Specific muscle strength is reduced in facioscapulohumeral dystrophy: An MRI based musculoskeletal analysis
•Specific muscle strength of the quadriceps muscle is reduced in patients with FSHD.•MRI and muscle modeling can be used to evaluate specific muscle strength in FSHD.•FSHD patients have lower contractile muscle volume and PCSA than healthy controls.•Clinical trials for FSHD should aim for restoratio...
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| Veröffentlicht in: | Neuromuscular disorders : NMD 2018-03, Vol.28 (3), p.238-245 |
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| creator | Marra, Marco A. Heskamp, Linda Mul, Karlien Lassche, Saskia van Engelen, Baziel G.M. Heerschap, Arend Verdonschot, Nico |
| description | •Specific muscle strength of the quadriceps muscle is reduced in patients with FSHD.•MRI and muscle modeling can be used to evaluate specific muscle strength in FSHD.•FSHD patients have lower contractile muscle volume and PCSA than healthy controls.•Clinical trials for FSHD should aim for restoration of specific muscle strength.
The aim was to test whether strength per unit of muscle area (specific muscle strength) is affected in facioscapulohumeral dystrophy (FSHD) patients, as compared to healthy controls. Ten patients and ten healthy volunteers underwent an MRI examination and maximum voluntary isometric contraction measurements (MVICs) of the quadriceps muscles. Contractile muscle volume, as obtained from the MR images, was combined with the MVICs to calculate the physiological cross-sectional area (PCSA) and muscle strength using a musculoskeletal model. Subsequently, specific strength was calculated for each subject as muscle strength divided by total PCSA. FSHD patients had a reduced quadriceps muscle strength (median(1st quartile–3rd quartile): 2011 (905.4–2775) N vs. 5510 (4727–8321) N, p |
| doi_str_mv | 10.1016/j.nmd.2017.11.017 |
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The aim was to test whether strength per unit of muscle area (specific muscle strength) is affected in facioscapulohumeral dystrophy (FSHD) patients, as compared to healthy controls. Ten patients and ten healthy volunteers underwent an MRI examination and maximum voluntary isometric contraction measurements (MVICs) of the quadriceps muscles. Contractile muscle volume, as obtained from the MR images, was combined with the MVICs to calculate the physiological cross-sectional area (PCSA) and muscle strength using a musculoskeletal model. Subsequently, specific strength was calculated for each subject as muscle strength divided by total PCSA. FSHD patients had a reduced quadriceps muscle strength (median(1st quartile–3rd quartile): 2011 (905.4–2775) N vs. 5510 (4727–8321) N, p < 0.001) and total PCSA (83.6 (62.3–124.8) cm2vs. 140.1(97.1–189.9) cm2, p = 0.015) compared to healthy controls. Furthermore, the specific strength of the quadriceps was significantly lower in patients compared to healthy controls (20.9 (14.7–24.0) N/cm2vs. 41.9 (38.3–49.0) N/cm2, p < 0.001). Thus, even when correcting for atrophy and fatty infiltration, patients with FSHD generated less force per unit area of residual muscle tissue than healthy controls. Possible explanations include impaired force propagation due to fatty infiltration, reduced intrinsic force-generating capacity of the muscle fibers, or mitochondrial abnormalities leading to impaired energy metabolism.</description><identifier>ISSN: 0960-8966</identifier><identifier>EISSN: 1873-2364</identifier><identifier>DOI: 10.1016/j.nmd.2017.11.017</identifier><identifier>PMID: 29395674</identifier><language>eng</language><publisher>England: Elsevier B.V</publisher><subject>Facioscapulohumeral muscular dystrophy ; Intrinsic weakness ; Musculoskeletal modeling ; Quantitative MRI ; Specific strength</subject><ispartof>Neuromuscular disorders : NMD, 2018-03, Vol.28 (3), p.238-245</ispartof><rights>2017 Elsevier B.V.</rights><rights>Copyright © 2017 Elsevier B.V. All rights reserved.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c396t-daa4ca7af7019d0ed5e9ea9db8d3fc2062f72f556bf983c083ae1dccbf825fbf3</citedby><cites>FETCH-LOGICAL-c396t-daa4ca7af7019d0ed5e9ea9db8d3fc2062f72f556bf983c083ae1dccbf825fbf3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.nmd.2017.11.017$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29395674$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Marra, Marco A.</creatorcontrib><creatorcontrib>Heskamp, Linda</creatorcontrib><creatorcontrib>Mul, Karlien</creatorcontrib><creatorcontrib>Lassche, Saskia</creatorcontrib><creatorcontrib>van Engelen, Baziel G.M.</creatorcontrib><creatorcontrib>Heerschap, Arend</creatorcontrib><creatorcontrib>Verdonschot, Nico</creatorcontrib><title>Specific muscle strength is reduced in facioscapulohumeral dystrophy: An MRI based musculoskeletal analysis</title><title>Neuromuscular disorders : NMD</title><addtitle>Neuromuscul Disord</addtitle><description>•Specific muscle strength of the quadriceps muscle is reduced in patients with FSHD.•MRI and muscle modeling can be used to evaluate specific muscle strength in FSHD.•FSHD patients have lower contractile muscle volume and PCSA than healthy controls.•Clinical trials for FSHD should aim for restoration of specific muscle strength.
The aim was to test whether strength per unit of muscle area (specific muscle strength) is affected in facioscapulohumeral dystrophy (FSHD) patients, as compared to healthy controls. Ten patients and ten healthy volunteers underwent an MRI examination and maximum voluntary isometric contraction measurements (MVICs) of the quadriceps muscles. Contractile muscle volume, as obtained from the MR images, was combined with the MVICs to calculate the physiological cross-sectional area (PCSA) and muscle strength using a musculoskeletal model. Subsequently, specific strength was calculated for each subject as muscle strength divided by total PCSA. FSHD patients had a reduced quadriceps muscle strength (median(1st quartile–3rd quartile): 2011 (905.4–2775) N vs. 5510 (4727–8321) N, p < 0.001) and total PCSA (83.6 (62.3–124.8) cm2vs. 140.1(97.1–189.9) cm2, p = 0.015) compared to healthy controls. Furthermore, the specific strength of the quadriceps was significantly lower in patients compared to healthy controls (20.9 (14.7–24.0) N/cm2vs. 41.9 (38.3–49.0) N/cm2, p < 0.001). Thus, even when correcting for atrophy and fatty infiltration, patients with FSHD generated less force per unit area of residual muscle tissue than healthy controls. Possible explanations include impaired force propagation due to fatty infiltration, reduced intrinsic force-generating capacity of the muscle fibers, or mitochondrial abnormalities leading to impaired energy metabolism.</description><subject>Facioscapulohumeral muscular dystrophy</subject><subject>Intrinsic weakness</subject><subject>Musculoskeletal modeling</subject><subject>Quantitative MRI</subject><subject>Specific strength</subject><issn>0960-8966</issn><issn>1873-2364</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><recordid>eNp9kEtLxDAUhYMoOj5-gBvJ0k1r0k7TRlcivkARfKxDmtw4GfsytxXm35th1KWrs_nOgfMRcsxZyhkXZ8u0a22aMV6mnKcxtsiMV2WeZLmYb5MZk4IllRRij-wjLhnjRSnKXbKXyVwWopzPyMfLAMY7b2g7oWmA4higex8X1CMNYCcDlvqOOm18j0YPU9MvphaCbqhdRbgfFqtzetnRx-d7WmuM-HopYvgBDYyR051uVujxkOw43SAc_eQBebu5fr26Sx6ebu-vLh8Sk0sxJlbrudGldiXj0jKwBUjQ0taVzZ3JmMhcmbmiELWTVW5YlWvg1pjaVVnhapcfkNPN7hD6zwlwVK1HA02jO-gnVFzG-3JeCRZRvkFN6BEDODUE3-qwUpyptWO1VNGxWjtWnKsYsXPyMz_VLdi_xq_UCFxsAIgnvzwEhcZDF036AGZUtvf_zH8DJ9uQHQ</recordid><startdate>201803</startdate><enddate>201803</enddate><creator>Marra, Marco A.</creator><creator>Heskamp, Linda</creator><creator>Mul, Karlien</creator><creator>Lassche, Saskia</creator><creator>van Engelen, Baziel G.M.</creator><creator>Heerschap, Arend</creator><creator>Verdonschot, Nico</creator><general>Elsevier B.V</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>201803</creationdate><title>Specific muscle strength is reduced in facioscapulohumeral dystrophy: An MRI based musculoskeletal analysis</title><author>Marra, Marco A. ; Heskamp, Linda ; Mul, Karlien ; Lassche, Saskia ; van Engelen, Baziel G.M. ; Heerschap, Arend ; Verdonschot, Nico</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c396t-daa4ca7af7019d0ed5e9ea9db8d3fc2062f72f556bf983c083ae1dccbf825fbf3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Facioscapulohumeral muscular dystrophy</topic><topic>Intrinsic weakness</topic><topic>Musculoskeletal modeling</topic><topic>Quantitative MRI</topic><topic>Specific strength</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Marra, Marco A.</creatorcontrib><creatorcontrib>Heskamp, Linda</creatorcontrib><creatorcontrib>Mul, Karlien</creatorcontrib><creatorcontrib>Lassche, Saskia</creatorcontrib><creatorcontrib>van Engelen, Baziel G.M.</creatorcontrib><creatorcontrib>Heerschap, Arend</creatorcontrib><creatorcontrib>Verdonschot, Nico</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Neuromuscular disorders : NMD</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Marra, Marco A.</au><au>Heskamp, Linda</au><au>Mul, Karlien</au><au>Lassche, Saskia</au><au>van Engelen, Baziel G.M.</au><au>Heerschap, Arend</au><au>Verdonschot, Nico</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Specific muscle strength is reduced in facioscapulohumeral dystrophy: An MRI based musculoskeletal analysis</atitle><jtitle>Neuromuscular disorders : NMD</jtitle><addtitle>Neuromuscul Disord</addtitle><date>2018-03</date><risdate>2018</risdate><volume>28</volume><issue>3</issue><spage>238</spage><epage>245</epage><pages>238-245</pages><issn>0960-8966</issn><eissn>1873-2364</eissn><abstract>•Specific muscle strength of the quadriceps muscle is reduced in patients with FSHD.•MRI and muscle modeling can be used to evaluate specific muscle strength in FSHD.•FSHD patients have lower contractile muscle volume and PCSA than healthy controls.•Clinical trials for FSHD should aim for restoration of specific muscle strength.
The aim was to test whether strength per unit of muscle area (specific muscle strength) is affected in facioscapulohumeral dystrophy (FSHD) patients, as compared to healthy controls. Ten patients and ten healthy volunteers underwent an MRI examination and maximum voluntary isometric contraction measurements (MVICs) of the quadriceps muscles. Contractile muscle volume, as obtained from the MR images, was combined with the MVICs to calculate the physiological cross-sectional area (PCSA) and muscle strength using a musculoskeletal model. Subsequently, specific strength was calculated for each subject as muscle strength divided by total PCSA. FSHD patients had a reduced quadriceps muscle strength (median(1st quartile–3rd quartile): 2011 (905.4–2775) N vs. 5510 (4727–8321) N, p < 0.001) and total PCSA (83.6 (62.3–124.8) cm2vs. 140.1(97.1–189.9) cm2, p = 0.015) compared to healthy controls. Furthermore, the specific strength of the quadriceps was significantly lower in patients compared to healthy controls (20.9 (14.7–24.0) N/cm2vs. 41.9 (38.3–49.0) N/cm2, p < 0.001). Thus, even when correcting for atrophy and fatty infiltration, patients with FSHD generated less force per unit area of residual muscle tissue than healthy controls. Possible explanations include impaired force propagation due to fatty infiltration, reduced intrinsic force-generating capacity of the muscle fibers, or mitochondrial abnormalities leading to impaired energy metabolism.</abstract><cop>England</cop><pub>Elsevier B.V</pub><pmid>29395674</pmid><doi>10.1016/j.nmd.2017.11.017</doi><tpages>8</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Facioscapulohumeral muscular dystrophy Intrinsic weakness Musculoskeletal modeling Quantitative MRI Specific strength |
| title | Specific muscle strength is reduced in facioscapulohumeral dystrophy: An MRI based musculoskeletal analysis |
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