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Special Issue "Diagnosis and Treatment of Moyamoya Disease"

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

Deadline for manuscript submissions: 30 April 2023 | Viewed by 1893

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Special Issue Editors

Dr. Sophia Vassilopoulou

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Guest Editor

Department of Neurology Eginition Hospital, National and Kapodistrian University of Athens, Athens, Greece
Interests: stroke, rare cerebral vasculopathies; stroke in young patients; cerebral venous thrombosis; intracerebral hemorrhage

Dr. Georgios Velonakis

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Guest Editor

Research Unit of Radiology and Medical Imaging, 2nd Department of Radiology, Attikon General University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
Interests: stroke, rare cerebral vasculopathies; stroke in young patients; cerebral venous thrombosis; intracerebral hemorrhage

Dr. Argyro Tountopoulou

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Guest Editor

1st Department of Neurology, University of Athens, Athens, Greece
Interests: stroke, rare cerebral vasculopathies; stroke in young patients; cerebral venous thrombosis; intracerebral hemorrhage

Dr. Dimitrios Kasselimis

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Guest Editor

Psychology Department, Panteion University, Athens, Greece
Interests: language functions; aphasia; executive functions; memory; neurological disorders

Special Issue Information

Dear Colleagues,

Moyamoya Vasculopathy (MMV) is a rare netlike vascular disease of the brain characterized by steno-occlusive lesions of intracranial arteries with an unknown etiology.

MMV affects both children and adults, and it can be presented either without an accompanying disease, which is then characterized as Disease, or with an accompanying disease, which is characterized as Syndrome.

The vasculopathy is quite well described in the Asian population, especially in Japan, due to its high incidence in this country. On the contrary, few Western studies have been reported, but differences in incidence, clinical and radiological presentation and in genetic background among different populations are emerging. Moreover, scarce studies have underlined the neuropsychological implications of MMV.

Overall, there is a need for more research on MMV to elucidate its possible etiology; to clarify the clinical, ethnic and demographic factors associated with the disease onset and progression; to highlight its neurocognitive manifestations; and to provide treatment options. The aforementioned issues constitute the focus of the present Special Issue. Some indicative topics are as follows:

Demographic and clinical characteristics of patients with MMV;
Neuroradiological diagnostic markers of MMV;
Neurocognitive manifestations of MMV;
Genetic background of MMV;
Incidence and differences of MMV between East and West.

Dr. Sophia Vassilopoulou
Dr. Georgios Velonakis
Dr. Argyro Tountopoulou
Dr. Dimitrios Kasselimis
Guest Editors

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Keywords

Moyamoya vasculopathy
stroke
steno-occlusive lesions
neurocognitive deficits

Published Papers (3 papers)

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Research

Open AccessArticle

Circulating Inflammatory Cytokine Associated with Poor Prognosis in Moyamoya Disease: A Prospective Cohort Study

and

J. Clin. Med. 2023, 12(3), 823; https://doi.org/10.3390/jcm12030823 - 19 Jan 2023

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Abstract

Inflammation is a key factor in the development of moyamoya disease. However, the cytokine distribution in moyamoya disease and its impact on prognosis remain unclear. A total of 204 patients with moyamoya disease were enrolled in this study. The peripheral blood was analyzed for baseline data and cytokines, which included IL-6, IL-1β, IL-2R, IL-8, and TNF-α. Patients with the RNF213 mutation and those without the mutation were compared in terms of their differences in cytokines. A mRS score ≥2 was defined as a poor prognosis, and a mRS score <2 was described as a good prognosis, and differences in cytokines were compared between the two groups. Regression analysis was performed to identify markers affecting prognosis. TNF-α and IL-6 levels were higher in the group without the RNF213 mutation compared to the mutation group. Multivariate stepwise regression analysis indicated that the G3 subgroup of IL-6 and the G4 subgroup of TNF-α were the independent risk factors for adverse prognosis in adults with moyamoya disease (OR 3.678, 95% CI [1.491, 9.074], p = 0.005; OR 2.996, 95% CI [1.180, 7.610], p = 0.021). IL-6 and TNF-α were associated with poor prognosis in adult patients with moyamoya disease. Full article

(This article belongs to the Special Issue Diagnosis and Treatment of Moyamoya Disease)

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Open AccessArticle

Clinical Outcome, Cognition, and Cerebrovascular Reactivity after Surgical Treatment for Moyamoya Vasculopathy: A Dutch Prospective, Single-Center Cohort Study

Pieter Thomas Deckers

Annick Kronenburg

Esther van den Berg

Monique M. van Schooneveld

Evert-Jan P. A. Vonken

Willem M. Otte

Bart N. M. van Berckel

Maqsood Yaqub

Catharina J. M. Klijn

Albert van der Zwan

and

Kees P. J. Braun

J. Clin. Med. 2022, 11(24), 7427; https://doi.org/10.3390/jcm11247427 - 14 Dec 2022

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Abstract

Background: It remains unclear whether revascularization of moyamoya vasculopathy (MMV) has a positive effect on cognitive function. In this prospective, single-center study, we investigated the effect of revascularization on cognitive function in patients with MMV. We report clinical and radiological outcome parameters and the associations between clinical determinants and change in neurocognitive functioning. Methods: We consecutively included all MMV patients at a Dutch tertiary referral hospital who underwent pre- and postoperative standardized neuropsychological evaluation, [¹⁵O]H₂O-PET (including cerebrovascular reactivity (CVR)), MRI, cerebral angiography, and completed standardized questionnaires on clinical outcome and quality of life (QOL). To explore the association between patient characteristics, imaging findings, and change in the z-scores of the cognitive domains, we used multivariable linear- and Bayesian regression analysis. Results: We included 40 patients of whom 35 (27 females, 21 children) were treated surgically. One patient died after surgery, and two withdrew from the study. TIA- and headache frequency and modified Rankin scale (mRS) improved (resp. p = 0.001, 0.019, 0.039). Eleven patients (seven children) developed a new infarct during follow-up (31%), five of which were symptomatic. CVR-scores improved significantly (p < 0.0005). The language domain improved (p = 0.029); other domains remained stable. In adults, there was an improvement in QOL. We could not find an association between change in imaging and cognitive scores. Conclusion: In this cohort of Western MMV patients, TIA frequency, headache, CVR, and mRS improved significantly after revascularization. The language domain significantly improved, while others remained stable. We could not find an association between changes in CVR and cognitive scores. Full article

(This article belongs to the Special Issue Diagnosis and Treatment of Moyamoya Disease)

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Open AccessArticle

Serum Kynurenic Acid and Kynurenine Are Negatively Associated with the Risk of Adult Moyamoya Disease

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J. Clin. Med. 2022, 11(23), 7069; https://doi.org/10.3390/jcm11237069 - 29 Nov 2022

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Abstract

Background and aim. Kynurenine (KYN) and kynurenic acid (KYNA) are key intermediate metabolites associated with inflammation and immune responses in the kynurenine pathway. It remains unknown whether KYN or KYNA is associated with the risk of adult moyamoya disease (MMD). The aim of this study was to prospectively investigate the association between serum KYN or KYNA and the risk of adult MMD. Methods. The study was conducted from July 2020 to December 2021. We measured serum KYN and KYNA levels for 360 adult MMD patients (259 cases of ischemic MMD, 101 cases of hemorrhagic MMD) and 89 age-sex-matched healthy controls. Clinical and laboratory characteristics were collected from the medical record. Results. After multivariate adjustment, decreased serum KYNA (OR, 0.085; 95% CI, 0.035–0.206; p = 0.000) or KYN (OR, 0.430; 95% CI, 0.225–0.820; p = 0.010) levels were associated with increased risk of MMD when upper and lower tertiles were compared. In addition, a higher trend of hemorrhagic MMD was found in MMD patients in KYNA tertile 1 compared with those in tertile 2 to 3 (OR, 0.584; 95% CI, 0.345–0.987; p = 0.044). Addition of serum KYNA (net reclassification improvement: 73.24%, p = 0.000; integrated discrimination improvement: 9.60%, p = 0.000) or KYN (integrated discrimination improvement: 1.70%, p = 0.037) to conventional risk factors significantly improved the risk prediction of MMD. In the exploratory analysis, we observed an interaction between KYN and age (≥40 versus <40 years) or homocysteine levels (≥13.0 versus <13.0 μmol/L) on the risk of MMD. Conclusions. Decreased serum KYNA or KYN levels were associated with an increased risk of adult MMD, suggesting that serum KYNA or KYN may be a valuable predictive biomarker for adult MMD. Full article

(This article belongs to the Special Issue Diagnosis and Treatment of Moyamoya Disease)

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Special Issue "Diagnosis and Treatment of Moyamoya Disease"

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