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Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome

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 Ran...

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Autores principales: Kronenburg, Annick, Kleinloog, Rachel, van der Zwan, Albert, Kappelle, L. Jaap, Regli, Luca, Braun, Kees P. J., Klijn, Catharina J. M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8124614/
https://www.ncbi.nlm.nih.gov/pubmed/33925657
http://dx.doi.org/10.3390/jcm10091898
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author Kronenburg, Annick
Kleinloog, Rachel
van der Zwan, Albert
Kappelle, L. Jaap
Regli, Luca
Braun, Kees P. J.
Klijn, Catharina J. M.
author_facet Kronenburg, Annick
Kleinloog, Rachel
van der Zwan, Albert
Kappelle, L. Jaap
Regli, Luca
Braun, Kees P. J.
Klijn, Catharina J. M.
author_sort Kronenburg, Annick
collection PubMed
description 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.
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spelling pubmed-81246142021-05-17 Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome Kronenburg, Annick Kleinloog, Rachel van der Zwan, Albert Kappelle, L. Jaap Regli, Luca Braun, Kees P. J. Klijn, Catharina J. M. J Clin Med Article 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. MDPI 2021-04-27 /pmc/articles/PMC8124614/ /pubmed/33925657 http://dx.doi.org/10.3390/jcm10091898 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Kronenburg, Annick
Kleinloog, Rachel
van der Zwan, Albert
Kappelle, L. Jaap
Regli, Luca
Braun, Kees P. J.
Klijn, Catharina J. M.
Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome
title Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome
title_full Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome
title_fullStr Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome
title_full_unstemmed Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome
title_short Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome
title_sort patients with moyamoya vasculopathy evaluated at a single-center in the netherlands; clinical presentation and outcome
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8124614/
https://www.ncbi.nlm.nih.gov/pubmed/33925657
http://dx.doi.org/10.3390/jcm10091898
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