Moyamoya disease and syndrome: a review.

Zeferino Demartini Jr,Gelson Luis Koppe,Luana A Maranha Gatto,Alex Roman,Bernardo Ca Teixeira,Renato Puppi Munhoz

doi:10.1590/0100-3984.2021.0010

Abstract

Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.

Highlights

Takeuchi et al[1] first described the so-called “hypoplasia of bilateral internal carotid arteries” in a paper published in 1957
We summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies
Unlike what is seen in conditions such as M1 stenosis, in which the lesions affect only one vessel, the hypoperfusion in MMD involves adjacent vascular territories, causing more significant ischemia[8], which chronically induces the formation of collateral vessels of arterial branches of the ICA and of the posterior circulation[6]

Summary

INTRODUCTION

Takeuchi et al[1] first described the so-called “hypoplasia of bilateral internal carotid arteries” in a paper published in 1957. Moyamoya disease (MMD), a chronic occlusive cerebrovascular disease, is a non-atherosclerotic structural arterial abnormality characterized by progressive stenosis or occlusion of the intracranial internal carotid arteries (ICAs) and their proximal branches, with abnormal formation of collateral vessels, known as rete mirabile[3,4,5,6,7]. Unlike what is seen in conditions such as M1 stenosis, in which the lesions affect only one vessel, the hypoperfusion in MMD involves adjacent vascular territories, causing more significant ischemia[8], which chronically induces the formation of collateral vessels of arterial branches of the ICA (prior to its bifurcation) and of the posterior circulation[6]. Patients with a vasculopathy similar to MMD in the setting of another (underlying) disease are classified as having moyamoya syndrome[9]. The prevalence of symptomatic MMD in the United States showed a fourfold increase between 2005 and 2008, 32 years being the median age of symptom onset and there being a trend toward a predominance of females[14]

ETIOLOGY AND HISTOLOGICAL FINDINGS

CLINICAL PRESENTATION

DIAGNOSTIC TESTS

Exacerbation of narrowed vessels and initial weakening of the collaterals

Findings

CONCLUSION