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Moyamoya Arteriopathy: Recent Advances and Future Challenges
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Moyamoya Arteriopathy: Recent Advances and Future Challenges

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Clinical Neurology".

Deadline for manuscript submissions: closed (1 May 2021) | Viewed by 13992

Special Issue Editor

Dr. Anna Bersano

E-Mail Website
Guest Editor
Cerebrovascular Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
Interests: ischemic stroke; stroke; neurological disease; arteriopathy; moyamoya angiopathy (MA)
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Moyamoya angiopathy (MA) is a cerebrovascular disease that leads to progressive stenosis of the terminal part of the internal carotid arteries (ICAs) and their proximal branches as well as the compensatory development of abnormal "moyamoya" vessels. The disease is responsible for ischemic and hemorrhagic strokes. The pathophysiological mechanisms associated with development of MA are still largely unknown, although an imbalance of vasculogenic/angiogenic mechanisms as well as the involvement of genetic factors have been proposed as possible factors. MA may cause a decreased quality of life and severe complications and, if untreated, the disease leads to a decreased life expectancy. There is no therapy available to slow the progression of stenotic lesions or the development of the abnormal fragile vessels, and currently, surgical revascularization is the only treatment that has been demonstrated as effective for reducing the ischemic and heamhorragic stroke risk.

This Special Issue aims to highlight not only the current knowledge regarding the diagnosis and treatment of MA but also the clinical and biological disease markers as well as the newest insights into the molecular basis of MA, because understanding the mechanisms of disease is essential for the development of new therapeutic strategies.

Dr. Anna Bersano
Guest Editor

Manuscript Submission Information

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Keywords

  • moyamoya
  • arteriopathy
  • RNF213
  • angiogenesis
  • VEGF
  • angiopoietin-2

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Published Papers (4 papers)

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Research

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11 pages, 870 KiB  
Article
Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome
by Annick Kronenburg, Rachel Kleinloog, Albert van der Zwan, L. Jaap Kappelle, Luca Regli, Kees P. J. Braun and Catharina J. M. Klijn
J. Clin. Med. 2021, 10(9), 1898; https://doi.org/10.3390/jcm10091898 - 27 Apr 2021
Cited by 3 | Viewed by 2233
Abstract
Information on presentation and outcome of moyamoya vasculopathy (MMV) in European countries is limited. We investigated patient characteristics, treatment and outcome of patients with MMV. We retrieved patient characteristics and treatment information and determined functional outcome (modified Rankin Score (mRS); type of school/work) [...] Read more.
Information on presentation and outcome of moyamoya vasculopathy (MMV) in European countries is limited. We investigated patient characteristics, treatment and outcome of patients with MMV. We retrieved patient characteristics and treatment information and determined functional outcome (modified Rankin Score (mRS); type of school/work) by structured telephone interviews. We performed uni- and multivariable logistic regression analysis to determine predictors of poor outcome. We included 64 patients with bilateral MMV. In children (31 patients), median age was 5 years (interquartile range (IQR) 2–11) and in adults (33 patients), it was 33 years (IQR 28–41). Predominant mode of presentation was ischemia (children 84%; adults 88%). Modified Rankin Scale (mRS) at presentation was ≤2 in 74%. Revascularization was performed in 42 patients (23 children). Median follow-up time was 46 months (IQR 26–90). During this period, 16 patients had recurrent stroke(s) and four patients died. In 73% of the patients (83% surgical group; 55% medically treated group), mRS was ≤2; 46% were able to return to regular school or work, of whom only 41% were on the same level. Univariable analysis revealed that surgical treatment was associated with lower odds of poor outcome ((mRS ≥ 3), OR 0.24; p = 0.017). This association was no longer statistically significant (OR 3.47; p = 0.067) in the multivariable model, including age and diagnosis (moyamoya disease or moyamoya syndrome). In this cohort of patients with MMV who presented in a single European center, a large proportion had good functional outcome. Nevertheless, less than half were able to attend regular school or were able to work at their previous level, indicating a large impact of the disease on their life. Full article
(This article belongs to the Special Issue Moyamoya Arteriopathy: Recent Advances and Future Challenges)
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Review

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20 pages, 1271 KiB  
Review
Clinical Management of Moyamoya Patients
by Isabella Canavero, Ignazio Gaspare Vetrano, Marialuisa Zedde, Rosario Pascarella, Laura Gatti, Francesco Acerbi, Sara Nava, Paolo Ferroli, Eugenio Agostino Parati and Anna Bersano
J. Clin. Med. 2021, 10(16), 3628; https://doi.org/10.3390/jcm10163628 - 17 Aug 2021
Cited by 21 | Viewed by 5975
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. [...] Read more.
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. Full article
(This article belongs to the Special Issue Moyamoya Arteriopathy: Recent Advances and Future Challenges)
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18 pages, 312 KiB  
Review
Differences in Clinical Features among Different Onset Patterns in Moyamoya Disease
by Yudai Hirano, Satoru Miyawaki, Hideaki Imai, Hiroki Hongo, Yu Teranishi, Shogo Dofuku, Daiichiro Ishigami, Kenta Ohara, Satoshi Koizumi, Hideaki Ono, Hirofumi Nakatomi and Nobuhito Saito
J. Clin. Med. 2021, 10(13), 2815; https://doi.org/10.3390/jcm10132815 - 25 Jun 2021
Cited by 14 | Viewed by 2779
Abstract
Moyamoya disease is characterized by severe stenosis at the ends of the bilateral internal carotid arteries and the development of collateral circulation. The disease is very diverse in terms of age at onset, onset patterns, radiological findings, and genetic phenotypes. The pattern of [...] Read more.
Moyamoya disease is characterized by severe stenosis at the ends of the bilateral internal carotid arteries and the development of collateral circulation. The disease is very diverse in terms of age at onset, onset patterns, radiological findings, and genetic phenotypes. The pattern of onset is mainly divided into ischemic and hemorrhagic onsets. Recently, the opportunity to identify asymptomatic moyamoya disease, which sometimes manifests as nonspecific symptoms such as headache and dizziness, through screening with magnetic resonance imaging has been increasing. Various recent reports have investigated the associations between the clinical features of different onset patterns of moyamoya disease and the corresponding imaging characteristics. In this article, we have reviewed the natural history, clinical features, and imaging features of each onset pattern of moyamoya disease. Full article
(This article belongs to the Special Issue Moyamoya Arteriopathy: Recent Advances and Future Challenges)
9 pages, 240 KiB  
Review
Characteristics of Moyamoya Disease in the Older Population: Is It Possible to Define a Typical Presentation and Optimal Therapeutical Management?
by Ignazio G. Vetrano, Anna Bersano, Isabella Canavero, Francesco Restelli, Gabriella Raccuia, Elisa F. Ciceri, Giuseppe Faragò, Andrea Gioppo, Morgan Broggi, Marco Schiariti, Laura Gatti, Paolo Ferroli and Francesco Acerbi
J. Clin. Med. 2021, 10(11), 2287; https://doi.org/10.3390/jcm10112287 - 25 May 2021
Cited by 6 | Viewed by 2177
Abstract
Whereas several studies have been so far presented about the surgical outcomes in terms of mortality and perioperative complications for elderly patients submitted to neurosurgical treatments, the management of elderly moyamoya patients is unclear. This review aims to explore the available data about [...] Read more.
Whereas several studies have been so far presented about the surgical outcomes in terms of mortality and perioperative complications for elderly patients submitted to neurosurgical treatments, the management of elderly moyamoya patients is unclear. This review aims to explore the available data about the clinical manifestation, characteristics, and outcome after surgery of older patients with moyamoya arteriopathy (MA). We found only two articles strictly concerning elderly patients with MA. We have also evaluated other reported adult series of moyamoya patients, including elderly cases in their analysis. Patients with MA above 50 years old may be considered a peculiar subset in which patients are often presenting with ischemic symptoms and a higher Suzuki grade. Conservative treatment may be proposed in asymptomatic or stable cases due to their fragility and possible increase of post-operative complications, while the best surgical options in symptomatic cases are still under investigation, although we believe that a minimal invasive superficial temporal artery—middle cerebral artery bypass could be considered the treatment of choice for the immediate effect on brain perfusion with a limited rate of post-operative complications. Full article
(This article belongs to the Special Issue Moyamoya Arteriopathy: Recent Advances and Future Challenges)
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