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Moyamoya
Anne G. Osborn, MD, FACR; Anna Illner, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Moyamoya disease (MMD)
          • Synonyms

            • Idiopathic progressive arteriopathy of childhood, spontaneous occlusion of circle of Willis (COW)
          • Definitions

            • Progressive bilateral stenosis or occlusion of terminal internal carotid artery (ICA)/proximal COW with abnormal vascular network at base of brain
              • Compensatory development of collateral vascular network = "puff of smoke"
            • Primary (idiopathic) moyamoya = moyamoya disease
              • More common Japan, Korea
            • Secondary (acquired) moyamoya
              • Cranial radiation
              • Atherosclerosis
              • Neurofibromatosis

          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. Acker G et al: Distinct clinical and radiographic characteristics of moyamoya disease amongst European Caucasians. Eur J Neurol. ePub, 2015
                                                2. Fujimura M et al: Diagnosis of moyamoya disease: international standard and regional differences. Neurol Med Chir (Tokyo). 55(3):189-93, 2015
                                                3. Guey S et al: Moyamoya disease and syndromes: from genetics to clinical management. Appl Clin Genet. 8:49-68, 2015
                                                4. Kim T et al: Morphology and related hemodynamics of the internal carotid arteries of moyamoya patients. Acta Neurochir (Wien). ePub, 2015
                                                5. 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
                                                6. Strother MK et al: Cerebrovascular collaterals correlate with disease severity in adult North American patients with moyamoya disease. AJNR Am J Neuroradiol. Epub ahead of print, 2014
                                                7. Wang C et al: Delayed cerebral vasculopathy following cranial radiation therapy for pediatric tumors. Pediatr Neurol. 50(6):549-56, 2014
                                                8. Zhao WG et al: Cerebral hyperperfusion syndrome after revascularization surgery in patients with moyamoya disease. Br J Neurosurg. 27(3):321-5, 2013
                                                9. Kamijo K et al: Dramatic disappearance of moyamoya disease-induced chorea after indirect bypass surgery. Neurol Med Chir (Tokyo). 48(9):390-3, 2008
                                                10. Kraemer M et al: Moyamoya disease in Europeans. Stroke. 39(12):3193-200, 2008
                                                11. Kuroda S et al: Moyamoya disease: current concepts and future perspectives. Lancet Neurol. 7(11):1056-66, 2008
                                                12. 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

                                                        • Abbreviations

                                                          • Moyamoya disease (MMD)
                                                        • Synonyms

                                                          • Idiopathic progressive arteriopathy of childhood, spontaneous occlusion of circle of Willis (COW)
                                                        • Definitions

                                                          • Progressive bilateral stenosis or occlusion of terminal internal carotid artery (ICA)/proximal COW with abnormal vascular network at base of brain
                                                            • Compensatory development of collateral vascular network = "puff of smoke"
                                                          • Primary (idiopathic) moyamoya = moyamoya disease
                                                            • More common Japan, Korea
                                                          • Secondary (acquired) moyamoya
                                                            • Cranial radiation
                                                            • Atherosclerosis
                                                            • Neurofibromatosis

                                                        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. Acker G et al: Distinct clinical and radiographic characteristics of moyamoya disease amongst European Caucasians. Eur J Neurol. ePub, 2015
                                                                                              2. Fujimura M et al: Diagnosis of moyamoya disease: international standard and regional differences. Neurol Med Chir (Tokyo). 55(3):189-93, 2015
                                                                                              3. Guey S et al: Moyamoya disease and syndromes: from genetics to clinical management. Appl Clin Genet. 8:49-68, 2015
                                                                                              4. Kim T et al: Morphology and related hemodynamics of the internal carotid arteries of moyamoya patients. Acta Neurochir (Wien). ePub, 2015
                                                                                              5. 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
                                                                                              6. Strother MK et al: Cerebrovascular collaterals correlate with disease severity in adult North American patients with moyamoya disease. AJNR Am J Neuroradiol. Epub ahead of print, 2014
                                                                                              7. Wang C et al: Delayed cerebral vasculopathy following cranial radiation therapy for pediatric tumors. Pediatr Neurol. 50(6):549-56, 2014
                                                                                              8. Zhao WG et al: Cerebral hyperperfusion syndrome after revascularization surgery in patients with moyamoya disease. Br J Neurosurg. 27(3):321-5, 2013
                                                                                              9. Kamijo K et al: Dramatic disappearance of moyamoya disease-induced chorea after indirect bypass surgery. Neurol Med Chir (Tokyo). 48(9):390-3, 2008
                                                                                              10. Kraemer M et al: Moyamoya disease in Europeans. Stroke. 39(12):3193-200, 2008
                                                                                              11. Kuroda S et al: Moyamoya disease: current concepts and future perspectives. Lancet Neurol. 7(11):1056-66, 2008
                                                                                              12. Park TS: Moyamoya disease in children. Neurosurg Focus. 24(2):E16a; discussion E16a, 2008