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Sturge-Weber Syndrome
Luke L. Linscott, MD
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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

                                              1. 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
                                              2. Brinjikji W et al: Cerebrofacial venous metameric syndrome-spectrum of imaging findings. Neuroradiology. 62(4):417-25, 2020
                                              3. 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
                                              4. Barros FS et al: The rare neurocutaneous disorders: update on clinical, molecular, and neuroimaging features. Top Magn Reson Imaging. 27(6):433-62, 2018
                                              5. Warne RR et al: The bone does not predict the brain in Sturge-Weber syndrome. AJNR Am J Neuroradiol. 39(8):1543-9, 2018
                                              6. Zallmann M et al: Screening for Sturge-Weber syndrome: a state-of-the-art review. Pediatr Dermatol. 35(1):30-42, 2018
                                              7. Pilli VK et al: Clinical and metabolic correlates of cerebral calcifications in Sturge-Weber syndrome. Dev Med Child Neurol. 59(9):952-8, 2017
                                              8. Maraña Pérez AI et al: Analysis of Sturge-Weber syndrome: a retrospective study of multiple associated variables. Neurologia. 32(6):363-70, 2016
                                              9. Comi AM: Sturge-Weber syndrome. Handb Clin Neurol. 132:157-68, 2015
                                              10. Pinto AL et al: Sturge-Weber syndrome: brain magnetic resonance imaging and neuropathology findings. Pediatr Neurol. 58:25-30, 2015
                                              11. Sudarsanam A et al: Sturge-Weber syndrome: from the past to the present. Eur J Paediatr Neurol. 18(3):257-66, 2014
                                              12. 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
                                              13. Fogarasi A et al: Sturge-Weber syndrome: clinical and radiological correlates in 86 patients. Ideggyogy Sz. 66(1-2):53-7, 2013
                                              14. 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
                                              15. 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
                                              16. 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
                                              17. 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
                                              18. 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
                                              19. George U et al: MR demonstration of accelerated myelination in early sturge Weber syndrome. Neurol India. 58(2):336-7, 2010
                                              20. 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
                                              21. Pascual-Castroviejo I et al: Sturge-Weber syndrome: study of 55 patients. Can J Neurol Sci. 35(3):301-7, 2008
                                              22. 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
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                                              Related Differential Diagnoses
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                                              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

                                                                                          1. 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
                                                                                          2. Brinjikji W et al: Cerebrofacial venous metameric syndrome-spectrum of imaging findings. Neuroradiology. 62(4):417-25, 2020
                                                                                          3. 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
                                                                                          4. Barros FS et al: The rare neurocutaneous disorders: update on clinical, molecular, and neuroimaging features. Top Magn Reson Imaging. 27(6):433-62, 2018
                                                                                          5. Warne RR et al: The bone does not predict the brain in Sturge-Weber syndrome. AJNR Am J Neuroradiol. 39(8):1543-9, 2018
                                                                                          6. Zallmann M et al: Screening for Sturge-Weber syndrome: a state-of-the-art review. Pediatr Dermatol. 35(1):30-42, 2018
                                                                                          7. Pilli VK et al: Clinical and metabolic correlates of cerebral calcifications in Sturge-Weber syndrome. Dev Med Child Neurol. 59(9):952-8, 2017
                                                                                          8. Maraña Pérez AI et al: Analysis of Sturge-Weber syndrome: a retrospective study of multiple associated variables. Neurologia. 32(6):363-70, 2016
                                                                                          9. Comi AM: Sturge-Weber syndrome. Handb Clin Neurol. 132:157-68, 2015
                                                                                          10. Pinto AL et al: Sturge-Weber syndrome: brain magnetic resonance imaging and neuropathology findings. Pediatr Neurol. 58:25-30, 2015
                                                                                          11. Sudarsanam A et al: Sturge-Weber syndrome: from the past to the present. Eur J Paediatr Neurol. 18(3):257-66, 2014
                                                                                          12. 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
                                                                                          13. Fogarasi A et al: Sturge-Weber syndrome: clinical and radiological correlates in 86 patients. Ideggyogy Sz. 66(1-2):53-7, 2013
                                                                                          14. 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
                                                                                          15. 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
                                                                                          16. 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
                                                                                          17. 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
                                                                                          18. 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
                                                                                          19. George U et al: MR demonstration of accelerated myelination in early sturge Weber syndrome. Neurol India. 58(2):336-7, 2010
                                                                                          20. 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
                                                                                          21. Pascual-Castroviejo I et al: Sturge-Weber syndrome: study of 55 patients. Can J Neurol Sci. 35(3):301-7, 2008
                                                                                          22. 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