{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T18:09:43Z","timestamp":1790014183617,"version":"4.0.1"},"reference-count":13,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,3,23]],"date-time":"2021-03-23T00:00:00Z","timestamp":1616457600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2021,3,23]],"date-time":"2021-03-23T00:00:00Z","timestamp":1616457600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100012041","name":"Versus Arthritis","doi-asserted-by":"publisher","award":["20164 and 12593"],"award-info":[{"award-number":["20164 and 12593"]}],"id":[{"id":"10.13039\/501100012041","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001279","name":"Great Ormond Street Hospital Charity","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001279","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Rheumatol"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>The aim of this study was to: (i) describe the abnormalities seen on brain imaging in a group of children with en coup de sabre (EDCS) with\/without Parry-Romberg syndrome (PRS); and (ii) identify clinical predictors of brain imaging abnormalities.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>This was a single centre (Great Ormond Street Hospital, London) retrospective case series of patients with ECDS\/PRS seen from 2000 to 2018. We identified patients with cutaneous manifestations consistent with the clinical descriptions of ECDS\/PRS. Presenting clinical, laboratory, and radiological brain findings are described. Results are expressed as medians and ranges or frequencies and percentages. Fisher\u2019s exact test was used to identify clinical associations with magnetic resonance imaging (MRI) abnormalities.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Fourteen patients were studied: 6 males and 8 females; median age 14\u2009years (range 3\u201320). We observed neuroimaging abnormalities in 2\/6 ECDS and 5\/8 ECDS\/PRS patients. White matter signal abnormality, dystrophic calcification, leptomeningeal enhancement, and sulcal crowding were the typical findings on brain imaging. A total of 50% of patients had no MRI abnormality despite some of these patients having neurological symptoms. The presence of seizures was significantly associated with ipsilateral enhanced white matter signalling on MRI (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.05).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>In summary, we observed several distinct radiographic patterns associated with ECDS\/PRS. Seizure disorder was strongly associated with the presence of ipsilateral enhanced white matter signalling. Improved neuroimaging techniques that combine morphological with functional imaging may improve the detection rate of brain involvement in children with ECDS\/PRS in the future.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12969-021-00512-6","type":"journal-article","created":{"date-parts":[[2021,3,23]],"date-time":"2021-03-23T12:13:22Z","timestamp":1616501602000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre"],"prefix":"10.1186","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8184-7208","authenticated-orcid":false,"given":"Harry","family":"Knights","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Minas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Faraan","family":"Khan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lindsay","family":"Shaw","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muthana","family":"Al Obaidi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kshitij","family":"Mankad","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Despina","family":"Eleftheriou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,3,23]]},"reference":[{"issue":"1","key":"512_CR1","doi-asserted-by":"publisher","first-page":"62","DOI":"10.1590\/abd1806-4841.20152890","volume":"90","author":"MF Careta","year":"2015","unstructured":"Careta MF, Romiti R. Localized scleroderma: clinical spectrum and therapeutic update. An Bras Dermatol. 2015;90(1):62.","journal-title":"An Bras Dermatol"},{"issue":"2","key":"512_CR2","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1016\/j.jaad.2006.10.959","volume":"56","author":"MM Tollefson","year":"2007","unstructured":"Tollefson MM, Witman PM. En coup de sabre morphea and parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol. 2007;56(2):257\u201363.","journal-title":"J Am Acad Dermatol"},{"key":"512_CR3","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1186\/s13023-015-0250-9","volume":"10","author":"SN Tolkachjov","year":"2015","unstructured":"Tolkachjov SN, Patel NG, Tollefson MM. Progressive hemifacial atrophy: a review. Orphanet J Rare Dis. 2015;10:39.","journal-title":"Orphanet J Rare Dis"},{"issue":"5","key":"512_CR4","doi-asserted-by":"publisher","first-page":"674","DOI":"10.1212\/WNL.61.5.674","volume":"61","author":"J Stone","year":"2003","unstructured":"Stone J. Parry-Romberg syndrome: a global survey of 205 patients using the internet. Neurology. 2003;61(5):674.","journal-title":"Neurology."},{"issue":"4","key":"512_CR5","first-page":"751","volume":"18","author":"RC Cory","year":"1997","unstructured":"Cory RC, Clayman DA, Faillace WJ, McKee SW, Gama CH. Clinical and radiologic findings in progressive facial hemiatrophy (parry-Romberg syndrome). Am J Neuroradiol. 1997;18(4):751\u20137.","journal-title":"Am J Neuroradiol"},{"issue":"8","key":"512_CR6","doi-asserted-by":"publisher","first-page":"1019","DOI":"10.1136\/annrheumdis-2018-214697","volume":"78","author":"F Zulian","year":"2019","unstructured":"Zulian F, Culpo R, Sperotto F, Anton J, Avcin T, Baildam EM, et al. Consensus-based recommendations for the management of juvenile localised scleroderma. Ann Rheum Dis. 2019;78(8):1019.","journal-title":"Ann Rheum Dis"},{"issue":"5","key":"512_CR7","doi-asserted-by":"publisher","first-page":"321","DOI":"10.1016\/S1010-5182(03)00028-3","volume":"31","author":"SB Moko","year":"2003","unstructured":"Moko SB, Mistry Y. Blandin de Chalain TM. Parry-Romberg syndrome: intracranial MRI appearances. J Cranio-Maxillofacial Surg. 2003;31(5):321\u20134.","journal-title":"J Cranio-Maxillofacial Surg"},{"issue":"3","key":"512_CR8","doi-asserted-by":"publisher","first-page":"335","DOI":"10.1016\/j.semarthrit.2013.05.002","volume":"43","author":"TN Amaral","year":"2013","unstructured":"Amaral TN, Peres FA, Lapa AT, Marques-Neto JF, Appenzeller S. Neurologic involvement in scleroderma: a systematic review. 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Childs Nerv Syst. 2006;22(4):409.","journal-title":"Childs Nerv Syst"}],"container-title":["Pediatric Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s12969-021-00512-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s12969-021-00512-6\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s12969-021-00512-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,3,23]],"date-time":"2021-03-23T12:15:16Z","timestamp":1616501716000},"score":1,"resource":{"primary":{"URL":"https:\/\/ped-rheum.biomedcentral.com\/articles\/10.1186\/s12969-021-00512-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,3,23]]},"references-count":13,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["512"],"URL":"https:\/\/doi.org\/10.1186\/s12969-021-00512-6","relation":{},"ISSN":["1546-0096"],"issn-type":[{"value":"1546-0096","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,3,23]]},"assertion":[{"value":"13 October 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 February 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"23 March 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Ethical approval was given by the Institute of Child Health\/ Great Ormond Street Research Ethics Committee for a retrospective case notes review; since this was a retrospective case note review using de-identified data, written consent from patients was not required.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Since this was a retrospective case note review using de-identified data, written consent from patients was not required.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"All authors declare no conflicts of interest relating to this work.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"42"}}