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CASE REPORT article

Front. Surg.
Sec. Neurosurgery
Volume 11 - 2024 | doi: 10.3389/fsurg.2024.1409692

Case Report: A Rare Presentation of Rapidly Progressive Moyamoya Disease Refractory to Unilateral Surgical Revascularization

Provisionally accepted
Daniel F. Leach III Daniel F. Leach III 1*Srivikram Margam S Srivikram Margam S 2Aaron J. Gustin Aaron J. Gustin 3*Paul J. Gustin Paul J. Gustin 3*Mohamad N. Jajeh Mohamad N. Jajeh 4*Yhana C. Chavis Yhana C. Chavis 5*Kristin V. Walker Kristin V. Walker 5*Josh Bentley Josh Bentley 4*
  • 1 Department of Radiation Oncology, School of Medicine, University of Virginia, Charlottesville, United States
  • 2 Alabama College of Osteopathic Medicine, Dothan, Alabama, United States
  • 3 Carle BroMenn Medical Center, Normal, United States
  • 4 Southeast Health Medical Center, Dothan, Alabama, United States
  • 5 University of Virginia Hospital, Charlottesville, Virginia, United States

The final, formatted version of the article will be published soon.

    Moyamoya disease (MMD) is a chronic, occlusive cerebrovasculopathy typified by progressive steno-occlusive disease of the intracranial internal carotid arteries (ICAs) and their proximal branches. Moyamoya syndrome (MMS) categorizes patients with characteristic MMD plus associated conditions. As such, the most usual presentations are those that occur with cerebral ischemia, specifically transient ischemic attack, acute ischemic stroke, and seizures. Hemorrhagic stroke, headaches, and migraines can also occur secondary to the compensatory growth of fragile collateral vessels propagated by chronic cerebral ischemia. While the pathophysiology of MMD is unknown, there remain numerous clinical associations including radiation therapy to the brain, inherited genetic syndromes, hematologic disorders, and autoimmune conditions. We describe the case of a 31-year-old woman who presented with recurrent ischemic cerebral infarcts secondary to rapidly progressive, bilateral MMD despite undergoing early unilateral surgical revascularization with direct arterial bypass. She had numerous metabolic conditions and rapidly decompensated, ultimately passing away despite intensive and aggressive interventions. The present case highlights that progression of moyamoya disease to bilateral involvement can occur very rapidly, within a mere 6 weeks, a phenomenon which has not been documented in the literature to our knowledge.

    Keywords: Moyamoya Disease, ischemic stroke, cervicocerebral catheter angiography, pre-operative Suzuki angiography staging, Surgical revascularization, post-operative Matsushima grade

    Received: 30 Mar 2024; Accepted: 11 Jul 2024.

    Copyright: © 2024 Leach III, Margam S, Gustin, Gustin, Jajeh, Chavis, Walker and Bentley. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence:
    Daniel F. Leach III, Department of Radiation Oncology, School of Medicine, University of Virginia, Charlottesville, United States
    Aaron J. Gustin, Carle BroMenn Medical Center, Normal, United States
    Paul J. Gustin, Carle BroMenn Medical Center, Normal, United States
    Mohamad N. Jajeh, Southeast Health Medical Center, Dothan, 36301, Alabama, United States
    Yhana C. Chavis, University of Virginia Hospital, Charlottesville, 22903, Virginia, United States
    Kristin V. Walker, University of Virginia Hospital, Charlottesville, 22903, Virginia, United States
    Josh Bentley, Southeast Health Medical Center, Dothan, 36301, Alabama, United States

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