Please use this identifier to cite or link to this item: http://doi.org/10.25358/openscience-7971
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dc.contributor.authorLelieveld, Irene M.-
dc.contributor.authorBöttcher, Anna-
dc.contributor.authorHennermann, Julia B.-
dc.contributor.authorBeck, Michael-
dc.contributor.authorFellgiebel, Andreas-
dc.date.accessioned2022-10-13T07:50:48Z-
dc.date.available2022-10-13T07:50:48Z-
dc.date.issued2015
dc.identifier.urihttps://openscience.ub.uni-mainz.de/handle/20.500.12030/7986-
dc.description.abstractBrain structural alterations and neuropsychiatric symptoms have been described repeatedly in Fabry disease, yet cognitive deficits have been shown to be only mild. Here, we aimed to investigate neuropsychiatric symptoms and brain structure longitudinally. We expected no clinically relevant increase of neuropsychiatric symptoms in parallel to increased brain structural alterations. We assessed 14 Fabry patients (46.1 ± 10.8 years) who had participated in our investigation eight years ago. Patients engaged in neuropsychiatric testing, as well as structural magnetic resonance imaging and angiography to determine white matter lesions, hippocampal volume, and the diameter of the larger intracranial arteries. While Fabry patients did not differ on cognitive performance, they showed progressive and significant hippocampal volume loss over the 8-year observation period. White matter lesions were associated with older age and higher white matter lesion load at baseline, but did not reach statistical significance when comparing baseline to follow-up. Likewise, intracranial artery diameters did not increase significantly. None of the imaging parameters were associated with the neuropsychiatric parameters. Depression frequency reduced from 50% at baseline to 21% at follow-up, but it did not reach significance. This investigation demonstrates clinical stability in cognitive function, while pronounced hippocampal atrophy is apparent throughout the 8 years. Our middle-aged Fabry patients appeared to compensate successfully for progressive hippocampal volume loss. The hippocampal volume decline indicates brain regional neuronal involvement in Fabry disease.en_GB
dc.description.sponsorshipDFG, Open Access-Publizieren Universität Mainz / Universitätsmedizinde
dc.language.isoengde
dc.rightsCC BY*
dc.rights.urihttps://creativecommons.org/licenses/by/4.0/*
dc.subject.ddc610 Medizinde_DE
dc.subject.ddc610 Medical sciencesen_GB
dc.titleEight-year follow-up of neuropsychiatric symptoms and brain structural changes in Fabry diseaseen_GB
dc.typeZeitschriftenaufsatzde
dc.identifier.doihttp://doi.org/10.25358/openscience-7971-
jgu.type.dinitypearticleen_GB
jgu.type.versionPublished versionde
jgu.type.resourceTextde
jgu.organisation.departmentFB 04 Medizinde
jgu.organisation.number2700-
jgu.organisation.nameJohannes Gutenberg-Universität Mainz-
jgu.rights.accessrightsopenAccess-
jgu.journal.titlePLoS onede
jgu.journal.volume10de
jgu.journal.issue9de
jgu.pages.alternativee0137603de
jgu.publisher.year2015-
jgu.publisher.namePLoSde
jgu.publisher.placeLawrence, Kan.de
jgu.publisher.urihttp://dx.doi.org/10.1371/journal.pone.0137603de
jgu.publisher.issn1932-6203de
jgu.organisation.placeMainz-
jgu.subject.ddccode610de
opus.date.modified2017-04-25T12:44:08Z
opus.subject.dfgcode00-000
opus.organisation.stringFB 04: Medizin: Psychiatrische Klinik und Poliklinikde_DE
opus.organisation.stringFB 04: Medizin: Zentrum für Kinder- und Jugendmedizinde_DE
opus.identifier.opusid56506
opus.institute.number0432
opus.institute.number0462
opus.metadataonlyfalse
opus.type.contenttypeKeinede_DE
opus.type.contenttypeNoneen_EN
opus.affiliatedLelieveld, Irene M.
opus.affiliatedBeck, Michael
opus.affiliatedFellgiebel, Andreas
jgu.publisher.doi10.1371/journal.pone.0137603de
jgu.organisation.rorhttps://ror.org/023b0x485-
Appears in collections:DFG-OA-Publizieren (2012 - 2017)

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