Case Report: A rare presentation of rapidly progressive moyamoya disease refractory to unilateral surgical revascularization
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 suc...
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Frontiers Media S.A.
2024-08-01
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| Series: | Frontiers in Surgery |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fsurg.2024.1409692/full |
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| author | Daniel Friel Leach Srivikram Margam S Aaron Gustin Paul J. Gustin Mohamad N. Jajeh Yhana C. Chavis Kristin V. Walker Joshua S. Bentley |
| author_facet | Daniel Friel Leach Srivikram Margam S Aaron Gustin Paul J. Gustin Mohamad N. Jajeh Yhana C. Chavis Kristin V. Walker Joshua S. Bentley |
| author_sort | Daniel Friel Leach |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-e66793c2ee194d5d9e233b58dfb847f7 |
| institution | Kabale University |
| issn | 2296-875X |
| language | English |
| publishDate | 2024-08-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Surgery |
| spelling | doaj-art-e66793c2ee194d5d9e233b58dfb847f72025-01-10T04:11:04ZengFrontiers Media S.A.Frontiers in Surgery2296-875X2024-08-011110.3389/fsurg.2024.14096921409692Case Report: A rare presentation of rapidly progressive moyamoya disease refractory to unilateral surgical revascularizationDaniel Friel Leach0Srivikram Margam S1Aaron Gustin2Paul J. Gustin3Mohamad N. Jajeh4Yhana C. Chavis5Kristin V. Walker6Joshua S. Bentley7Department of Radiation Oncology, University of Virginia Health, Charlottesville, VA, United StatesResearch, Alabama College of Osteopathic Medicine, Dothan, AL, United StatesNeurological Surgery, Carle BroMenn Medical Center, Normal, IL, United StatesNeurological Surgery, Carle BroMenn Medical Center, Normal, IL, United StatesInternal Medicine, Southeast Health, Dothan, AL, United StatesDepartment of Radiation Oncology, University of Virginia Health, Charlottesville, VA, United StatesDepartment of Radiation Oncology, University of Virginia Health, Charlottesville, VA, United StatesCerebrovascular and Endovascular Neurosurgery, Southeast Health, Dothan, AL, United StatesMoyamoya 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.https://www.frontiersin.org/articles/10.3389/fsurg.2024.1409692/fullmoyamoya diseaseischemic strokecervicocerebral catheter angiographypre-operative Suzuki angiography stagingsurgical revascularizationpost-operative Matsushima grade |
| spellingShingle | Daniel Friel Leach Srivikram Margam S Aaron Gustin Paul J. Gustin Mohamad N. Jajeh Yhana C. Chavis Kristin V. Walker Joshua S. Bentley Case Report: A rare presentation of rapidly progressive moyamoya disease refractory to unilateral surgical revascularization Frontiers in Surgery moyamoya disease ischemic stroke cervicocerebral catheter angiography pre-operative Suzuki angiography staging surgical revascularization post-operative Matsushima grade |
| title | Case Report: A rare presentation of rapidly progressive moyamoya disease refractory to unilateral surgical revascularization |
| title_full | Case Report: A rare presentation of rapidly progressive moyamoya disease refractory to unilateral surgical revascularization |
| title_fullStr | Case Report: A rare presentation of rapidly progressive moyamoya disease refractory to unilateral surgical revascularization |
| title_full_unstemmed | Case Report: A rare presentation of rapidly progressive moyamoya disease refractory to unilateral surgical revascularization |
| title_short | Case Report: A rare presentation of rapidly progressive moyamoya disease refractory to unilateral surgical revascularization |
| title_sort | case report a rare presentation of rapidly progressive moyamoya disease refractory to unilateral surgical revascularization |
| topic | moyamoya disease ischemic stroke cervicocerebral catheter angiography pre-operative Suzuki angiography staging surgical revascularization post-operative Matsushima grade |
| url | https://www.frontiersin.org/articles/10.3389/fsurg.2024.1409692/full |
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