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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. Maraña Pérez AI et al: Analysis of Sturge-Weber syndrome: a retrospective study of multiple associated variables. Neurologia. ePub, 2016
                                              2. Comi AM: Sturge-Weber syndrome. Handb Clin Neurol. 132:157-68, 2015
                                              3. Pinto AL et al: Sturge-Weber syndrome: brain magnetic resonance imaging and neuropathology findings. Pediatr Neurol. 58:25-30, 2015
                                              4. Sudarsanam A et al: Sturge-Weber syndrome: from the past to the present. Eur J Paediatr Neurol. 18(3):257-66, 2014
                                              5. 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
                                              6. Fogarasi A et al: Sturge-Weber syndrome: clinical and radiological correlates in 86 patients. Ideggyogy Sz. 66(1-2):53-7, 2013
                                              7. 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
                                              8. 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
                                              9. 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
                                              10. 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
                                              11. 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
                                              12. George U et al: MR demonstration of accelerated myelination in early sturge Weber syndrome. Neurol India. 58(2):336-7, 2010
                                              13. 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
                                              14. Pascual-Castroviejo I et al: Sturge-Weber syndrome: study of 55 patients. Can J Neurol Sci. 35(3):301-7, 2008
                                              15. 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. Maraña Pérez AI et al: Analysis of Sturge-Weber syndrome: a retrospective study of multiple associated variables. Neurologia. ePub, 2016
                                                                                          2. Comi AM: Sturge-Weber syndrome. Handb Clin Neurol. 132:157-68, 2015
                                                                                          3. Pinto AL et al: Sturge-Weber syndrome: brain magnetic resonance imaging and neuropathology findings. Pediatr Neurol. 58:25-30, 2015
                                                                                          4. Sudarsanam A et al: Sturge-Weber syndrome: from the past to the present. Eur J Paediatr Neurol. 18(3):257-66, 2014
                                                                                          5. 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
                                                                                          6. Fogarasi A et al: Sturge-Weber syndrome: clinical and radiological correlates in 86 patients. Ideggyogy Sz. 66(1-2):53-7, 2013
                                                                                          7. 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
                                                                                          8. 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
                                                                                          9. 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
                                                                                          10. 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
                                                                                          11. 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
                                                                                          12. George U et al: MR demonstration of accelerated myelination in early sturge Weber syndrome. Neurol India. 58(2):336-7, 2010
                                                                                          13. 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
                                                                                          14. Pascual-Castroviejo I et al: Sturge-Weber syndrome: study of 55 patients. Can J Neurol Sci. 35(3):301-7, 2008
                                                                                          15. 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