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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
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
- 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: Retrospective review of screening for Sturge-Weber syndrome with brain magnetic resonance imaging and electroencephalography in infants with high-risk port-wine stains. Pediatr Dermatol. 35(5):575-81, 2018
- Zallmann M et al: Screening for Sturge-Weber syndrome: a state-of-the-art review. Pediatr Dermatol. 35(1):30-42, 2018
- Maraña Pérez AI et al: Analysis of Sturge-Weber syndrome: a retrospective study of multiple associated variables. Neurologia. 32(6):363-70, 2017
- Pilli VK et al: Clinical and metabolic correlates of cerebral calcifications in Sturge-Weber syndrome. Dev Med Child Neurol. 59(9):952-8, 2017
- 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
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
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
- 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: Retrospective review of screening for Sturge-Weber syndrome with brain magnetic resonance imaging and electroencephalography in infants with high-risk port-wine stains. Pediatr Dermatol. 35(5):575-81, 2018
- Zallmann M et al: Screening for Sturge-Weber syndrome: a state-of-the-art review. Pediatr Dermatol. 35(1):30-42, 2018
- Maraña Pérez AI et al: Analysis of Sturge-Weber syndrome: a retrospective study of multiple associated variables. Neurologia. 32(6):363-70, 2017
- Pilli VK et al: Clinical and metabolic correlates of cerebral calcifications in Sturge-Weber syndrome. Dev Med Child Neurol. 59(9):952-8, 2017
- 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
