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Sturge-Weber Syndrome
Luke L. Linscott, MD
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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

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

                                                        • 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

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