glaucoma implant was placed 10 days before trabeculectomy with mitomycin C. There were no complications with either procedure. ..
  24. Rodríguez Bujaldón A, Vazquez Bayo C, Jiménez Puya R, Moreno Gimenez J. [Sturge-Weber syndrome and type 1 neurofibromatosis: a chance association?]. Actas Dermosifiliogr. 2008;99:313-4 pubmed
  25. Onesti M, Fioramonti P, Carella S, Spinelli G, Scuderi N. Surgical and laser treatment of Sturge-Weber syndrome. Aesthetic Plast Surg. 2009;33:666-8 pubmed publisher
    ..An innovative treatment based on surgical and laser techniques of a patient affected with Sturge-Weber syndrome is reported. The aesthetic and functional results were satisfactory. ..
  26. Poliak N, Rainey A. Concurrent Sturge-Weber syndrome, facial infantile hemangioma, and cutis marmorata telangiectatica congenita. Cutis. 2017;100:252-254 pubmed
  27. Yildiz S. [Arteriovenous malformations mimicking dilated medulla oblongata veins in Sturge Weber syndrome]. Tani Girisim Radyol. 2003;9:109-10 pubmed
  28. Park C, Bodensteiner J. An infant with a segmental hemangioma: Sturge-Weber?. Semin Pediatr Neurol. 2008;15:164-6 pubmed publisher
    ..Subsequent evaluation suggested the diagnosis of PHACES syndrome, which is discussed. The risks to vision presented by the orbital involvement of the hemangioma should be noted. ..
  29. Arzimanoglou A, Andermann F, Aicardi J, Sainte Rose C, Beaulieu M, Villemure J, et al. Sturge-Weber syndrome: indications and results of surgery in 20 patients. Neurology. 2000;55:1472-9 pubmed
    ..The authors' results suggest that lesionectomy is a good approach, provided that the pial angioma is unilateral and the resection can be complete. ..
  30. Zhou Q, Zheng J, Yang X, Wang Y, Ye W, Zhu H, et al. Fibronectin: characterization of a somatic mutation in Sturge-Weber syndrome (SWS). Med Hypotheses. 2009;73:199-200 pubmed publisher
    ..Understanding the pathophysiology of Sturge-Weber syndrome will help us to establish future neuroprotective strategies and novel treatment modalities. ..
  31. Shin R, Moonis G, Imbesi S. Transient focal leptomeningeal enhancement in Sturge-Weber syndrome. J Neuroimaging. 2002;12:270-2 pubmed
    ..Magnetic resonance imaging of the brain demonstrated focal leptomeningeal enhancement, which subsequently resolved. ..
  32. Utsunomiya T, Shimizu H, Sunaga S, Sugano H, Arai N. [A case of Sturge-Weber syndrome with severe temper tantrum]. No Shinkei Geka. 2006;34:819-24 pubmed
    ..This case may indicate that severe temper tantrum was associated with a change in amygdala structures. ..
  33. Sivaswamy L, Rajamani K, Juhasz C, Maqbool M, Makki M, Chugani H. The corticospinal tract in Sturge-Weber syndrome: a diffusion tensor tractography study. Brain Dev. 2008;30:447-53 pubmed publisher
    ..Thus, DTI can be a clinically useful method to evaluate the integrity of the corticospinal tract in young children who are at risk for progressive motor dysfunction. ..
  34. Wu Y, Yu R, Lin X, Guo W. [Sturge-Weber syndrome in port-wine stain patients: a retrospective study on the clinical features and screening strategy]. Zhonghua Yan Ke Za Zhi. 2017;53:753-757 pubmed publisher
    ..b>Conclusions: There is a high proportion of SWS with glaucoma in ophthalmic division affected PWS patients. Fundus examinations were necessary for this type of patients. (Chin J Ophthalmol, 2017, 53:753-757)...
  35. Lisotto C, Mainardi F, Maggioni F, Zanchin G. Headache in Sturge-Weber syndrome: a case report and review of the literature. Cephalalgia. 2004;24:1001-4 pubmed
  36. Comi A. Update on Sturge-Weber syndrome: diagnosis, treatment, quantitative measures, and controversies. Lymphat Res Biol. 2007;5:257-64 pubmed publisher
  37. Wu Y, Yu R, Chen D, Xu L, Zhu L, Li M, et al. Early Trabeculotomy Ab Externo in Treatment of Sturge-Weber Syndrome. Am J Ophthalmol. 2017;182:141-146 pubmed publisher
    ..Early trabeculotomy ab externo was safe and led to good intermediate-term surgical outcomes for early-onset glaucoma in SWS patients. Higher preoperative IOP and corneal edema were associated with a greater risk of surgery failure. ..
  38. Randon M, Levy Gabriel C, Abbas R, Dendale R, Lumbroso L, Desjardins L, et al. Results of external beam radiotherapy for diffuse choroidal hemangiomas in Sturge-Weber syndrome. Eye (Lond). 2018;32:1067-1073 pubmed publisher
    ..EBRT using 20 Gy in 10 fractions is efficient, decreases tumor thickness, reattaches the retina, and stabilizes visual acuity. In the long term, retinal reattachment allows ocular conservation by preventing phthisis bulbi. ..
  39. Comi A. Pathophysiology of Sturge-Weber syndrome. J Child Neurol. 2003;18:509-16 pubmed
    ..Neurologic deterioration in Sturge-Weber syndrome is likely secondary to impaired blood flow to the brain and is worsened by the presence of seizures. Insights from related areas are discussed, and future research studies are suggested. ..
  40. Kossoff E, Hatfield L, Ball K, Comi A. Comorbidity of epilepsy and headache in patients with Sturge-Weber syndrome. J Child Neurol. 2005;20:678-82 pubmed
    ..Correlations of family history with both age at symptom onset and behavior problems suggest that genetic substrate could be one factor determining the variable neurologic manifestations seen in Sturge-Weber syndrome. ..
  41. Oziebło Kupczyk M, Urban B, Mrugacz M, Bakunowicz Lazarczyk A. [The evaluation of the efficacy of treatment in glaucoma associated with Sturge-Weber syndrome]. Klin Oczna. 2007;109:46-8 pubmed
    ..After antiglaucoma medications mean IOP was 15.2 mmHg (range from 12 to 18 mmHg). The results of therapy for glaucoma associated with the Sturge-Weber syndrome are often disappointing. ..
  42. Higueros E, roe E, Granell E, Baselga E. Sturge-Weber Syndrome: A Review. Actas Dermosifiliogr. 2017;108:407-417 pubmed publisher
    ..Prognosis depends on the extent of leptomeningeal involvement and the severity of the glaucoma. ..
  43. Sood D, Rathore A, Sood I, Kumar D, Sood N. Long-term intraocular pressure after combined trabeculotomy-trabeculectomy in glaucoma associated with Sturge-Weber syndrome. Eur J Ophthalmol. 2017;: pubmed publisher
    ..Vision loss in Sturge-Weber syndrome (SWS), a rare congenital disorder, is primarily due to glaucoma...
  44. Naouri M, Lorette G. [Angiomas]. Rev Prat. 2006;56:1835-9 pubmed
  45. Alkonyi B, Chugani H, Behen M, Halverson S, Helder E, Makki M, 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;14:425-33 pubmed publisher
    ..Sturge-Weber syndrome (SWS) with unilateral hemispheric involvement is a clinical model of early onset, chronic, often progressive hemispheric injury, resulting in variable neuro-cognitive impairment...
  46. Barry C, Morgan W, Jitskaia L, Eikelboom R, Kanagasingam Y. Computer-assisted planimetry associated with Sturge-Weber syndrome. J Audiov Media Med. 2000;23:149-52 pubmed
    ..To maximize the information obtained from optic disc images, low cost software can assist with quantifying disc parameters aiding clinical interpretation...
  47. Schreiber S, Doepp F, Bender A, Schmierer K, Valdueza J. Diffuse cerebral angiomatosis. Neurology. 2003;60:1216-8 pubmed
  48. Fogelholm R. Ionising radiation in infancy and adult cognitive function: radiation may not solely explain later cognitive function. BMJ. 2004;328:581-2; author reply 582 pubmed
  49. Romero A, Echebarria A, Sierrasesumaga L. [Vascular malformations as syndromic markers]. An Sist Sanit Navar. 2004;27 Suppl 1:45-56 pubmed
    ..This paper describes the principal signs and symptoms of those syndromes in which a vascular malformation is the key that raises suspicion about the existence of other associated lesions...
  50. Singh A, Kaiser P, Sears J. Choroidal hemangioma. Ophthalmol Clin North Am. 2005;18:151-61, ix pubmed
    ..Diffuse choroidal hemangiomas are usually evident at birth and generally occur as a part of neuro-oculo-cutaneous hemangiomatosis (Sturge-Weber syndrome)...
  51. Chen T, Young L. Sturge-Weber syndrome (choroidal hemangioma and glaucoma). J Pediatr Ophthalmol Strabismus. 2005;42:320 pubmed
  52. Quigg M, Rust R, Miller J. Clinical findings of the phakomatoses: Sturge-Weber syndrome. Neurology. 2006;66:E17-8 pubmed
  53. Yallapragada A, Cure J, Holden K. Sturge-Weber syndrome variant with atypical intracranial findings: case report. J Child Neurol. 2006;21:155-7 pubmed
    ..Considering that intracranial venous anomalies also are likely compatible with the embryologic explanation of Sturge-Weber syndrome, this child can serve as an unusual example of Sturge-Weber syndrome type II...
  54. Delvi M, Takrouri M. Anesthesia for encephalo-trigeminal angiomatosis (Sturge-Weber syndrome). Middle East J Anaesthesiol. 2006;18:785-90 pubmed
    ..This patient tolerated balanced anesthesia well. Anesthesia should be planned to avoid trauma to the hemangiomata, increases in intraocular and intracranial pressure, and be cognizent of current anticonvulsant therapy...
  55. Morino M, Shimizu H, Uda T, Naitoh K, Kawahara S, Ishiguro T, et al. Transventricular hemispherotomy for surgical treatment of intractable epilepsy. J Clin Neurosci. 2007;14:171-5 pubmed
  56. Inutsuka M, Ohta H, Ogawa K, Yoshinaga H, Ohtsuka Y. [Case of Sturge-Weber syndrome manifesting complex partial status epilepticus]. No To Hattatsu. 2009;41:52-6 pubmed
    ..CPSE should be recognized as one of the seizure types of SWS. ..
  57. Sharan S, Swamy B, Taranath D, Jamieson R, Yu T, Wargon O, et al. Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome. J AAPOS. 2009;13:374-8 pubmed publisher
    ..The aim of this study was to review clinical features and management of ocular complications of SWS and assess the effects of dermatological laser treatment on the incidence of glaucoma or ocular hypertension...
  58. Paller A. The Sturge-Weber syndrome. Pediatr Dermatol. 1987;4:300-4 pubmed
    ..Laser therapy is the most promising therapeutic option for cosmetic management of the facial nevus flammeus...
  59. Shields W. Catastrophic epilepsy in childhood. Epilepsia. 2000;41 Suppl 2:S2-6 pubmed
    ..In such patients, control of the seizures may lead to more normal intellectual development. Thus, every effort should be made to control seizures in children with catastrophic epilepsy...
  60. Celebi S, Alagoz G, Aykan U. Ocular findings in Sturge-Weber syndrome. Eur J Ophthalmol. 2000;10:239-43 pubmed
    ..We reviewed the rare ocular findings of Sturge-Weber syndrome (SWS) and the results of implantation of the Ahmed valve in cases associated with glaucoma...
  61. Lee H, Choi S, Kim S, Hong Y. A case of glaucoma associated with Sturge-Weber syndrome and Nevus of Ota. Korean J Ophthalmol. 2001;15:48-53 pubmed
    ..Elevated intraocular pressure, with or without glaucomatous damage, is observed in 10% of the cases. We report the first case of glaucoma associated with Sturge-Weber syndrome and Nevus of Ota in Korea...
  62. Huq A, Chugani D, Hukku B, Serajee F. Evidence of somatic mosaicism in Sturge-Weber syndrome. Neurology. 2002;59:780-2 pubmed
  63. Garcia Muret M, Puig L, Allard C, Alomar A. Hypomelanosis of Ito with Sturge-Weber syndrome-like leptomeningeal angiomatosis. Pediatr Dermatol. 2002;19:536-40 pubmed
  64. Cakirer S, Yagmurlu B, Savas M. Sturge-Weber syndrome: diffusion magnetic resonance imaging and proton magnetic resonance spectroscopy findings. Acta Radiol. 2005;46:407-10 pubmed
    ..Proton MR spectroscopy revealed decreased N-acetyl aspartate and increased choline peaks, indicating disintegration of neural tissue associated with neuronal loss as well...
  65. Corey S, O Donovan C. Sturge-Weber syndrome and accompanying Dyke-Davidoff-Masson syndrome. Arch Neurol. 2005;62:1928-9 pubmed
  66. Bourgeois M, Crimmins D, de Oliveira R, Arzimanoglou A, Garnett M, Roujeau T, et al. Surgical treatment of epilepsy in Sturge-Weber syndrome in children. J Neurosurg. 2007;106:20-8 pubmed
  67. Greene A, Taber S, Ball K, Padwa B, Mulliken J. Sturge-Weber syndrome: soft-tissue and skeletal overgrowth. J Craniofac Surg. 2009;20 Suppl 1:617-21 pubmed publisher
    ..0%), or skeletal overgrowth (11.0%). In conclusion, facial hypertrophy is a major component of SWS; these patients should be counseled about the risk of overgrowth and about the types of possible operative correction...
  68. Reesman J, Gray R, Suskauer S, Ferenc L, Kossoff E, Lin D, et al. Hemiparesis is a clinical correlate of general adaptive dysfunction in children and adolescents with Sturge-Weber syndrome. J Child Neurol. 2009;24:701-8 pubmed publisher
    ..Information obtained during neurological examination of children and adolescents with Sturge-Weber syndrome particularly hemiparetic status is useful for identifying children who may need additional intervention...
  69. Fischbein N, Barkovich A, Wu Y, Berg B. Sturge-Weber syndrome with no leptomeningeal enhancement on MRI. Neuroradiology. 1998;40:177-80 pubmed
    ..We present a case of SWS with no evidence of leptomeningeal enhancement. This case illustrates that leptomeningeal enhancement need not be present in SWS, and the absence of this characteristic finding does not preclude the diagnosis...
  70. Ville D, Enjolras O, Chiron C, Dulac O. Prophylactic antiepileptic treatment in Sturge-Weber disease. Seizure. 2002;11:145-50 pubmed
  71. Grant A, Jain V, Bose S. Epileptic monocular nystagmus. Neurology. 2002;59:1438-41 pubmed
    ..It is hypothesized that the seizure discharge either activated a cortical saccade region and caused simultaneous supranuclear inhibition of ipsilateral eye movement or triggered monocular eye movement commands...
  72. Iizuka T, Sakai F, Yamakawa K, Suzuki K, Suzuki N. Vasogenic leakage and the mechanism of migraine with prolonged aura in Sturge-Weber syndrome. Cephalalgia. 2004;24:767-70 pubmed
  73. Namer I, Battaglia F, Hirsch E, Constantinesco A, Marescaux C. Subtraction ictal SPECT co-registered to MRI (SISCOM) in Sturge-Weber syndrome. Clin Nucl Med. 2005;30:39-40 pubmed
  74. Juhasz C, Chugani H. An almost missed leptomeningeal angioma in Sturge-Weber syndrome. Neurology. 2007;68:243 pubmed
  75. Yu T, Liu H, Lee W. The correlation between motor impairment and cerebral blood flow in Sturge-Weber syndrome. Eur J Paediatr Neurol. 2007;11:96-103 pubmed
    ..Therefore, the quantitative CBF study is one of the diagnostic modality of choice in evaluating the neurological status in patients with SWS...
  76. Redondo P. [Vascular malformations (I). Concept, classification, pathogenesis and clinical features]. Actas Dermosifiliogr. 2007;98:141-58 pubmed
    ..Additionally, clinical features of the different subtypes of vascular anomalies as well as their association in certain syndromes are reviewed...
  77. Georgescu E, Stănescu L, Dumitrescu D, Ionescu R, Georgescu I. Portal cavernomatous transformation leading to variceal hemorrhage in Sturge-Webber syndrome. A rare, but possible association. Rom J Morphol Embryol. 2007;48:171-5 pubmed
    ..The clinical presentation and imagistic assessment confirmed the diagnosis of Sturge-Weber syndrome associated with upper non-cirrhotic portal hypertension generated by a malformation of portal vein...
  78. Shirazi F, Cohen C, Fried L, Arbiser J. Mammalian target of rapamycin (mTOR) is activated in cutaneous vascular malformations in vivo. Lymphat Res Biol. 2007;5:233-6 pubmed publisher
    ..Since there are no Akt inhibitors approved for clinical use, we examined phosphorylated S6 expression, a downstream target of Akt. Phosphorylated S6 indicates potential sensitivity to rapamycin...
  79. Suskauer S, Trovato M, Zabel T, Comi A. Physiatric findings in individuals with Sturge-Weber syndrome. Am J Phys Med Rehabil. 2010;89:323-30 pubmed publisher
  80. Martinez Bermejo A, Tendero A, Lopez Martin V, Arcas J, Royo A, Polanco I, et al. [Occipital leptomeningeal angiomatosis without facial angioma. Could it be considered a variant of Sturge-Weber syndrome?]. Rev Neurol. 2000;30:837-41 pubmed
    ..The cases with absence of a facial angioma are usually considered to be variants of the syndrome...
  81. Comi A, Hunt P, Vawter M, Pardo C, Becker K, Pevsner J. Increased fibronectin expression in sturge-weber syndrome fibroblasts and brain tissue. Pediatr Res. 2003;53:762-9 pubmed
    ..The reproducible differences in fibronectin gene expression between the SWS port-wine-derived fibroblasts and the SWS normal skin-derived fibroblasts are consistent with the presence of a hypothesized somatic mutation underlying SWS...
  82. Comi A, Fischer R, Kossoff E. Encephalofacial angiomatosis sparing the occipital lobe and without facial nevus: on the spectrum of Sturge-Weber syndrome variants?. J Child Neurol. 2003;18:35-8 pubmed
    ..This diagnosis should be considered in any child presenting with seizures or complicated migraines and intracranial calcifications...
  83. Kumar S, Muranjan M, Tullu M, Lahiri K. Incomplete monosymptomatic leptomeningeal angiomatosis. Indian J Pediatr. 2004;71:947 pubmed
    ..Our case qualifies to be called incomplete monosymptomatic leptomeningeal angiomatosis...
  84. Müller Forell W. [Neuroradiology of the intracranial visual pathway. Part II]. Radiologe. 2005;45:1043-55 pubmed
    ..A second focus is on vascular pathology, not only infarction, but cavernoma and venous malformation in Sturge-Weber syndrome. The third topic deals with inflammatory processes such as multiple sclerosis or sarcoidosis...
  85. Rafalowska J, Dziewulska D, Podlecka A, Zakrzewska Pniewska B. Extensive mixed vascular malformation clinically imitating multiple sclerosis--case report. Clin Neuropathol. 2006;25:237-42 pubmed
    ..The presence of various types of pathological vessels originating from different ontogenic periods indicates remittent or prolonged influence of teratogenic factor(s) in all periods of fetal vessel development...
  86. Juhasz C, Batista C, Chugani D, Muzik O, Chugani H. Evolution of cortical metabolic abnormalities and their clinical correlates in Sturge-Weber syndrome. Eur J Paediatr Neurol. 2007;11:277-84 pubmed
    ..The natural course of Sturge-Weber syndrome (SWS) is poorly understood, although neurological symptoms are often progressive...
  87. Siewiera I, Wysocki M, Latkowski I. [Lasers in plastic surgery--vascular lasers]. Wiad Lek. 2007;60:178-84 pubmed
    ..In the paper we discuss the most common vascular lesions and available lasers used to treat them. Described indications and safety rules might be useful for many specialists for their patient therapy choice...
  88. Matysik Woźniak A, Gerkowicz M, Pawłowska Wakowicz B. Basal cell carcinoma in an eyelid of a farmer with Sturge-Weber syndrome. Ann Agric Environ Med. 2007;14:325-7 pubmed
    ..It is difficult to diagnose neoplastic transformations that could arise in the nevus flammeus. Radiotherapy followed by complete surgical excision were used as a treatment in the described patient...
  89. Horgan N, O Keefe M, McLoone E, Lanigan B. Fundus fluorescein angiographic characterization of diffuse choroidal hemangiomas. J Pediatr Ophthalmol Strabismus. 2008;45:26-30 pubmed
    ..To evaluate the clinical usefulness of fundus fluorescein angiography (FFA) in characterizing diffuse choroidal hemangiomas in Sturge-Weber syndrome...
  90. Sen Y, Dilber E, Odemis E, Ahmetoglu A, Aynaci F. Sturge-Weber syndrome in a 14-year-old girl without facial naevus. Eur J Pediatr. 2002;161:505-6 pubmed
  91. Eibschitz Tsimhoni M, Lichter P, Del Monte M, Archer S, Musch D, Schertzer R, et al. Assessing the need for posterior sclerotomy at the time of filtering surgery in patients with Sturge-Weber syndrome. Ophthalmology. 2003;110:1361-3 pubmed
    ..In this study, we evaluated this unproven clinical perception...
  92. Baselga E. Sturge-Weber syndrome. Semin Cutan Med Surg. 2004;23:87-98 pubmed
    ..Early neuroimaging features are important to recognize. Management of patients with Sturge-Weber syndrome is focused on treating associated neurologic and ocular abnormalities...