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Moyamoya Disease

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Neurovascular Sonography

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Abstract

Moyamoya disease (MMD) is a rare disease, characterized by chronic progressive steno-occlusion at the terminal portion of the internal carotid artery, proximal portion of the anterior cerebral artery, and the middle cerebral artery (MCA) with concomitant abnormal collateral networks (‘puff of smoke’ appearance on angiography). The exact pathogenesis of MMD is unknown, partly due to lack of animal models. The clinical picture of MMD is complex, depending on age and underlying mechanism (steno-occlusion vs collateral vessel network). Transcranial Doppler sonography (TCD) is non-invasive, reliable method to evaluate intracranial stenosis or occlusion. TCD criteria for the diagnosis of intracranial stenosis include circumscribed flow velocity increase, distal signal damping, and side-to-side differences in velocity. The clinical role of microembolic signals (MES) in MMD has yet to be identified. Cerebral hemodynamics have been intensively studied in MMD suggesting that a decreased cerebrovascular reserve predicts an increased risk of ischemic events. Vasomotor reactivity can be assessed with TCD by measuring changes in flow velocities in response to acetazolamide injection, hyperventilation, or CO2 inhalation. For adults with ischemic MMD, antiplatelet agents (mostly aspirin) can be recommended.

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Authors and Affiliations

  1. The Ohio State University Wexner Medical Center, Columbus, OH, USA

    Deepak Gulati

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  1. Deepak Gulati
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Correspondence to Deepak Gulati .

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Editors and Affiliations

  1. Departments of Neurology, Neurosurgery, Anesthesiology and Critical Care Medicine, The Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Wendy C. Ziai

  2. Neurosonology Consultant Cardiovascular Ultrasound Services, Inc., Columbus, OH, USA

    Christy L. Cornwell

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Gulati, D. (2022). Moyamoya Disease. In: Ziai, W.C., Cornwell, C.L. (eds) Neurovascular Sonography . Springer, Cham. https://doi.org/10.1007/978-3-030-96893-9_23

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  • DOI: https://doi.org/10.1007/978-3-030-96893-9_23

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-96892-2

  • Online ISBN: 978-3-030-96893-9

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