Increased arterial diameters in the posterior cerebral circulation in men with Fabry disease.

A high load of white matter lesions and enlarged basilar arteries have been shown in selected patients with Fabry disease, a disorder associated with an increased stroke risk. We studied a large cohort of patients with Fabry disease to differentially investigate white matter lesion load and cerebral...

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Autores principales:	Nurcan Uçeyler, György A Homola, Hans Guerrero González, Daniela Kramer, Christoph Wanner, Frank Weidemann, László Solymosi, Claudia Sommer
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2014
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/966c740c76c244b1b81379ab9c8f7329
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spelling	oai:doaj.org-article:966c740c76c244b1b81379ab9c8f73292021-11-18T08:35:36ZIncreased arterial diameters in the posterior cerebral circulation in men with Fabry disease.1932-620310.1371/journal.pone.0087054https://doaj.org/article/966c740c76c244b1b81379ab9c8f73292014-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/24475221/?tool=EBIhttps://doaj.org/toc/1932-6203A high load of white matter lesions and enlarged basilar arteries have been shown in selected patients with Fabry disease, a disorder associated with an increased stroke risk. We studied a large cohort of patients with Fabry disease to differentially investigate white matter lesion load and cerebral artery diameters. We retrospectively analyzed cranial magnetic resonance imaging scans of 87 consecutive Fabry patients, 20 patients with ischemic stroke, and 36 controls. We determined the white matter lesion load applying the Fazekas score on fluid-attenuated inversion recovery sequences and measured the diameters of cerebral arteries on 3D-reconstructions of the time-of-flight-MR-angiography scans. Data of different Fabry patient subgroups (males-females; normal-impaired renal function) were compared with data of patients with stroke and controls. A history of stroke or transient ischemic attacks was present in 4/30 males (13%) and 5/57 (9%) females with Fabry disease, all in the anterior circulation. Only one man with Fabry disease showed confluent cerebral white matter lesions in the Fazekas score assessment (1%). Male Fabry patients had a larger basilar artery (p<0.01) and posterior cerebral artery diameter (p<0.05) compared to male controls. This was independent of disease severity as measured by renal function and did not lead to changes in arterial blood flow properties. A basilar artery diameter of >3.2 mm distinguished between men with Fabry disease and controls (sensitivity: 87%, specificity: 86%, p<0.001), but not from stroke patients. Enlarged arterial diameters of the posterior circulation are present only in men with Fabry disease independent of disease severity.Nurcan UçeylerGyörgy A HomolaHans Guerrero GonzálezDaniela KramerChristoph WannerFrank WeidemannLászló SolymosiClaudia SommerPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 9, Iss 1, p e87054 (2014)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Nurcan Uçeyler György A Homola Hans Guerrero González Daniela Kramer Christoph Wanner Frank Weidemann László Solymosi Claudia Sommer Increased arterial diameters in the posterior cerebral circulation in men with Fabry disease.
description	A high load of white matter lesions and enlarged basilar arteries have been shown in selected patients with Fabry disease, a disorder associated with an increased stroke risk. We studied a large cohort of patients with Fabry disease to differentially investigate white matter lesion load and cerebral artery diameters. We retrospectively analyzed cranial magnetic resonance imaging scans of 87 consecutive Fabry patients, 20 patients with ischemic stroke, and 36 controls. We determined the white matter lesion load applying the Fazekas score on fluid-attenuated inversion recovery sequences and measured the diameters of cerebral arteries on 3D-reconstructions of the time-of-flight-MR-angiography scans. Data of different Fabry patient subgroups (males-females; normal-impaired renal function) were compared with data of patients with stroke and controls. A history of stroke or transient ischemic attacks was present in 4/30 males (13%) and 5/57 (9%) females with Fabry disease, all in the anterior circulation. Only one man with Fabry disease showed confluent cerebral white matter lesions in the Fazekas score assessment (1%). Male Fabry patients had a larger basilar artery (p<0.01) and posterior cerebral artery diameter (p<0.05) compared to male controls. This was independent of disease severity as measured by renal function and did not lead to changes in arterial blood flow properties. A basilar artery diameter of >3.2 mm distinguished between men with Fabry disease and controls (sensitivity: 87%, specificity: 86%, p<0.001), but not from stroke patients. Enlarged arterial diameters of the posterior circulation are present only in men with Fabry disease independent of disease severity.
format	article
author	Nurcan Uçeyler György A Homola Hans Guerrero González Daniela Kramer Christoph Wanner Frank Weidemann László Solymosi Claudia Sommer
author_facet	Nurcan Uçeyler György A Homola Hans Guerrero González Daniela Kramer Christoph Wanner Frank Weidemann László Solymosi Claudia Sommer
author_sort	Nurcan Uçeyler
title	Increased arterial diameters in the posterior cerebral circulation in men with Fabry disease.
title_short	Increased arterial diameters in the posterior cerebral circulation in men with Fabry disease.
title_full	Increased arterial diameters in the posterior cerebral circulation in men with Fabry disease.
title_fullStr	Increased arterial diameters in the posterior cerebral circulation in men with Fabry disease.
title_full_unstemmed	Increased arterial diameters in the posterior cerebral circulation in men with Fabry disease.
title_sort	increased arterial diameters in the posterior cerebral circulation in men with fabry disease.
publisher	Public Library of Science (PLoS)
publishDate	2014
url	https://doaj.org/article/966c740c76c244b1b81379ab9c8f7329
work_keys_str_mv	AT nurcanuceyler increasedarterialdiametersintheposteriorcerebralcirculationinmenwithfabrydisease AT gyorgyahomola increasedarterialdiametersintheposteriorcerebralcirculationinmenwithfabrydisease AT hansguerrerogonzalez increasedarterialdiametersintheposteriorcerebralcirculationinmenwithfabrydisease AT danielakramer increasedarterialdiametersintheposteriorcerebralcirculationinmenwithfabrydisease AT christophwanner increasedarterialdiametersintheposteriorcerebralcirculationinmenwithfabrydisease AT frankweidemann increasedarterialdiametersintheposteriorcerebralcirculationinmenwithfabrydisease AT laszlosolymosi increasedarterialdiametersintheposteriorcerebralcirculationinmenwithfabrydisease AT claudiasommer increasedarterialdiametersintheposteriorcerebralcirculationinmenwithfabrydisease
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Increased arterial diameters in the posterior cerebral circulation in men with Fabry disease.

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