Bookmarks
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Sturge-Weber syndrome (SWS)
Synonyms
- Sturge-Weber-Dimitri, encephalotrigeminal angiomatosis
Definitions
- Congenital (but not inherited) syndrome in which cortical veins fail to develop normally, leading to numerous long-term intracranial manifestations of chronic ischemia & expanded alternate routes of venous drainage
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Sabeti S et al: Consensus statement for the management and treatment of sturge-weber syndrome: neurology, neuroimaging, and ophthalmology recommendations. Pediatr Neurol. 121:59-66, 2021
- Brinjikji W et al: Cerebrofacial venous metameric syndrome-spectrum of imaging findings. Neuroradiology. 62(4):417-25, 2020
- Andica C et al: Aberrant myelination in patients with Sturge-Weber syndrome analyzed using synthetic quantitative magnetic resonance imaging. Neuroradiology. 61(9):1055-66, 2019
- Barros FS et al: The rare neurocutaneous disorders: update on clinical, molecular, and neuroimaging features. Top Magn Reson Imaging. 27(6):433-62, 2018
- Warne RR et al: The bone does not predict the brain in Sturge-Weber syndrome. AJNR Am J Neuroradiol. 39(8):1543-9, 2018
- Zallmann M et al: Screening for Sturge-Weber syndrome: a state-of-the-art review. Pediatr Dermatol. 35(1):30-42, 2018
- Pilli VK et al: Clinical and metabolic correlates of cerebral calcifications in Sturge-Weber syndrome. Dev Med Child Neurol. 59(9):952-8, 2017
- Maraña Pérez AI et al: Analysis of Sturge-Weber syndrome: a retrospective study of multiple associated variables. Neurologia. 32(6):363-70, 2016
- Comi AM: Sturge-Weber syndrome. Handb Clin Neurol. 132:157-68, 2015
- Pinto AL et al: Sturge-Weber syndrome: brain magnetic resonance imaging and neuropathology findings. Pediatr Neurol. 58:25-30, 2015
- Sudarsanam A et al: Sturge-Weber syndrome: from the past to the present. Eur J Paediatr Neurol. 18(3):257-66, 2014
- Waelchli R et al: New vascular classification of port-wine stains: improving prediction of Sturge-Weber risk. Br J Dermatol. 171(4):861-7, 2014
- Fogarasi A et al: Sturge-Weber syndrome: clinical and radiological correlates in 86 patients. Ideggyogy Sz. 66(1-2):53-7, 2013
- Shirley MD et al: Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med. 368(21):1971-9, 2013
- Lo W et al: Updates and future horizons on the understanding, diagnosis, and treatment of Sturge-Weber syndrome brain involvement. Dev Med Child Neurol. 2012 Mar;54(3):214-23, 2012
- Alkonyi B et al: Transient focal cortical increase of interictal glucose metabolism in Sturge-Weber syndrome: implications for epileptogenesis. Epilepsia. 52(7):1265-72, 2011
- Wu J et al: Cortical calcification in Sturge-Weber syndrome on MRI-SWI: relation to brain perfusion status and seizure severity. J Magn Reson Imaging. 34(4):791-8, 2011
- Arulrajah S et al: MRI with diffusion-weighted imaging in children and young adults with simultaneous supra- and infratentorial manifestations of Sturge-Weber syndrome. J Neuroradiol. 37(1):51-9, 2010
- George U et al: MR demonstration of accelerated myelination in early sturge Weber syndrome. Neurol India. 58(2):336-7, 2010
- Hu J et al: MR susceptibility weighted imaging (SWI) complements conventional contrast enhanced T1 weighted MRI in characterizing brain abnormalities of Sturge-Weber Syndrome. J Magn Reson Imaging. 28(2):300-7, 2008
- Pascual-Castroviejo I et al: Sturge-Weber syndrome: study of 55 patients. Can J Neurol Sci. 35(3):301-7, 2008
- Lin DD et al: Dynamic MR perfusion and proton MR spectroscopic imaging in Sturge-Weber syndrome: correlation with neurological symptoms. J Magn Reson Imaging. 24(2):274-81, 2006
Related Anatomy
Loading...
Related Differential Diagnoses
Loading...
References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Sturge-Weber syndrome (SWS)
Synonyms
- Sturge-Weber-Dimitri, encephalotrigeminal angiomatosis
Definitions
- Congenital (but not inherited) syndrome in which cortical veins fail to develop normally, leading to numerous long-term intracranial manifestations of chronic ischemia & expanded alternate routes of venous drainage
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Sabeti S et al: Consensus statement for the management and treatment of sturge-weber syndrome: neurology, neuroimaging, and ophthalmology recommendations. Pediatr Neurol. 121:59-66, 2021
- Brinjikji W et al: Cerebrofacial venous metameric syndrome-spectrum of imaging findings. Neuroradiology. 62(4):417-25, 2020
- Andica C et al: Aberrant myelination in patients with Sturge-Weber syndrome analyzed using synthetic quantitative magnetic resonance imaging. Neuroradiology. 61(9):1055-66, 2019
- Barros FS et al: The rare neurocutaneous disorders: update on clinical, molecular, and neuroimaging features. Top Magn Reson Imaging. 27(6):433-62, 2018
- Warne RR et al: The bone does not predict the brain in Sturge-Weber syndrome. AJNR Am J Neuroradiol. 39(8):1543-9, 2018
- Zallmann M et al: Screening for Sturge-Weber syndrome: a state-of-the-art review. Pediatr Dermatol. 35(1):30-42, 2018
- Pilli VK et al: Clinical and metabolic correlates of cerebral calcifications in Sturge-Weber syndrome. Dev Med Child Neurol. 59(9):952-8, 2017
- Maraña Pérez AI et al: Analysis of Sturge-Weber syndrome: a retrospective study of multiple associated variables. Neurologia. 32(6):363-70, 2016
- Comi AM: Sturge-Weber syndrome. Handb Clin Neurol. 132:157-68, 2015
- Pinto AL et al: Sturge-Weber syndrome: brain magnetic resonance imaging and neuropathology findings. Pediatr Neurol. 58:25-30, 2015
- Sudarsanam A et al: Sturge-Weber syndrome: from the past to the present. Eur J Paediatr Neurol. 18(3):257-66, 2014
- Waelchli R et al: New vascular classification of port-wine stains: improving prediction of Sturge-Weber risk. Br J Dermatol. 171(4):861-7, 2014
- Fogarasi A et al: Sturge-Weber syndrome: clinical and radiological correlates in 86 patients. Ideggyogy Sz. 66(1-2):53-7, 2013
- Shirley MD et al: Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med. 368(21):1971-9, 2013
- Lo W et al: Updates and future horizons on the understanding, diagnosis, and treatment of Sturge-Weber syndrome brain involvement. Dev Med Child Neurol. 2012 Mar;54(3):214-23, 2012
- Alkonyi B et al: Transient focal cortical increase of interictal glucose metabolism in Sturge-Weber syndrome: implications for epileptogenesis. Epilepsia. 52(7):1265-72, 2011
- Wu J et al: Cortical calcification in Sturge-Weber syndrome on MRI-SWI: relation to brain perfusion status and seizure severity. J Magn Reson Imaging. 34(4):791-8, 2011
- Arulrajah S et al: MRI with diffusion-weighted imaging in children and young adults with simultaneous supra- and infratentorial manifestations of Sturge-Weber syndrome. J Neuroradiol. 37(1):51-9, 2010
- George U et al: MR demonstration of accelerated myelination in early sturge Weber syndrome. Neurol India. 58(2):336-7, 2010
- Hu J et al: MR susceptibility weighted imaging (SWI) complements conventional contrast enhanced T1 weighted MRI in characterizing brain abnormalities of Sturge-Weber Syndrome. J Magn Reson Imaging. 28(2):300-7, 2008
- Pascual-Castroviejo I et al: Sturge-Weber syndrome: study of 55 patients. Can J Neurol Sci. 35(3):301-7, 2008
- Lin DD et al: Dynamic MR perfusion and proton MR spectroscopic imaging in Sturge-Weber syndrome: correlation with neurological symptoms. J Magn Reson Imaging. 24(2):274-81, 2006
