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Moyamoya
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Progressive stenoocclusive arteriopathy, spontaneous occlusion of circle of Willis
          • Definitions

            • Progressive narrowing of distal internal carotid artery (ICA) & proximal circle of Willis (COW) vessels → characteristic adjacent clusters of collateral flow appearing as "puff of smoke" on real-time angiography
            • Moyamoya disease: Primary (idiopathic) moyamoya
              • More common in Japan, Korea
            • Moyamoya arteriopathy (a.k.a. moyamoya syndrome or secondary moyamoya) occurs in association with other disorders or after radiation treatment

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Ultrasonographic Findings

                  • Angiographic Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              • Reporting Tips

                                                Selected References

                                                1. Blauwblomme T et al: Cerebral blood flow improvement after indirect revascularization for pediatric moyamoya disease: a statistical analysis of arterial spin-labeling MRI. AJNR Am J Neuroradiol. 37(4):706-12, 2016
                                                2. Kim JS: Moyamoya disease: epidemiology, clinical features, and diagnosis. J Stroke. 18(1):2-11, 2016
                                                3. Takagi Y et al: Histopathological characteristics of distal middle cerebral artery in adult and pediatric patients with moyamoya disease. Neurol Med Chir (Tokyo). ePub, 2016
                                                4. Titsworth WL et al: National analysis of 2454 pediatric moyamoya admissions and the effect of hospital volume on outcomes. Stroke. 47(5):1303-11, 2016
                                                5. Griessenauer CJ et al: Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease. J Neurosurg Pediatr. 16(1):64-73, 2015
                                                6. Derdeyn CP: Direct bypass reduces the risk of recurrent hemorrhage in moyamoya syndrome, but effect on functional outcome is less certain. Stroke. 45(5):1245-6, 2014
                                                7. Ryoo S et al: High-resolution magnetic resonance wall imaging findings of moyamoya disease. Stroke. 45(8):2457-60, 2014
                                                8. Hishikawa T et al: Assessment of the difference in posterior circulation involvement between pediatric and adult patients with moyamoya disease. J Neurosurg. 119(4):961-5, 2013
                                                9. Noguchi T et al: Arterial spin-labeling MR imaging in moyamoya disease compared with clinical assessments and other MR imaging findings. Eur J Radiol. 82(12):e840-7, 2013
                                                10. Currie S et al: Childhood moyamoya disease and moyamoya syndrome: a pictorial review. Pediatr Neurol. 44(6):401-13, 2011
                                                11. Mugikura S et al: Posterior circulation and high prevalence of ischemic stroke among young pediatric patients with Moyamoya disease: evidence of angiography-based differences by age at diagnosis. AJNR Am J Neuroradiol. 32(1):192-8, 2011
                                                12. Ibrahimi DM et al: Moyamoya disease in children. Childs Nerv Syst. 26(10):1297-308, 2010
                                                13. Kim SK et al: Pediatric moyamoya disease: an analysis of 410 consecutive cases. Ann Neurol. 68(1):92-101, 2010
                                                14. Mori N et al: The leptomeningeal "ivy sign" on fluid-attenuated inversion recovery MR imaging in Moyamoya disease: a sign of decreased cerebral vascular reserve? AJNR Am J Neuroradiol. 30(5):930-5, 2009
                                                15. Park TS: Moyamoya disease in children. Neurosurg Focus. 24(2):E16a; discussion E16a, 2008
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Pathology

                                                      • Clinical Issues

                                                        TERMINOLOGY

                                                        • Synonyms

                                                          • Progressive stenoocclusive arteriopathy, spontaneous occlusion of circle of Willis
                                                        • Definitions

                                                          • Progressive narrowing of distal internal carotid artery (ICA) & proximal circle of Willis (COW) vessels → characteristic adjacent clusters of collateral flow appearing as "puff of smoke" on real-time angiography
                                                          • Moyamoya disease: Primary (idiopathic) moyamoya
                                                            • More common in Japan, Korea
                                                          • Moyamoya arteriopathy (a.k.a. moyamoya syndrome or secondary moyamoya) occurs in association with other disorders or after radiation treatment

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Angiographic Findings

                                                                  • Nuclear Medicine Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Staging, Grading, & Classification

                                                                            • Gross Pathologic & Surgical Features

                                                                              • Microscopic Features

                                                                                CLINICAL ISSUES

                                                                                • Presentation

                                                                                  • Demographics

                                                                                    • Natural History & Prognosis

                                                                                      • Treatment

                                                                                        DIAGNOSTIC CHECKLIST

                                                                                        • Consider

                                                                                          • Image Interpretation Pearls

                                                                                            • Reporting Tips

                                                                                              Selected References

                                                                                              1. Blauwblomme T et al: Cerebral blood flow improvement after indirect revascularization for pediatric moyamoya disease: a statistical analysis of arterial spin-labeling MRI. AJNR Am J Neuroradiol. 37(4):706-12, 2016
                                                                                              2. Kim JS: Moyamoya disease: epidemiology, clinical features, and diagnosis. J Stroke. 18(1):2-11, 2016
                                                                                              3. Takagi Y et al: Histopathological characteristics of distal middle cerebral artery in adult and pediatric patients with moyamoya disease. Neurol Med Chir (Tokyo). ePub, 2016
                                                                                              4. Titsworth WL et al: National analysis of 2454 pediatric moyamoya admissions and the effect of hospital volume on outcomes. Stroke. 47(5):1303-11, 2016
                                                                                              5. Griessenauer CJ et al: Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease. J Neurosurg Pediatr. 16(1):64-73, 2015
                                                                                              6. Derdeyn CP: Direct bypass reduces the risk of recurrent hemorrhage in moyamoya syndrome, but effect on functional outcome is less certain. Stroke. 45(5):1245-6, 2014
                                                                                              7. Ryoo S et al: High-resolution magnetic resonance wall imaging findings of moyamoya disease. Stroke. 45(8):2457-60, 2014
                                                                                              8. Hishikawa T et al: Assessment of the difference in posterior circulation involvement between pediatric and adult patients with moyamoya disease. J Neurosurg. 119(4):961-5, 2013
                                                                                              9. Noguchi T et al: Arterial spin-labeling MR imaging in moyamoya disease compared with clinical assessments and other MR imaging findings. Eur J Radiol. 82(12):e840-7, 2013
                                                                                              10. Currie S et al: Childhood moyamoya disease and moyamoya syndrome: a pictorial review. Pediatr Neurol. 44(6):401-13, 2011
                                                                                              11. Mugikura S et al: Posterior circulation and high prevalence of ischemic stroke among young pediatric patients with Moyamoya disease: evidence of angiography-based differences by age at diagnosis. AJNR Am J Neuroradiol. 32(1):192-8, 2011
                                                                                              12. Ibrahimi DM et al: Moyamoya disease in children. Childs Nerv Syst. 26(10):1297-308, 2010
                                                                                              13. Kim SK et al: Pediatric moyamoya disease: an analysis of 410 consecutive cases. Ann Neurol. 68(1):92-101, 2010
                                                                                              14. Mori N et al: The leptomeningeal "ivy sign" on fluid-attenuated inversion recovery MR imaging in Moyamoya disease: a sign of decreased cerebral vascular reserve? AJNR Am J Neuroradiol. 30(5):930-5, 2009
                                                                                              15. Park TS: Moyamoya disease in children. Neurosurg Focus. 24(2):E16a; discussion E16a, 2008