Clinical Management of Moyamoya Patients.

Isabella Canavero,Marialuisa Zedde,Rosario Pascarella,Francesco Acerbi,Paolo Ferroli,Ignazio Gaspare Vetrano,Sara Nava,Anna Bersano,Eugenio Agostino Parati,Laura Gatti

doi:10.3390/jcm10163628

Abstract

Moyamoya angiopathy (MMA) is a peculiar cerebrovascular condition characterized by progressive steno-occlusion of the terminal part of the internal carotid arteries (ICAs) and their proximal branches, associated with the development of a network of fragile collateral vessels at the base of the brain. The diagnosis is essentially made by radiological angiographic techniques. MMA is often idiopathic (moyamoya disease-MMD); conversely, it can be associated with acquired or hereditary conditions (moyamoya Syndrome-MMS); however, the pathophysiology underlying either MMD or MMS has not been fully elucidated to date, and this poor knowledge reflects uncertainties and heterogeneity in patient management. MMD and MMS also have similar clinical expressions, including, above all, ischemic and hemorrhagic strokes, then headaches, seizures, cognitive impairment, and movement disorders. The available treatment strategies are currently shared between idiopathic MMD and MMS, including pharmacological and surgical stroke prevention treatments and symptomatic drugs. No pharmacological treatment able to reverse the progressive disappearance of the ICAs has been found to date in both idiopathic and syndromic cases. Antithrombotic agents are usually prescribed in ischemic MMA, although the coexisting hemorrhagic risk should be considered. Surgical revascularization techniques, which are currently the best available treatment in symptomatic MMA, are associated with good long-term outcomes and reduced ischemic and hemorrhagic risks. Given the lack of dedicated randomized clinical trials, current treatment is mainly based on observational studies and physicians’ and surgeons’ expertise.

Highlights

Moyamoya angiopathy is a peculiar affection of the cerebral arteries characterized by progressive steno-occlusive lesions of the terminal part and proximal branches of the internal carotid arteries (ICAs), associated with the development of an unstable network of collateral vessels at the base of the brain [1,2].These vascular hallmarks are responsible for the main disease clinical features, which are recurrent ischemic and hemorrhagic strokes
Other than the intrinsic limitations of a non-systematic review approach, our aim was to provide a practical overview, a global perspective on the current state-of-the-art for the available strategies for management and treatment of middle meningeal artery (MMA) patients, with particular consideration for the Western countries setting, to empower disease recognition and to set a grounding for the appropriate clinical work-up for suspected and confirmed cases, still bearing in mind that the best therapeutic strategy should be tailored for each case
Preoperative digital subtraction angiography (DSA) is recommended to assess the extension of the occlusion and the collateral network, identify donor arteries, and avoid the disruption of pre-existing collateral vessels possibly developed from ECA

Summary

Introduction

Moyamoya angiopathy (or arteriopathy; MMA) is a peculiar affection of the cerebral arteries characterized by progressive steno-occlusive lesions of the terminal part and proximal branches of the internal carotid arteries (ICAs), associated with the development of an unstable network of collateral vessels at the base of the brain (moyamoya vessels) [1,2]. These vascular hallmarks are responsible for the main disease clinical features, which are recurrent ischemic and hemorrhagic strokes. Autonomy loss at 5-years follow-up has been observed in 60% of children and 20% of adults [3]

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