Depletion and sizes of motor units in spinal muscular atrophy
Motor unit number estimation (MUNE) was applied to the biceps brachii muscles of 13 young patients (age 5–24 years) with spinal muscular atrophy (SMA) and the results compared with those of healthy control subjects matched for age and gender. In the SMA patients, all motor unit (MU) estimates fell b...
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| Veröffentlicht in: | Muscle & nerve 2001-09, Vol.24 (9), p.1168-1172 |
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| creator | Galea, Victoria Fehlings, Darcy Kirsch, Susan McComas, Alan |
| description | Motor unit number estimation (MUNE) was applied to the biceps brachii muscles of 13 young patients (age 5–24 years) with spinal muscular atrophy (SMA) and the results compared with those of healthy control subjects matched for age and gender. In the SMA patients, all motor unit (MU) estimates fell below the control range, and there was good correspondence between the values for the two arms in the same subject. No correlation could be found between the MUNEs and the severity of the weakness. This unexpected result was attributed to the presence of small and normal‐sized MUs in the muscles of patients, in addition to MUs that appeared to be considerably enlarged. The threefold mean increase in MU potential size was insufficient to compensate for the MU loss. In addition, the study confirmed that there are, on average, approximately 130 MUs in the healthy biceps brachii muscle. © 2001 John Wiley & Sons, Inc. Muscle Nerve 24: 1166–1172, 2001 |
| doi_str_mv | 10.1002/mus.1128 |
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In the SMA patients, all motor unit (MU) estimates fell below the control range, and there was good correspondence between the values for the two arms in the same subject. No correlation could be found between the MUNEs and the severity of the weakness. This unexpected result was attributed to the presence of small and normal‐sized MUs in the muscles of patients, in addition to MUs that appeared to be considerably enlarged. The threefold mean increase in MU potential size was insufficient to compensate for the MU loss. In addition, the study confirmed that there are, on average, approximately 130 MUs in the healthy biceps brachii muscle. © 2001 John Wiley & Sons, Inc. Muscle Nerve 24: 1166–1172, 2001</description><identifier>ISSN: 0148-639X</identifier><identifier>EISSN: 1097-4598</identifier><identifier>DOI: 10.1002/mus.1128</identifier><identifier>PMID: 11494269</identifier><identifier>CODEN: MUNEDE</identifier><language>eng</language><publisher>New York: John Wiley & Sons, Inc</publisher><subject>Adolescent ; Adult ; Biological and medical sciences ; Cell Count - standards ; Child ; Child, Preschool ; collateral reinnervation ; Electrodiagnosis. Electric activity recording ; Electromyography - methods ; Electromyography - standards ; Female ; Humans ; Investigative techniques, diagnostic techniques (general aspects) ; Male ; Medical sciences ; Motor Neurons - pathology ; Motor Neurons - physiology ; motor units ; muscle denervation ; Muscle, Skeletal - innervation ; Muscle, Skeletal - physiopathology ; Nervous system ; Reproducibility of Results ; Spinal Muscular Atrophies of Childhood - pathology ; Spinal Muscular Atrophies of Childhood - physiopathology ; spinal muscular atrophy</subject><ispartof>Muscle & nerve, 2001-09, Vol.24 (9), p.1168-1172</ispartof><rights>Copyright © 2001 John Wiley & Sons, Inc.</rights><rights>2002 INIST-CNRS</rights><rights>Copyright 2001 John Wiley & Sons, Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3858-6c7731aebbddae4af6cfee7048d6044940258222fa767b6a002605b902e3fe033</citedby><cites>FETCH-LOGICAL-c3858-6c7731aebbddae4af6cfee7048d6044940258222fa767b6a002605b902e3fe033</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fmus.1128$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fmus.1128$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1417,27924,27925,45574,45575</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=14083902$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11494269$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Galea, Victoria</creatorcontrib><creatorcontrib>Fehlings, Darcy</creatorcontrib><creatorcontrib>Kirsch, Susan</creatorcontrib><creatorcontrib>McComas, Alan</creatorcontrib><title>Depletion and sizes of motor units in spinal muscular atrophy</title><title>Muscle & nerve</title><addtitle>Muscle Nerve</addtitle><description>Motor unit number estimation (MUNE) was applied to the biceps brachii muscles of 13 young patients (age 5–24 years) with spinal muscular atrophy (SMA) and the results compared with those of healthy control subjects matched for age and gender. In the SMA patients, all motor unit (MU) estimates fell below the control range, and there was good correspondence between the values for the two arms in the same subject. No correlation could be found between the MUNEs and the severity of the weakness. This unexpected result was attributed to the presence of small and normal‐sized MUs in the muscles of patients, in addition to MUs that appeared to be considerably enlarged. The threefold mean increase in MU potential size was insufficient to compensate for the MU loss. In addition, the study confirmed that there are, on average, approximately 130 MUs in the healthy biceps brachii muscle. © 2001 John Wiley & Sons, Inc. Muscle Nerve 24: 1166–1172, 2001</description><subject>Adolescent</subject><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Cell Count - standards</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>collateral reinnervation</subject><subject>Electrodiagnosis. Electric activity recording</subject><subject>Electromyography - methods</subject><subject>Electromyography - standards</subject><subject>Female</subject><subject>Humans</subject><subject>Investigative techniques, diagnostic techniques (general aspects)</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Motor Neurons - pathology</subject><subject>Motor Neurons - physiology</subject><subject>motor units</subject><subject>muscle denervation</subject><subject>Muscle, Skeletal - innervation</subject><subject>Muscle, Skeletal - physiopathology</subject><subject>Nervous system</subject><subject>Reproducibility of Results</subject><subject>Spinal Muscular Atrophies of Childhood - pathology</subject><subject>Spinal Muscular Atrophies of Childhood - physiopathology</subject><subject>spinal muscular atrophy</subject><issn>0148-639X</issn><issn>1097-4598</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2001</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp10E1P3DAQBmCrKioLrcQvqHxpxSV0bCe2c-gBLd-i5cBCuVlOMlFN81U7ESy_HqON4MRpLo_mfWcI2WNwwAD4j3YKB4xx_YEsGOQqSbNcfyQLYKlOpMjvtslOCPcAwLRUn8g2Y2mecpkvyM8jHBocXd9R21U0uCcMtK9p24-9p1PnxkBdR8PgOtvQmFNOjfXUjr4f_q4_k63aNgG_zHOX3Jwcr5ZnyeXV6fny8DIphc5ihVIpwSwWRVVZTG0tyxpRQaorCWmsAjzTnPPaKqkKaeNJErIiB46iRhBil3zf7B18_3_CMJrWhRKbxnbYT8EoxoDrTEW4v4Gl70PwWJvBu9b6tWFgXl5l4gnm5VWRfp13TkWL1RucfxPBtxnYUNqm9rYrXXhzKWgRK0aXbNyDa3D9bqD5dXM9B8_ehREfX731_4xUQmXmz-9To1fL25W6VeZCPANk_44S</recordid><startdate>200109</startdate><enddate>200109</enddate><creator>Galea, Victoria</creator><creator>Fehlings, Darcy</creator><creator>Kirsch, Susan</creator><creator>McComas, Alan</creator><general>John Wiley & Sons, Inc</general><general>Wiley</general><scope>BSCLL</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>200109</creationdate><title>Depletion and sizes of motor units in spinal muscular atrophy</title><author>Galea, Victoria ; Fehlings, Darcy ; Kirsch, Susan ; McComas, Alan</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3858-6c7731aebbddae4af6cfee7048d6044940258222fa767b6a002605b902e3fe033</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2001</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>Cell Count - standards</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>collateral reinnervation</topic><topic>Electrodiagnosis. Electric activity recording</topic><topic>Electromyography - methods</topic><topic>Electromyography - standards</topic><topic>Female</topic><topic>Humans</topic><topic>Investigative techniques, diagnostic techniques (general aspects)</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Motor Neurons - pathology</topic><topic>Motor Neurons - physiology</topic><topic>motor units</topic><topic>muscle denervation</topic><topic>Muscle, Skeletal - innervation</topic><topic>Muscle, Skeletal - physiopathology</topic><topic>Nervous system</topic><topic>Reproducibility of Results</topic><topic>Spinal Muscular Atrophies of Childhood - pathology</topic><topic>Spinal Muscular Atrophies of Childhood - physiopathology</topic><topic>spinal muscular atrophy</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Galea, Victoria</creatorcontrib><creatorcontrib>Fehlings, Darcy</creatorcontrib><creatorcontrib>Kirsch, Susan</creatorcontrib><creatorcontrib>McComas, Alan</creatorcontrib><collection>Istex</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Muscle & nerve</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Galea, Victoria</au><au>Fehlings, Darcy</au><au>Kirsch, Susan</au><au>McComas, Alan</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Depletion and sizes of motor units in spinal muscular atrophy</atitle><jtitle>Muscle & nerve</jtitle><addtitle>Muscle Nerve</addtitle><date>2001-09</date><risdate>2001</risdate><volume>24</volume><issue>9</issue><spage>1168</spage><epage>1172</epage><pages>1168-1172</pages><issn>0148-639X</issn><eissn>1097-4598</eissn><coden>MUNEDE</coden><abstract>Motor unit number estimation (MUNE) was applied to the biceps brachii muscles of 13 young patients (age 5–24 years) with spinal muscular atrophy (SMA) and the results compared with those of healthy control subjects matched for age and gender. In the SMA patients, all motor unit (MU) estimates fell below the control range, and there was good correspondence between the values for the two arms in the same subject. No correlation could be found between the MUNEs and the severity of the weakness. This unexpected result was attributed to the presence of small and normal‐sized MUs in the muscles of patients, in addition to MUs that appeared to be considerably enlarged. The threefold mean increase in MU potential size was insufficient to compensate for the MU loss. In addition, the study confirmed that there are, on average, approximately 130 MUs in the healthy biceps brachii muscle. © 2001 John Wiley & Sons, Inc. Muscle Nerve 24: 1166–1172, 2001</abstract><cop>New York</cop><pub>John Wiley & Sons, Inc</pub><pmid>11494269</pmid><doi>10.1002/mus.1128</doi><tpages>5</tpages></addata></record> |
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| subjects | Adolescent Adult Biological and medical sciences Cell Count - standards Child Child, Preschool collateral reinnervation Electrodiagnosis. Electric activity recording Electromyography - methods Electromyography - standards Female Humans Investigative techniques, diagnostic techniques (general aspects) Male Medical sciences Motor Neurons - pathology Motor Neurons - physiology motor units muscle denervation Muscle, Skeletal - innervation Muscle, Skeletal - physiopathology Nervous system Reproducibility of Results Spinal Muscular Atrophies of Childhood - pathology Spinal Muscular Atrophies of Childhood - physiopathology spinal muscular atrophy |
| title | Depletion and sizes of motor units in spinal muscular atrophy |
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