Since its initial description in 1957 as an idiopathic disease, moyamoya disease has proved challenging to treat. Although the basic pathophysiology of this disease involves narrowing of the terminal carotid artery with compensatory angiogenesis, the molecular and cellular mechanisms underlying these changes are far more complex. In this article, the authors review the literature on the molecular and cellular pathophysiology of moyamoya disease with an emphasis on potential therapeutic targets.
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| http://dx.doi.org/10.3171/2021.6.FOCUS21289 | DOI Listing |
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Cerebrovascular reactivity: a stress test of brain vascular health in moyamoya disease.
Brain Commun
January 2025
Athinoula A. Martinos Center for Biomedical Imaging, Massachusetts General Hospital, Charlestown, MA 02129, USA.
This scientific commentary refers to 'Cerebrovascular reactivity and response times describe recent ischemic symptomatology in patients with moyamoya', by Han . (https://doi.org/10.
View Article and Find Full Text PDFCerebrovascular reactivity and response times describe recent ischaemic symptomatology in patients with moyamoya.
Brain Commun
November 2024
Department of Neurology, Vanderbilt University Medical Center, Nashville, TN 37232, USA.
Moyamoya is a non-atherosclerotic intracranial steno-occlusive condition that places patients at high risk for ischaemic stroke. Randomized trials of surgical revascularization demonstrating efficacy in ischaemic moyamoya have not been performed, and as such, biomarkers of parenchymal haemodynamic impairment are needed to assist with triage and evaluate post-surgical response. In this prospective study, we test the hypothesis that parenchymal cerebrovascular reactivity (CVR) metrics in response to a fixed-inspired 5% carbon dioxide challenge correlate with recent focal ischaemic symptoms.
View Article and Find Full Text PDFEffects of Scalp Nerve Block on Symptomatic Cerebral Hyperperfusion Syndrome After Combined Revascularization Surgery for Moyamoya Disease.
J Neurosurg Anesthesiol
January 2025
Departments of Anesthesiology and Pain Medicine.
Background: Strict blood pressure control can be used to prevent or treat cerebral hyperperfusion syndrome. This study investigated whether scalp nerve block (SNB) is associated with a reduced risk of postoperative symptomatic cerebral hyperperfusion syndrome (SCHS) by reducing postoperative blood pressure in adult patients who underwent combined revascularization surgery for moyamoya disease.
Methods: Patients were retrospectively divided into the SNB (n=167) and control (n=221) groups depending on whether SNB was performed immediately before placement of wound dressings at the end of surgery.
Identification of Sulcal Hyperintense Vessel (Vessel Wall MR Ivy Sign) in Adult Moyamoya Disease with High-resolution Vessel Wall Imaging : A Pilot Study.
J Korean Neurosurg Soc
January 2025
Department of Neurosurgery, Kyung Hee University Hospital, Kyung Hee University College of Medicine, Seoul, Korea.
Objective: The leptomeningeal ivy sign is a distinctive finding of moyamoya disease (MMD), characterized by a linear high signal intensity along the cortical sulci on contrast-enhanced T1 magnetic resonance imaging (MRI) and fluid-attenuated inversion-recovery MRI. We recently identified a similar linear enhancement along the cortical sulci using gadolinium-enhanced vessel wall MRI (VWMR) in patients with MMD. The aim of this study was to introduce the concept of the "VWMR ivy sign (VIS)".
View Article and Find Full Text PDFIntra-individual comparison of long-term outcomes between combined and indirect bypass surgery in adult moyamoya disease.
Acta Neurochir (Wien)
January 2025
Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea.
Purpose: Bypass surgery is regarded as the standard treatment option for symptomatic and hemodynamically unstable moyamoya disease (MMD). However, there is ongoing debate about the most effective type of bypass surgery. We aimed to analyze the long-term outcomes of combined and indirect bypasses for MMD patients through intra-individual comparisons.
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