{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:14:48Z","timestamp":1791296088764,"version":"4.1.0"},"reference-count":45,"publisher":"Public Library of Science (PLoS)","issue":"4","license":[{"start":{"date-parts":[[2016,4,18]],"date-time":"2016-04-18T00:00:00Z","timestamp":1460937600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100007393","name":"Association Fran\u00e7aise contre les Myopathies","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007393","id-type":"DOI","asserted-by":"publisher"}]},{"name":"the Institut pour la Recherche sur la Moelle \u00e9pini\u00e8re et l'Enc\u00e9phale (IRME)"},{"name":"the program \u201cInvestissements d\u2019Avenir\u201d","award":["ANR-10-IAIHU-06"],"award-info":[{"award-number":["ANR-10-IAIHU-06"]}]}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0152439","type":"journal-article","created":{"date-parts":[[2016,4,18]],"date-time":"2016-04-18T17:33:03Z","timestamp":1461000783000},"page":"e0152439","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":22,"title":["Cervical Spinal Cord Atrophy Profile in Adult SMN1-Linked SMA"],"prefix":"10.1371","volume":"11","author":[{"given":"Mohamed-Mounir","family":"El Mendili","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timoth\u00e9e","family":"Lenglet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tanya","family":"Stojkovic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony","family":"Behin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raquel","family":"Guimar\u00e3es-Costa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois","family":"Salachas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Meininger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gaelle","family":"Bruneteau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadine","family":"Le Forestier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"Lafor\u00eat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phane","family":"Leh\u00e9ricy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Habib","family":"Benali","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre-Fran\u00e7ois","family":"Pradat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2016,4,18]]},"reference":[{"issue":"11","key":"ref1","doi-asserted-by":"crossref","first-page":"1279","DOI":"10.1007\/s00415-003-0235-9","article-title":"The spectrum of lower motor neuron syndromes","volume":"250","author":"RM Van Den Berg-Vos","year":"2003","journal-title":"J Neurol"},{"issue":"1","key":"ref2","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/0092-8674(95)90460-3","article-title":"Identification and characterization of a spinal muscular atrophy-determining gene","volume":"80","author":"S Lefebvre","year":"1995","journal-title":"Cell"},{"issue":"5","key":"ref3","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1001\/archneur.1995.00540290108025","article-title":"Natural history in proximal spinal muscular atrophy. Clinical analysis of 445 patients and suggestions for a modification of existing classifications","volume":"52","author":"K Zerres","year":"1995","journal-title":"Arch Neurol"},{"issue":"10","key":"ref4","doi-asserted-by":"crossref","first-page":"2191","DOI":"10.1016\/j.biocel.2013.05.016","article-title":"Muscle type and fiber type specificity in muscle wasting","volume":"45","author":"S Ciciliot","year":"2013","journal-title":"Int J Biochem Cell Biol"},{"issue":"1","key":"ref5","doi-asserted-by":"crossref","first-page":"30","DOI":"10.3109\/17482968.2012.701308","article-title":"Involvement of spinal sensory pathway in ALS and specificity of cord atrophy to lower motor neuron degeneration","volume":"14","author":"J Cohen-Adad","year":"2013","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"issue":"4","key":"ref6","doi-asserted-by":"crossref","first-page":"e95516","DOI":"10.1371\/journal.pone.0095516","article-title":"Multi-parametric spinal cord MRI as potential progression marker in amyotrophic lateral sclerosis","volume":"9","author":"MM El Mendili","year":"2014","journal-title":"PLoS One"},{"issue":"11","key":"ref7","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.1212\/01.WNL.0000095961.66830.03","article-title":"A comparison of muscle strength testing techniques in amyotrophic lateral sclerosis","volume":"61","year":"2003","journal-title":"Neurology"},{"issue":"7","key":"ref8","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1016\/j.nmd.2005.03.004","article-title":"A motor function measure for neuromuscular diseases. Construction and validation study","volume":"15","author":"C B\u00e9rard","year":"2005","journal-title":"Neuromuscul Disord"},{"issue":"1\u20132","key":"ref9","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/S0022-510X(99)00210-5","article-title":"The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III)","volume":"169","author":"JM Cedarbaum","year":"1999","journal-title":"J Neurol Sci"},{"issue":"1","key":"ref10","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1109\/42.668698","article-title":"A nonparametric method for automatic correction of intensity nonuniformity in MRI data","volume":"17","author":"JG Sled","year":"1998","journal-title":"IEEE Trans Med Imaging"},{"issue":"3","key":"ref11","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1148\/radiol.12120813","article-title":"Regional cervical cord atrophy and disability in multiple sclerosis: a voxel-based analysis","volume":"266","author":"P Valsasina","year":"2013","journal-title":"Radiology"},{"issue":"3","key":"ref12","doi-asserted-by":"crossref","first-page":"e0122224","DOI":"10.1371\/journal.pone.0122224","article-title":"Fast and Accurate Semi-Automated Segmentation Method of Spinal Cord MR Images at 3T Applied to the Construction of a Cervical Spinal Cord Template","volume":"10","author":"MM El Mendili","year":"2015","journal-title":"PLoS one"},{"issue":"1","key":"ref13","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1038\/sc.2010.87","article-title":"Independent spinal cord atrophy measures correlate to motor and sensory deficits in individuals with spinal cord injury","volume":"49","author":"H Lundell","year":"2011","journal-title":"Spinal Cord"},{"issue":"2","key":"ref14","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1002\/jmri.24571","article-title":"Validation of a semiautomated spinal cord segmentation method","volume":"41","author":"MM El Mendili","year":"2015","journal-title":"J Magn Reson Imaging"},{"issue":"Pt 3","key":"ref15","first-page":"701","article-title":"Spinal cord atrophy and disability in multiple sclerosis. A new reproducible and sensitive MRI method with potential to monitor disease progression","volume":"119","author":"NA Losseff","year":"1996","journal-title":"Brain"},{"issue":"1 Suppl","key":"ref16","doi-asserted-by":"crossref","first-page":"S3","DOI":"10.1016\/j.neuroimage.2008.10.043","article-title":"Automated mapping of hippocampal atrophy in 1-year repeat MRI data from 490 subjects with Alzheimer's disease, mild cognitive impairment, and elderly controls","volume":"45","author":"JH Morra","year":"2009","journal-title":"Neuroimage"},{"issue":"4","key":"ref17","first-page":"1397","article-title":"Mapping hippocampal and ventricular change in Alzheimer disease","volume":"36","author":"PM Thompson","year":"2007","journal-title":"Neuroimage"},{"key":"ref18","doi-asserted-by":"crossref","DOI":"10.1201\/9781420011814","article-title":"Randomization tests","author":"ES Edgington","year":"2007"},{"issue":"4","key":"ref19","doi-asserted-by":"crossref","first-page":"803","DOI":"10.3174\/ajnr.A4192","article-title":"Characterizing the location of spinal and vertebral levels in the human cervical spinal cord","volume":"36","author":"DW Cadotte","year":"2015","journal-title":"AJNR Am J Neuroradiol"},{"issue":"2","key":"ref20","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1016\/0022-510X(83)90009-6","article-title":"Adult spinal muscular atrophy. A report of four cases","volume":"61","author":"K Huang","year":"1983","journal-title":"J Neurol Sci"},{"issue":"5","key":"ref21","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1007\/s00401-003-0743-9","article-title":"Neuropathological analysis in spinal muscular atrophy type II","volume":"106","author":"S Araki","year":"2003","journal-title":"Acta Neuropathol"},{"issue":"1","key":"ref22","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1017\/S0317167100028535","article-title":"Sensory neuron degeneration in familial Kugelberg-Welander disease","volume":"16","author":"TR Winder","year":"1989","journal-title":"Can J Neurol Sci"},{"issue":"1","key":"ref23","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1111\/j.1440-1789.2008.00910.x","article-title":"An autopsy case of spinal muscular atrophy type III (Kugelberg-Welander disease)","volume":"29","author":"S Kuru","year":"2009","journal-title":"Neuropathology"},{"issue":"12","key":"ref24","doi-asserted-by":"crossref","first-page":"e82654","DOI":"10.1371\/journal.pone.0082654","article-title":"Selective vulnerability of spinal and cortical motor neuron subpopulations in delta7 SMA mice","volume":"8","author":"P d'Errico","year":"2013","journal-title":"PLoS One"},{"issue":"5 Suppl","key":"ref25","doi-asserted-by":"crossref","first-page":"S150","DOI":"10.1097\/00002060-199509001-00009","article-title":"Profiles of neuromuscular diseases. Spinal muscular atrophy","volume":"74","author":"GT Carter","year":"1995","journal-title":"Am J Phys Med Rehabil"},{"issue":"1","key":"ref26","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/0022-510X(67)90013-5","article-title":"Benign spinal muscular atrophy arising in childhood and adolescence","volume":"5","author":"D Gardner-Medwin","year":"1967","journal-title":"J Neurol Sci"},{"key":"ref27","first-page":"31","article-title":"Progressive spinal muscular atrophies","author":"I Hausmanowa-Petrusewicz","year":"1984"},{"issue":"9","key":"ref28","doi-asserted-by":"crossref","first-page":"1400","DOI":"10.1007\/s00415-008-0929-0","article-title":"A natural history study of late onset spinal muscular atrophy types 3b and 4","volume":"255","author":"S Piepers","year":"2008","journal-title":"J Neurol"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1016\/0022-510X(69)90093-8","article-title":"Chronic spinal muscular atrophy in adults. Part 1. The Kugelberg-Welander syndrome","volume":"9","author":"JC Meadows","year":"1969","journal-title":"J Neurol Sci"},{"issue":"11","key":"ref30","doi-asserted-by":"crossref","first-page":"e15457","DOI":"10.1371\/journal.pone.0015457","article-title":"Synaptic defects in the spinal and neuromuscular circuitry in a mouse model of spinal muscular atrophy","volume":"5","author":"KK Ling","year":"2010","journal-title":"PLoS One"},{"issue":"1","key":"ref31","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1016\/j.molmed.2012.11.002","article-title":"Spinal muscular atrophy: going beyond the motor neuron","volume":"19","author":"G Hamilton","year":"2013","journal-title":"Trends Mol Med"},{"issue":"16","key":"ref32","doi-asserted-by":"crossref","first-page":"2552","DOI":"10.1093\/hmg\/ddn156","article-title":"Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy","volume":"17","author":"S Kariya","year":"2008","journal-title":"Hum Mol Genet"},{"issue":"7","key":"ref33","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1093\/hmg\/ddm367","article-title":"Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophy","volume":"17","author":"LM Murray","year":"2008","journal-title":"Hum Mol Genet"},{"issue":"3","key":"ref34","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1523\/JNEUROSCI.4434-08.2009","article-title":"Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy mice","volume":"29","author":"L Kong","year":"2009","journal-title":"J Neurosci"},{"issue":"20","key":"ref35","doi-asserted-by":"crossref","first-page":"2050","DOI":"10.1212\/WNL.0b013e3182749eca","article-title":"Dysfunction of the neuromuscular junction in spinal muscular atrophy types 2 and 3","volume":"79","author":"RI Wadman","year":"2012","journal-title":"Neurology"},{"issue":"16","key":"ref36","doi-asserted-by":"crossref","first-page":"4249","DOI":"10.1093\/hmg\/ddu142","article-title":"Myogenic program dysregulation is contributory to disease pathogenesis in spinal muscular atrophy","volume":"23","author":"JG Boyer","year":"2014","journal-title":"Hum Mol Genet"},{"issue":"8","key":"ref37","doi-asserted-by":"crossref","first-page":"1737","DOI":"10.1002\/cne.23010","article-title":"Failure of lower motor neuron radial outgrowth precedes retrograde degeneration in a feline model of spinal muscular atrophy","volume":"520","author":"EN Wakeling","year":"2012","journal-title":"J Comp Neurol"},{"issue":"5","key":"ref38","doi-asserted-by":"crossref","first-page":"407","DOI":"10.3109\/17482968.2011.649760","article-title":"Spinal cord markers in ALS: diagnostic and biomarker considerations","volume":"13","author":"P Bede","year":"2012","journal-title":"Amyotroph Lateral Scler"},{"issue":"12","key":"ref39","doi-asserted-by":"crossref","first-page":"1069","DOI":"10.1016\/j.nmd.2012.06.352","article-title":"Change in muscle strength over time in spinal muscular atrophy types II and III. A long-term follow-up study","volume":"22","author":"U Werlauff","year":"2012","journal-title":"Neuromuscul Disord"},{"issue":"3","key":"ref40","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1136\/jnnp.50.3.285","article-title":"Focal cervical poliopathy causing juvenile muscular atrophy of distal upper extremity: a pathological study","volume":"50","author":"K Hirayama","year":"1987","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"1","key":"ref41","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1007\/s00415-013-7193-7","article-title":""Sand-watch" spinal cord: a case of inferior cervical spinal cord atrophy","volume":"261","author":"P Bede","year":"2014","journal-title":"J Neurol"},{"issue":"3","key":"ref42","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1007\/s10334-013-0403-6","article-title":"Construction of an in vivo human spinal cord atlas based on high-resolution MR images at cervical and thoracic levels: preliminary results","volume":"27","author":"M Taso","year":"2014","journal-title":"MAGMA"},{"key":"ref43","article-title":"A reliable spatially normalized template of the human spinal cord\u2014Applications to automated white matter\/gray matter segmentation and Tensor-Based Morphometry (TBM) mapping of gray matter alterations occurring with age","author":"M Taso","year":"2015","journal-title":"Neuroimage"},{"issue":"3","key":"ref44","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1177\/0883073810380457","article-title":"Muscle volume estimation by magnetic resonance imaging in spinal muscular atrophy","volume":"26","author":"DM Sproule","year":"2011","journal-title":"J Child Neurol"},{"issue":"5","key":"ref45","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1002\/mus.23233","article-title":"Electrical impedance myography in spinal muscular atrophy: a longitudinal study","volume":"45","author":"SB Rutkove","year":"2012","journal-title":"Muscle Nerve"}],"updated-by":[{"DOI":"10.1371\/journal.pone.0167886","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2016,12,1]],"date-time":"2016-12-01T00:00:00Z","timestamp":1480550400000}}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0152439","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,9,18]],"date-time":"2020-09-18T20:38:14Z","timestamp":1600461494000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0152439"}},"subtitle":[],"editor":[{"given":"Cedric","family":"Raoul","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2016,4,18]]},"references-count":45,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2016,4,18]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0152439","relation":{"correction":[{"id-type":"doi","id":"10.1371\/journal.pone.0167886","asserted-by":"object"}]},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,4,18]]}}}