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Unilateral Moyamoya Disease vs. Asymmetric Manifestations of Definite Moyamoya disease: Angiographic Pattern Analysis wi

Bilateral progressive steno-occlusive changes of supraclinoid ICA segments involving carotid fork region; Unilateral involvements (overall 20%) are also seen

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Unilateral Moyamoya Disease vs. Asymmetric Manifestations of Definite Moyamoya disease: Angiographic Pattern Analysis wi

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    1. CHUNG, J.I., Department of Radiology, Suncheon Pyunghwa Hospital, Suncheon City, Republic of Korea WEON, Y.C., Department of Radiology, Samsung Medical Center, Seoul, Republic of Korea Unilateral Moyamoya Disease vs. Asymmetric Manifestations of Definite Moyamoya disease: Angiographic Pattern Analysis with Its Pathognomonic Findings

    2. Bilateral progressive steno-occlusive changes of supraclinoid ICA segments involving carotid fork region; Unilateral involvements (overall 20%) are also seen “Puff of Smoke”; (1) Classical basal collaterals recruiting leptomeningeal vessels and deep parenchymal vessels of striatum (2) Collateral vessels are formed during prenatal period Pathophysiology; Fibrocellular intimal thickenings with wavy internal elastic lamina and thinning of media Moyamoya disease (MMD)

    3. Etiology; Unknown Some Genetic Predispositions; 10% of familial incidence, hereditary and multifactorial, autosomal dominant familial MMD with reduced penetrance Genetically Susceptible Loci; 3p, 6p, 17q, and band 8q23 Angiographic Classification of MMD; Suzuki & Takaku since 1969, but often fail to represent the disease progression Controversies about its etiologies as congenital vs. acquired Moyamoya disease (MMD)

    4. Moyamoya disease (MMD)

    5. Visualization of primitive embryonic arteries with remnant anastomosis and annexations : crucial to understand the pathophysiologic mechanisms Pathophysiologic target arterial segment could be located in proximal MCA just distal to AChA bifurcation during 14-20mm embryo stage: Stage IV & V, Padget DH Persisted and annexed embryonic vessels which depicted as collaterals in MMD patients: reveal the time specific occlusive changes of carotid arteries and show adjusted hemodynamic balances during the early embryonic period. Moyamoya disease (MMD)

    6. Moyamoya disease (MMD)

    7. Moyamoya disease (MMD)

    8. Progression of Unilateral MMD: A Clinical Series. Kelly ME et al. Cerebrovasc Dis 2006;22:109-15 7/18 (38.9 %) of Angiographic Progression 6/7 (85.7 %) of Initial Carotid Fork Abnormalities Presence of contralateral carotid fork abnormalities are important prognostic factors of progression “True definition of Unilateral MMD is Unclear” Unilateral MMD vs. Asymmetric Definite MMD

    9. Anatomic configuration of the cerebral vessels of Unilateral MMD : Presence of Contralateral carotid fork abnormalities & Remnant embryonic annexations and anastomosis represented as Collaterals “Undoubtedly plays a role in outcome” Immediate Surgery for Moyamoya Syndrome? Not Necessarily Roach ES. Arch Neurol 2001;58:130-31 Try to discern the Pathognomonic Angiographic Manifestations Verifications and Predictable Natural History about MMD “ true definition of Unilateral MMD ?” Unilateral MMD vs. Asymmetric Definite MMD

    10. March 2002 to April 2004 Angiographic Analysis: Independently Reviewed by Two Experienced Neuroradiologists (C.J.I & W.Y.C.) Based upon the Significant Contributions by Dorcas Hager Padget (1906-1973) “The development of the cranial arteries in the human embryo. Contrib Embryol 32:205-261, 1948” Analysis of the Pathognomonic Angiographic Manifestations Carotid Fork Appearances Identification and Classification of Remnant Embryonic Anastomosis and Annexations Retrospective Review 204 Angiograms (102 MMD patients)

    11. All 20 patient showed Asymmetric Contralateral Carotid Fork Abnormalities with Typical MMD Collaterals Disproportional Vessel Caliber Changes (2/19, 10.5%) and Mild degree Stenosis of Carotid Fork Region (17/19, 89.5%) Typical MMD Collaterals POlfA with or without ORM (ophthalmic rete mirabile): 8/19, 42.1% Ophthalmic Ethmoidal Collaterals: 2/19, 10.5% Callosal Artery Collaterals: 3/19, 15.8% 20 (19.6%) of Unilateral MMD vs. Asymmetric Definite MMD

    12. Disproportional Vessel Caliber Changes

    13. Mild Degree Stenosis of Carotid Fork Regions: A1 segment narrowings POlfA Collaterals

    14. Typical MMD Collaterals of POlfA with or without ORM (Ophthalmic Rete Mirabile) POlfA Collaterals Basal Collaterals

    15. Ophthalmic Ethmoidal Collaterals Basal Collaterals

    16. Callosal Artery Collaterals POlfA Collaterals with ORM (Ophthalmic Rete Mirabile)

    17. The definition of Unilateral MMD is Incorrect MMD is Bilateral Disease without Exception, but Sometimes do Manifests as Asymmetric Involvements Must Understand the Characteristic Angiographic Findings of Typical MMD Collaterals: Remnant Embryonic Anastomosis and Annexations Pathognomonic Angiographic Findings of Asymmetric MMD Carotid Fork Abnormalities Identification and Understand the patterns of Remnant Embryonic Anastomosis and Annexations Unilateral MMD vs. Asymmetric Definite MMD

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