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Case Reports
. 2024 Aug 16:11:1409692.
doi: 10.3389/fsurg.2024.1409692. eCollection 2024.

Case Report: A rare presentation of rapidly progressive moyamoya disease refractory to unilateral surgical revascularization

Daniel Friel Leach 3rd  1 , Srivikram Margam  2 , Aaron Gustin  3 , Paul J Gustin  3 , Mohamad N Jajeh  4 , Yhana C Chavis  1 , Kristin V Walker  1 , Joshua S Bentley  5
Affiliations
Case Reports

Case Report: A rare presentation of rapidly progressive moyamoya disease refractory to unilateral surgical revascularization

Daniel Friel Leach 3rd et al. Front Surg. .

Erratum in

Abstract

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: cervicocerebral catheter angiography; ischemic stroke; moyamoya disease; post-operative Matsushima grade; pre-operative Suzuki angiography staging; surgical revascularization.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Preoperative MRI brain without contrast, DWI (A) and ADC (B) sequences, demonstrating acute ischemia involving the left frontal lobe at the ACA-MCA border zone.
Figure 2
Figure 2
Preoperative DSA of the right ICA, anteroposterior (AP) (A) and lateral (B) views, early arterial phase, demonstrating occlusion of the right distal intracranial ICA distal to the pCoA, and AP (C) and lateral (D) views, late arterial phase, demonstrating leptomeningeal collateralization. DSA of the right VA, AP (E) and lateral (F) views, late arterial phase, demonstrating collateralization to the MCA and ACA territories. DSA of the left ICA, AP (G) and lateral (H) views, early arterial phase, demonstrating moderate stenosis of the distal ICA and proximal MCA and ACA with lenticulostriate collateralization.
Figure 3
Figure 3
Postoperative MRI brain without contrast, DWI (A) and ADC (B) sequences, demonstrating acute ischemia involving the bilateral ACA and MCA territories.
Figure 4
Figure 4
Postoperative DSA of the left ICA, AP (A) and lateral (B) views, demonstrating occlusion of the left distal intracranial ICA distal to the pCoA.
See this image and copyright information in PMC

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