Imaging of Moyamoya Disease and Moyamoya Syndrome: Current Status

Abstract

Moyamoya disease (MMD) and Moyamoya syndrome (MMS) are referring to a progressive steno-occlusive vasculopathy at terminal portions of the bilateral internal carotid arteries and their proximal branches with prominent collateral artery formation. They can be found throughout the world and cause irreversible damage to the cerebral hemodynamics due to the progressive nature. Prompt diagnosis and accurate assessment could significantly improve the prognosis of MMD and MMS. Some imaging modalities could be used for diagnosis and nonquantitative evaluation of MMD and MMS, such as conventional computed tomography (CT) and magnetic resonance imaging (MRI), digital subtraction angiography, CT angiography (CTA), and magnetic resonance angiography. Some could quantitatively evaluate the cerebral hemodynamics of MMD and MMS, such as single-photon emission CT, positron emission tomography, xenon-enhanced CT, perfusion CT, dynamic susceptibility contrast MRI, arterial spin labeling MRI, and the hemodynamic parameters measured by those imaging methods could guide treatment of MMD and MMS. All the imaging modalities have their merits and demerits, and they can play a part in certain situation. We need establish standardized protocols for preoperative and postoperative evaluation with different imaging techniques in the further science for MMD and MMS.

Internal carotid artery origin of the anterior cerebral artery: A rare anatomic intracranial arterial variation in a child with morning glory disc anomaly and moyamoya vascular pattern; case report and review of literature

“…Diffusion and perfusion magnetic resonance, single photon emission computed tomography, and/or advanced MRA techniques can reliably demonstrate vessels occlusion and/or parenchymal hypoperfusion or infarcts, both on symptomatic and asymptomatic patients. [ 36 37 38 ] Moreover, these modern techniques can point out reperfusion candidates or evaluate surgical results.…”

What and why: the current situation and future prospects of “ivy sign” in moyamoya disease

“… 48 Among these, CVR may be the most valuable indicator to reflect the cerebral hemodynamic changes of MMD patients. 46 …”

“…48 Among these, CVR may be the most valuable indicator to reflect the cerebral hemodynamic changes of MMD patients. 46 In order to compensate for the decreased cerebral perfusion, three major collateral pathways develop 49,50 : (1) basal moyamoya perforator vessels arising from the anterior two-thirds of the circle of Willis. Indeed, in 2009, Kawashima et al found that unilateral hemispheric ivy proliferation correlated highly with the existence of an ipsilateral decreased CVR associated with the development of leptomeningeal collaterals in patients with MMD.…”

Contemporary and emerging magnetic resonance imaging methods for evaluation of moyamoya disease

“…Cerebral hemodynamics may be assessed with a variety of perfusion imaging techniques, including several nuclear medicine methods, contrast-bolus or xenon CT, and several MRI methods that have been extensively reviewed previously. 2,37,40,44,73 These vary in both qualitative or quantita-tive capability and type of information provided, but in general provide measures of CBF, cerebral blood volume (CBV), mean transit time (MTT), time to peak (TTP), and/or relative oxygen extraction. 2,38,40,44,73 An understanding of the underlying pathophysiology is essential for the application and interpretation of imaging examinations that assess cerebral perfusion and CVR.…”