{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T21:46:55Z","timestamp":1759960015834,"version":"3.41.2"},"reference-count":23,"publisher":"Oxford University Press (OUP)","issue":"1085","license":[{"start":{"date-parts":[[2018,2,13]],"date-time":"2018-02-13T00:00:00Z","timestamp":1518480000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,5,1]]},"abstract":"<jats:sec>\n                  <jats:title>Objective:<\/jats:title>\n                  <jats:p>To evaluate spinal MRI features of mucopolysaccharidosis (MPS) VI and to assess the correlation with clinical findings.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods:<\/jats:title>\n                  <jats:p>We retrospectively evaluated spinal MRI scans and clinical findings at the time of imaging in 14 patients (8 male, 6 female) with MPS VI. Craniometric measurements were performed and the images were assessed for bony anomalies, spinal stenosis and spinal cord compression. The degree of cervical cord compression was scored and correlated with neurological examination findings at the time of imaging. Vertebral alignment, structural changes in spinal ligaments and intervertebral discs were also assessed.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results:<\/jats:title>\n                  <jats:p>All patients had cervical stenosis due to bony stenosis and thickened retrodental tissue (median: 6.05\u2009mm, range 3.3\u20138\u2009mm). Retrodental tissue thickness was found to increase with age (p = 0.042). Compressive myelopathy was detected at upper cervical level in 11 (79%) and lower thoracic level in 2 patients (14%). Significant inverse correlation was found between cervical myelopathy scores and neurological strength scores. The most common bony changes were hypo\/dysplastic odontoid; cervical platyspondyly with anterior inferior beaking; thoracic posterior end plate depressions and lumbar posterior scalloping. Kyphosis due to retrolisthesis of the beaked lumbar vertebrae and acute sacrococcygeal angulations were other remarkable findings.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion:<\/jats:title>\n                  <jats:p>MRI is an essential component in evaluation of spinal involvement in MPS VI, and scanning of the entire spine is recommended to rule out thoracic cord compression.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Advances in knowledge:<\/jats:title>\n                  <jats:p>This study provides a detailed description of spinal MRI findings in MPS VI and underlines the role of MRI in management of cord compression.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1259\/bjr.20170744","type":"journal-article","created":{"date-parts":[[2018,1,29]],"date-time":"2018-01-29T16:39:00Z","timestamp":1517243940000},"source":"Crossref","is-referenced-by-count":9,"title":["Evaluation of spinal involvement in children with mucopolysaccharidosis VI: the role of MRI"],"prefix":"10.1093","volume":"91","author":[{"given":"Elif","family":"Bulut","sequence":"first","affiliation":[{"name":"Department of Radiology, Hacettepe University Faculty of Medicine , Ankara , Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emine","family":"Pektas","sequence":"additional","affiliation":[{"name":"Department of Pediatric Metabolism, Hacettepe University Faculty of Medicine , Ankara , Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hatice S","family":"Sivri","sequence":"additional","affiliation":[{"name":"Department of Pediatric Metabolism, Hacettepe University Faculty of Medicine , Ankara , Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Burcak","family":"Bilginer","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Hacettepe University Faculty of Medicine , Ankara , Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mumtaz M","family":"Umaroglu","sequence":"additional","affiliation":[{"name":"Department of Biostatistics, Hacettepe University , Ankara , Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Burce","family":"Ozgen","sequence":"additional","affiliation":[{"name":"Department of Radiology, Hacettepe University Faculty of Medicine , Ankara , Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2018,2,13]]},"reference":[{"key":"2024050214244448700_b1","first-page":"3421","volume-title":"The metabolic and molecular basis of inherited disease","author":"Neufeld","year":"2001","edition":"8th ed"},{"key":"2024050214244448700_b2","doi-asserted-by":"crossref","DOI":"10.1186\/1750-1172-5-5","article-title":"Mucopolysaccharidosis VI","volume":"5","author":"Valayannopoulos","year":"2010","journal-title":"Orphanet J Rare Dis"},{"key":"2024050214244448700_b3","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.ymgme.2012.07.018","article-title":"A multinational, multidisciplinary consensus for the diagnosis and management of spinal cord compression among patients with mucopolysaccharidosis VI","volume":"107","author":"Solanki","year":"2012","journal-title":"Mol Genet Metab"},{"key":"2024050214244448700_b4","doi-asserted-by":"crossref","first-page":"1005","DOI":"10.1007\/s10545-013-9591-5","article-title":"Craniocervical decompression in patients with mucopolysaccharidosis VI: development of a scoring system to determine indication and outcome of surgery","volume":"36","author":"Lampe","year":"2013","journal-title":"J Inherit Metab Dis"},{"key":"2024050214244448700_b5","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1016\/j.ymgme.2011.07.019","article-title":"Spinal cord compression in young children with type VI mucopolysaccharidosis","volume":"104","author":"Horovitz","year":"2011","journal-title":"Mol Genet Metab"},{"key":"2024050214244448700_b6","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1159\/000345635","article-title":"Spinal cord compression in maroteaux-lamy syndrome: case report and review of the literature with effects of enzyme replacement therapy","volume":"48","author":"Jurecka","year":"2012","journal-title":"Pediatr Neurosurg"},{"key":"2024050214244448700_b7","doi-asserted-by":"crossref","first-page":"1448","DOI":"10.1148\/rg.2016150168","article-title":"Neuroimaging findings in patients with mucopolysaccharidosis: what you really need to know","volume":"36","author":"Reichert","year":"2016","journal-title":"Radiographics"},{"key":"2024050214244448700_b8","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1016\/j.ymgme.2016.06.001","article-title":"Cervical cord compression in mucopolysaccharidosis VI (MPS VI): findings from the MPS VI clinical surveillance program (CSP)","volume":"118","author":"Solanki","year":"2016","journal-title":"Mol Genet Metab"},{"key":"2024050214244448700_b9","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1259\/bjr\/59197814","article-title":"Pictorial review of mucopolysaccharidosis with emphasis on MRI features of brain and spine","volume":"84","author":"Rasalkar","year":"2011","journal-title":"Br J Radiol"},{"issue":"Suppl 5","key":"2024050214244448700_b10","doi-asserted-by":"crossref","first-page":"v19","DOI":"10.1093\/rheumatology\/ker397","article-title":"Musculoskeletal manifestations of mucopolysaccharidoses","volume":"50","author":"Morishita","year":"2011","journal-title":"Rheumatology"},{"key":"2024050214244448700_b11","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1590\/S0100-879X2012007500072","article-title":"Severity score system for progressive myelopathy: development and validation of a new clinical scale","volume":"45","author":"Castilhos","year":"2012","journal-title":"Braz J Med Biol Res"},{"key":"2024050214244448700_b12","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1148\/radiographics.14.2.8190952","article-title":"Craniovertebral junction: normal anatomy, craniometry, and congenital anomalies","volume":"14","author":"Smoker","year":"1994","journal-title":"Radiographics"},{"key":"2024050214244448700_b13","doi-asserted-by":"crossref","first-page":"1484","DOI":"10.1136\/adc.63.12.1484","article-title":"Atlantoaxial instability and abnormalities of the odontoid in down's syndrome","volume":"63","author":"Elliott","year":"1988","journal-title":"Arch Dis Child"},{"key":"2024050214244448700_b14","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1007\/978-1-4471-1844-2_11","volume-title":"Measurements in pediatric radiology","author":"Pettersson","year":"1991"},{"key":"2024050214244448700_b15","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1016\/j.clineuro.2004.05.003","article-title":"Multilevel myelopathy in maroteaux-lamy syndrome and review of the literature","volume":"107","author":"Mut","year":"2005","journal-title":"Clin Neurol Neurosurg"},{"key":"2024050214244448700_b16","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1159\/000050383","article-title":"Neurosurgical interventions in children with maroteaux-lamy syndrome. Case report and review of the literature","volume":"35","author":"Vougioukas","year":"2001","journal-title":"Pediatr Neurosurg"},{"key":"2024050214244448700_b17","doi-asserted-by":"crossref","first-page":"5","DOI":"10.3174\/ajnr.A2832","article-title":"Brain and spinal MR imaging findings in mucopolysaccharidoses: a review","volume":"34","author":"Zafeiriou","year":"2013","journal-title":"AJNR Am J Neuroradiol"},{"key":"2024050214244448700_b18","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1097\/00002142-200202000-00002","article-title":"Magnetic resonance of metabolic and degenerative diseases in children","volume":"13","author":"Faerber","year":"2002","journal-title":"Top Magn Reson Imaging"},{"key":"2024050214244448700_b19","doi-asserted-by":"crossref","first-page":"2751","DOI":"10.1007\/s00586-014-3186-1","article-title":"Combined spinal arthrodesis with instrumentation for the management of progressive thoracolumbar kyphosis in children with mucopolysaccharidosis","volume":"23","author":"Garrido","year":"2014","journal-title":"Eur Spine J"},{"key":"2024050214244448700_b20","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1016\/j.ymgme.2012.03.014","article-title":"Pathogenesis of lumbar spine disease in mucopolysaccharidosis VII","volume":"107","author":"Smith","year":"2012","journal-title":"Mol Genet Metab"},{"key":"2024050214244448700_b21","doi-asserted-by":"crossref","first-page":"E1437","DOI":"10.1097\/BRS.0b013e3182a45e07","article-title":"Magnetic resonance imaging morphology and morphometry of the coccyx in coccydynia","volume":"38","author":"Woon","year":"2013","journal-title":"Spine"},{"key":"2024050214244448700_b22","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1007\/s10545-012-9559-x","article-title":"Brain magnetic resonance imaging findings in patients with mucopolysaccharidosis VI","volume":"36","author":"Azevedo","year":"2013","journal-title":"J Inherit Metab Dis"},{"key":"2024050214244448700_b23","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1016\/j.braindev.2013.07.016","article-title":"New insights in mucopolysaccharidosis type VI: neurological perspective","volume":"36","author":"Borlot","year":"2014","journal-title":"Brain Dev"}],"container-title":["The British Journal of Radiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/bjr\/article-pdf\/91\/1085\/20170744\/57380236\/bjr.20170744.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/bjr\/article-pdf\/91\/1085\/20170744\/57380236\/bjr.20170744.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,2]],"date-time":"2024-05-02T14:27:50Z","timestamp":1714660070000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/bjr\/article\/doi\/10.1259\/bjr.20170744\/7449430"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,2,13]]},"references-count":23,"journal-issue":{"issue":"1085","published-online":{"date-parts":[[2018,2,13]]},"published-print":{"date-parts":[[2018,5,1]]}},"URL":"https:\/\/doi.org\/10.1259\/bjr.20170744","relation":{},"ISSN":["0007-1285","1748-880X"],"issn-type":[{"type":"print","value":"0007-1285"},{"type":"electronic","value":"1748-880X"}],"subject":[],"published-other":{"date-parts":[[2018,5,1]]},"published":{"date-parts":[[2018,2,13]]},"article-number":"20170744"}}