{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T11:01:15Z","timestamp":1777892475315,"version":"3.51.4"},"reference-count":12,"publisher":"SAGE Publications","issue":"2","license":[{"start":{"date-parts":[[2018,6,1]],"date-time":"2018-06-01T00:00:00Z","timestamp":1527811200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of the Royal College of Physicians of Edinburgh"],"published-print":{"date-parts":[[2018,6]]},"abstract":"<jats:p>Type A aortic dissection is a rare but important cardiac surgical emergency. Few reports exist in the literature describing anterior spinal artery syndrome as a presenting feature.<\/jats:p>\n                  <jats:p>We report a case of anterior spinal artery syndrome due to aortic dissection in a patient with Marfan syndrome caused by a novel fibrillin mutation. A 53-year old female presented with chest pain and sudden-onset paralysis. Neurological examination revealed normal upper limb examination, reduced lower limb power and reflexes but normal sensation. CT scanning revealed type A acute aortic dissection which was treated with emergent cardiac surgical repair. At clinic follow up 3 years later, signs of Marfan syndrome were opportunistically noted and genetic testing revealed a novel mutation in the Fibrillin 1 gene.<\/jats:p>\n                  <jats:p>This case emphasises the importance of a good initial clinical assessment, including thorough neurological examination, as well as a low threshold of clinical suspicion for an aortic dissection in such a constellation of symptoms. The importance of family history should also be emphasised given the coincidental diagnosis of Marfan syndrome in a first-degree relative. Furthermore, this case illustrates the classical signs of anterior spinal artery syndrome.<\/jats:p>","DOI":"10.4997\/jrcpe.2018.204","type":"journal-article","created":{"date-parts":[[2018,6,15]],"date-time":"2018-06-15T06:20:08Z","timestamp":1529043608000},"page":"120-123","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Anterior Spinal Artery Syndrome from Type a Aortic Dissection in a Patient with Marfan Syndrome Due to a Novel Fibrillin Mutation"],"prefix":"10.1177","volume":"48","author":[{"given":"K","family":"Cheng","sequence":"first","affiliation":[{"name":"Honorary Clinical Research Fellow, Royal Brompton Hospital, London"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M","family":"Perenyei","sequence":"additional","affiliation":[{"name":"Core trainee, John Radcliffe Hospital, Oxford, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R","family":"Sayeed","sequence":"additional","affiliation":[{"name":"Consultant Cardiothoracic Surgeon, John Radcliffe Hospital, Oxford, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2018,6,1]]},"reference":[{"key":"bibr1-jrcpe.2018.204","doi-asserted-by":"publisher","DOI":"10.1053\/apmr.2000.7158"},{"key":"bibr2-jrcpe.2018.204","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajem.2008.03.051"},{"key":"bibr3-jrcpe.2018.204","doi-asserted-by":"publisher","DOI":"10.1016\/S0196-0644(88)80567-5"},{"key":"bibr4-jrcpe.2018.204","doi-asserted-by":"publisher","DOI":"10.1038\/sj.sc.3101087"},{"key":"bibr5-jrcpe.2018.204","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajem.2007.09.020"},{"key":"bibr6-jrcpe.2018.204","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/67.2.69"},{"key":"bibr7-jrcpe.2018.204","doi-asserted-by":"publisher","DOI":"10.1016\/j.jvs.2011.05.007"},{"key":"bibr8-jrcpe.2018.204","doi-asserted-by":"publisher","DOI":"10.4103\/0976-3147.203825"},{"key":"bibr9-jrcpe.2018.204","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(88)90117-9"},{"key":"bibr10-jrcpe.2018.204","first-page":"369","volume":"17","author":"Chiang J","year":"2005","journal-title":"Tzu Chi Med J"},{"key":"bibr11-jrcpe.2018.204","first-page":"49","volume":"7","author":"Oh E","year":"2005","journal-title":"J Korean Soc Clin Neurophysiol"},{"key":"bibr12-jrcpe.2018.204","doi-asserted-by":"publisher","DOI":"10.1002\/humu.10249"}],"container-title":["Journal of the Royal College of Physicians of Edinburgh"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.4997\/jrcpe.2018.204","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.4997\/jrcpe.2018.204","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T06:30:12Z","timestamp":1777617012000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.4997\/jrcpe.2018.204"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,6]]},"references-count":12,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2018,6]]}},"alternative-id":["10.4997\/jrcpe.2018.204"],"URL":"https:\/\/doi.org\/10.4997\/jrcpe.2018.204","relation":{},"ISSN":["1478-2715","2042-8189"],"issn-type":[{"value":"1478-2715","type":"print"},{"value":"2042-8189","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,6]]}}}