Dorsal root ganglia in vivo morphometry and perfusion in female patients with Fabry disease
Purpose To examine dorsal root ganglia and the proximal nerve segments in female patients with Fabry disease by functional and morphometric magnetic resonance neurography. Methods In this prospective multicenter study the lumbosacral dorsal root ganglia and proximal sciatic nerve were examined in te...
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| Veröffentlicht in: | Journal of neurology 2018-11, Vol.265 (11), p.2723-2729 |
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| container_title | Journal of neurology |
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| creator | Godel, Tim Köhn, Anja Muschol, Nicole Kronlage, Moritz Schwarz, Daniel Kollmer, Jennifer Heiland, Sabine Bendszus, Martin Mautner, Victor-Felix Bäumer, Philipp |
| description | Purpose
To examine dorsal root ganglia and the proximal nerve segments in female patients with Fabry disease by functional and morphometric magnetic resonance neurography.
Methods
In this prospective multicenter study the lumbosacral dorsal root ganglia and proximal sciatic nerve were examined in ten female patients with Fabry disease by a standardized magnetic resonance neurography protocol at 3 T. Volumes of dorsal root ganglia L3–S2, permeability of dorsal root ganglia L5 and S1 and the spinal nerve L5 as well as the cross-sectional area of the proximal sciatic nerve were compared to 16 gender-matched healthy controls.
Results
Dorsal root ganglia were symmetrically enlarged by 54% (L3), 79% (L4), 60% (L5), 94% (S1), and 106% (S2) (
p
|
| doi_str_mv | 10.1007/s00415-018-9053-y |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_2103669020</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2103357262</sourcerecordid><originalsourceid>FETCH-LOGICAL-c398t-86fa77e1260d60e18858c23047a336b5e66afec2aa5ad29c5c648adc53f706083</originalsourceid><addsrcrecordid>eNp1kE1P3DAQhq2qVVk-fgCXylIvvaQd27HjHBEttBJSL-XEwZpNJotREgc7odp_j7cLRULiNId55p2Zh7FTAV8FQPUtAZRCFyBsUYNWxfYdW4lSyUKUun7PVqBKKLTS5QE7TOkOAGxufGQHCiTURssVu_keYsKexxBmvsFx03vkfuQP_iHwIcTpNgw0xy3HseUTxW5JPow7oqMBe-ITzp7GOfG_fr7lF7jObOsTYaJj9qHDPtHJUz1i1xc__pz_LK5-X_46P7sqGlXbubCmw6oiIQ20BkhYq20jFZQVKmXWmozBjhqJqLGVdaMbU1psG626CgxYdcS-7HOnGO4XSrMbfGqo73GksCQnBShj6vxzRj-_Qu_CEsd83T9K6UoamSmxp5oYUorUuSn6AePWCXA7825v3mXzbmfebfPMp6fkZT1Q-3_iWXUG5B5IuTVuKL6sfjv1EUw0jlk</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2103357262</pqid></control><display><type>article</type><title>Dorsal root ganglia in vivo morphometry and perfusion in female patients with Fabry disease</title><source>SpringerNature Journals</source><creator>Godel, Tim ; Köhn, Anja ; Muschol, Nicole ; Kronlage, Moritz ; Schwarz, Daniel ; Kollmer, Jennifer ; Heiland, Sabine ; Bendszus, Martin ; Mautner, Victor-Felix ; Bäumer, Philipp</creator><creatorcontrib>Godel, Tim ; Köhn, Anja ; Muschol, Nicole ; Kronlage, Moritz ; Schwarz, Daniel ; Kollmer, Jennifer ; Heiland, Sabine ; Bendszus, Martin ; Mautner, Victor-Felix ; Bäumer, Philipp</creatorcontrib><description>Purpose
To examine dorsal root ganglia and the proximal nerve segments in female patients with Fabry disease by functional and morphometric magnetic resonance neurography.
Methods
In this prospective multicenter study the lumbosacral dorsal root ganglia and proximal sciatic nerve were examined in ten female patients with Fabry disease by a standardized magnetic resonance neurography protocol at 3 T. Volumes of dorsal root ganglia L3–S2, permeability of dorsal root ganglia L5 and S1 and the spinal nerve L5 as well as the cross-sectional area of the proximal sciatic nerve were compared to 16 gender-matched healthy controls.
Results
Dorsal root ganglia were symmetrically enlarged by 54% (L3), 79% (L4), 60% (L5), 94% (S1), and 106% (S2) (
p
< 0.001). Additionally, permeability of the blood–tissue interface was decreased by 47% (
p
< 0.001). This finding was most pronounced in the peripheral zone of the dorsal root ganglia, where the cell bodies of the primary sensory neurons are located (
p
< 0.001). While spinal nerve permeability showed no differences compared to healthy controls, proximal sciatic nerve cross-sectional area was mildly increased by 6% (
p
< 0.01).
Conclusion
Although heterozygous, Fabry females show severe enlarged dorsal root ganglia with a concomitant dysfunctional perfusion, even in patients with minor disease progression and in patients who are not considered for enzyme replacement therapy yet. Alterations in dorsal root ganglia volume and perfusion might serve as a very early in vivo marker for involvement of the peripheral nervous system in Fabry disease, even in patients with residual enzyme activity.</description><identifier>ISSN: 0340-5354</identifier><identifier>EISSN: 1432-1459</identifier><identifier>DOI: 10.1007/s00415-018-9053-y</identifier><identifier>PMID: 30209652</identifier><language>eng</language><publisher>Berlin/Heidelberg: Springer Berlin Heidelberg</publisher><subject>Dorsal root ganglia ; Enzymatic activity ; Enzymes ; Fabry's disease ; Females ; Medicine ; Medicine & Public Health ; Morphometry ; Nervous system ; Neurology ; Neuroradiology ; Neurosciences ; Original Communication ; Perfusion ; Permeability ; Sciatic nerve ; Sensory neurons</subject><ispartof>Journal of neurology, 2018-11, Vol.265 (11), p.2723-2729</ispartof><rights>Springer-Verlag GmbH Germany, part of Springer Nature 2018</rights><rights>Journal of Neurology is a copyright of Springer, (2018). All Rights Reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c398t-86fa77e1260d60e18858c23047a336b5e66afec2aa5ad29c5c648adc53f706083</citedby><cites>FETCH-LOGICAL-c398t-86fa77e1260d60e18858c23047a336b5e66afec2aa5ad29c5c648adc53f706083</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00415-018-9053-y$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00415-018-9053-y$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>315,781,785,27929,27930,41493,42562,51324</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/30209652$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Godel, Tim</creatorcontrib><creatorcontrib>Köhn, Anja</creatorcontrib><creatorcontrib>Muschol, Nicole</creatorcontrib><creatorcontrib>Kronlage, Moritz</creatorcontrib><creatorcontrib>Schwarz, Daniel</creatorcontrib><creatorcontrib>Kollmer, Jennifer</creatorcontrib><creatorcontrib>Heiland, Sabine</creatorcontrib><creatorcontrib>Bendszus, Martin</creatorcontrib><creatorcontrib>Mautner, Victor-Felix</creatorcontrib><creatorcontrib>Bäumer, Philipp</creatorcontrib><title>Dorsal root ganglia in vivo morphometry and perfusion in female patients with Fabry disease</title><title>Journal of neurology</title><addtitle>J Neurol</addtitle><addtitle>J Neurol</addtitle><description>Purpose
To examine dorsal root ganglia and the proximal nerve segments in female patients with Fabry disease by functional and morphometric magnetic resonance neurography.
Methods
In this prospective multicenter study the lumbosacral dorsal root ganglia and proximal sciatic nerve were examined in ten female patients with Fabry disease by a standardized magnetic resonance neurography protocol at 3 T. Volumes of dorsal root ganglia L3–S2, permeability of dorsal root ganglia L5 and S1 and the spinal nerve L5 as well as the cross-sectional area of the proximal sciatic nerve were compared to 16 gender-matched healthy controls.
Results
Dorsal root ganglia were symmetrically enlarged by 54% (L3), 79% (L4), 60% (L5), 94% (S1), and 106% (S2) (
p
< 0.001). Additionally, permeability of the blood–tissue interface was decreased by 47% (
p
< 0.001). This finding was most pronounced in the peripheral zone of the dorsal root ganglia, where the cell bodies of the primary sensory neurons are located (
p
< 0.001). While spinal nerve permeability showed no differences compared to healthy controls, proximal sciatic nerve cross-sectional area was mildly increased by 6% (
p
< 0.01).
Conclusion
Although heterozygous, Fabry females show severe enlarged dorsal root ganglia with a concomitant dysfunctional perfusion, even in patients with minor disease progression and in patients who are not considered for enzyme replacement therapy yet. Alterations in dorsal root ganglia volume and perfusion might serve as a very early in vivo marker for involvement of the peripheral nervous system in Fabry disease, even in patients with residual enzyme activity.</description><subject>Dorsal root ganglia</subject><subject>Enzymatic activity</subject><subject>Enzymes</subject><subject>Fabry's disease</subject><subject>Females</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Morphometry</subject><subject>Nervous system</subject><subject>Neurology</subject><subject>Neuroradiology</subject><subject>Neurosciences</subject><subject>Original Communication</subject><subject>Perfusion</subject><subject>Permeability</subject><subject>Sciatic nerve</subject><subject>Sensory neurons</subject><issn>0340-5354</issn><issn>1432-1459</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNp1kE1P3DAQhq2qVVk-fgCXylIvvaQd27HjHBEttBJSL-XEwZpNJotREgc7odp_j7cLRULiNId55p2Zh7FTAV8FQPUtAZRCFyBsUYNWxfYdW4lSyUKUun7PVqBKKLTS5QE7TOkOAGxufGQHCiTURssVu_keYsKexxBmvsFx03vkfuQP_iHwIcTpNgw0xy3HseUTxW5JPow7oqMBe-ITzp7GOfG_fr7lF7jObOsTYaJj9qHDPtHJUz1i1xc__pz_LK5-X_46P7sqGlXbubCmw6oiIQ20BkhYq20jFZQVKmXWmozBjhqJqLGVdaMbU1psG626CgxYdcS-7HOnGO4XSrMbfGqo73GksCQnBShj6vxzRj-_Qu_CEsd83T9K6UoamSmxp5oYUorUuSn6AePWCXA7825v3mXzbmfebfPMp6fkZT1Q-3_iWXUG5B5IuTVuKL6sfjv1EUw0jlk</recordid><startdate>20181101</startdate><enddate>20181101</enddate><creator>Godel, Tim</creator><creator>Köhn, Anja</creator><creator>Muschol, Nicole</creator><creator>Kronlage, Moritz</creator><creator>Schwarz, Daniel</creator><creator>Kollmer, Jennifer</creator><creator>Heiland, Sabine</creator><creator>Bendszus, Martin</creator><creator>Mautner, Victor-Felix</creator><creator>Bäumer, Philipp</creator><general>Springer Berlin Heidelberg</general><general>Springer Nature B.V</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7TK</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>20181101</creationdate><title>Dorsal root ganglia in vivo morphometry and perfusion in female patients with Fabry disease</title><author>Godel, Tim ; Köhn, Anja ; Muschol, Nicole ; Kronlage, Moritz ; Schwarz, Daniel ; Kollmer, Jennifer ; Heiland, Sabine ; Bendszus, Martin ; Mautner, Victor-Felix ; Bäumer, Philipp</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c398t-86fa77e1260d60e18858c23047a336b5e66afec2aa5ad29c5c648adc53f706083</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Dorsal root ganglia</topic><topic>Enzymatic activity</topic><topic>Enzymes</topic><topic>Fabry's disease</topic><topic>Females</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Morphometry</topic><topic>Nervous system</topic><topic>Neurology</topic><topic>Neuroradiology</topic><topic>Neurosciences</topic><topic>Original Communication</topic><topic>Perfusion</topic><topic>Permeability</topic><topic>Sciatic nerve</topic><topic>Sensory neurons</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Godel, Tim</creatorcontrib><creatorcontrib>Köhn, Anja</creatorcontrib><creatorcontrib>Muschol, Nicole</creatorcontrib><creatorcontrib>Kronlage, Moritz</creatorcontrib><creatorcontrib>Schwarz, Daniel</creatorcontrib><creatorcontrib>Kollmer, Jennifer</creatorcontrib><creatorcontrib>Heiland, Sabine</creatorcontrib><creatorcontrib>Bendszus, Martin</creatorcontrib><creatorcontrib>Mautner, Victor-Felix</creatorcontrib><creatorcontrib>Bäumer, Philipp</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Neurosciences Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of neurology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Godel, Tim</au><au>Köhn, Anja</au><au>Muschol, Nicole</au><au>Kronlage, Moritz</au><au>Schwarz, Daniel</au><au>Kollmer, Jennifer</au><au>Heiland, Sabine</au><au>Bendszus, Martin</au><au>Mautner, Victor-Felix</au><au>Bäumer, Philipp</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Dorsal root ganglia in vivo morphometry and perfusion in female patients with Fabry disease</atitle><jtitle>Journal of neurology</jtitle><stitle>J Neurol</stitle><addtitle>J Neurol</addtitle><date>2018-11-01</date><risdate>2018</risdate><volume>265</volume><issue>11</issue><spage>2723</spage><epage>2729</epage><pages>2723-2729</pages><issn>0340-5354</issn><eissn>1432-1459</eissn><abstract>Purpose
To examine dorsal root ganglia and the proximal nerve segments in female patients with Fabry disease by functional and morphometric magnetic resonance neurography.
Methods
In this prospective multicenter study the lumbosacral dorsal root ganglia and proximal sciatic nerve were examined in ten female patients with Fabry disease by a standardized magnetic resonance neurography protocol at 3 T. Volumes of dorsal root ganglia L3–S2, permeability of dorsal root ganglia L5 and S1 and the spinal nerve L5 as well as the cross-sectional area of the proximal sciatic nerve were compared to 16 gender-matched healthy controls.
Results
Dorsal root ganglia were symmetrically enlarged by 54% (L3), 79% (L4), 60% (L5), 94% (S1), and 106% (S2) (
p
< 0.001). Additionally, permeability of the blood–tissue interface was decreased by 47% (
p
< 0.001). This finding was most pronounced in the peripheral zone of the dorsal root ganglia, where the cell bodies of the primary sensory neurons are located (
p
< 0.001). While spinal nerve permeability showed no differences compared to healthy controls, proximal sciatic nerve cross-sectional area was mildly increased by 6% (
p
< 0.01).
Conclusion
Although heterozygous, Fabry females show severe enlarged dorsal root ganglia with a concomitant dysfunctional perfusion, even in patients with minor disease progression and in patients who are not considered for enzyme replacement therapy yet. Alterations in dorsal root ganglia volume and perfusion might serve as a very early in vivo marker for involvement of the peripheral nervous system in Fabry disease, even in patients with residual enzyme activity.</abstract><cop>Berlin/Heidelberg</cop><pub>Springer Berlin Heidelberg</pub><pmid>30209652</pmid><doi>10.1007/s00415-018-9053-y</doi><tpages>7</tpages></addata></record> |
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| source | SpringerNature Journals |
| subjects | Dorsal root ganglia Enzymatic activity Enzymes Fabry's disease Females Medicine Medicine & Public Health Morphometry Nervous system Neurology Neuroradiology Neurosciences Original Communication Perfusion Permeability Sciatic nerve Sensory neurons |
| title | Dorsal root ganglia in vivo morphometry and perfusion in female patients with Fabry disease |
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