{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,27]],"date-time":"2025-07-27T07:46:19Z","timestamp":1753602379928,"version":"3.37.3"},"reference-count":9,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2022,4,25]],"date-time":"2022-04-25T00:00:00Z","timestamp":1650844800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,4,25]],"date-time":"2022-04-25T00:00:00Z","timestamp":1650844800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Neurol"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>In this case, we reported the pseudobulbar affect (PBA) in a patient with Susac\u2019s syndrome\u2014a rare condition that was caused by a rare syndrome. Previous case reports of Susac syndrome described psychiatric symptoms such as emotional disturbances or personality changes. Only a few case reports have reported psychiatric disorders in patients with Susac\u2019s syndrome. There were no reported cases of Susac syndrome with PBA as an initial presentation.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>Our patient was 56\u2009years old and presented with involuntary crying, left-sided headache, left-sided hearing loss, and tinnitus. Brain MRI showed numerous areas of restricted diffusion and enhancement involving the corpus callosum, bilateral hemispheres, and brainstem. Ophthalmological evaluation showed bilateral branch retinal artery occlusion. She was diagnosed with Susac\u2019s syndrome and PBA. She was treated with cyclophosphamide and dextromethorphan hydrobromide\/quinidine sulfate with excellent recovery. This is a 2-year clinical course.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Discussion and conclusions<\/jats:title>\n                <jats:p>Recognition of the clinical presentation of Susac\u2019s syndrome and PBA with early diagnosis and treatment are the keys to preventing further disability and impact on patients and their families.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12883-022-02639-9","type":"journal-article","created":{"date-parts":[[2022,4,25]],"date-time":"2022-04-25T11:02:58Z","timestamp":1650884578000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Involuntary crying episodes with Susac\u2019s syndrome\u2014a rare presentation of a rare disease: a case report"],"prefix":"10.1186","volume":"22","author":[{"given":"O.","family":"Alshaqi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Moodie","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4129-2455","authenticated-orcid":false,"given":"A.","family":"Alchaki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,4,25]]},"reference":[{"issue":"19 Pt 2","key":"2639_CR1","doi-asserted-by":"publisher","first-page":"1867","DOI":"10.1212\/01.wnl.0000280580.95671.01","volume":"70","author":"I Kleffner","year":"2008","unstructured":"Kleffner I, Deppe M, Mohammadi S, Schiffbauer H, Stupp N, Lohmann H, et al. Diffusion tensor imaging demonstrates fiber impairment in Susac syndrome. Neurology. 2008;70(19 Pt 2):1867\u20139. https:\/\/doi.org\/10.1212\/01.wnl.0000280580.95671.01 Epub 2007 Oct 24. PMID: 17959768.","journal-title":"Neurology"},{"issue":"6","key":"2639_CR2","doi-asserted-by":"publisher","first-page":"307","DOI":"10.1038\/nrneurol.2013.82","volume":"9","author":"J D\u00f6rr","year":"2013","unstructured":"D\u00f6rr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M, Duning T, et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013;9(6):307\u201316. https:\/\/doi.org\/10.1038\/nrneurol.2013.82 Epub 2013 Apr 30. PMID: 23628737.","journal-title":"Nat Rev Neurol"},{"issue":"2","key":"2639_CR3","doi-asserted-by":"publisher","first-page":"114","DOI":"10.1111\/j.1600-0404.1989.tb03851.x","volume":"80","author":"JP Gallagher","year":"1989","unstructured":"Gallagher JP. Pathologic laughter and crying in ALS: a search for their origin. Acta Neurol Scand. 1989;80(2):114\u20137. https:\/\/doi.org\/10.1111\/j.1600-0404.1989.tb03851.x PMID: 2816272.","journal-title":"Acta Neurol Scand"},{"key":"2639_CR4","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1186\/1742-2094-11-46","volume":"11","author":"S Jarius","year":"2014","unstructured":"Jarius S, Kleffner I, D\u00f6rr JM, et al. Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study. J Neuroinflammation. 2014;11:46. https:\/\/doi.org\/10.1186\/1742-2094-11-46.","journal-title":"J Neuroinflammation"},{"key":"2639_CR5","doi-asserted-by":"publisher","first-page":"5779","DOI":"10.1038\/s41467-019-13593-5","volume":"10","author":"CC Gross","year":"2019","unstructured":"Gross CC, Meyer C, Bhatia U, et al. CD8+ T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome. Nat Commun. 2019;10:5779. https:\/\/doi.org\/10.1038\/s41467-019-13593-5.","journal-title":"Nat Commun"},{"issue":"4\u20135","key":"2639_CR6","doi-asserted-by":"publisher","first-page":"347","DOI":"10.1016\/j.autrev.2014.01.038","volume":"13","author":"M Garc\u00eda-Carrasco","year":"2014","unstructured":"Garc\u00eda-Carrasco M, Mendoza-Pinto C, Cervera R. Diagnosis and classification of Susac syndrome. Autoimmun Rev. 2014;13(4\u20135):347\u201350. https:\/\/doi.org\/10.1016\/j.autrev.2014.01.038 Epub 2014 Jan 11. PMID: 24424186.","journal-title":"Autoimmun Rev"},{"issue":"3","key":"2639_CR7","doi-asserted-by":"publisher","first-page":"688","DOI":"10.1016\/j.jstrokecerebrovasdis.2015.11.038","volume":"25","author":"DC Gillespie","year":"2016","unstructured":"Gillespie DC, Cadden AP, Lees R, West RM, Broomfield NM. Prevalence of Pseudobulbar affect following stroke: a systematic review and Meta-analysis. J Stroke Cerebrovasc Dis. 2016;25(3):688\u201394. https:\/\/doi.org\/10.1016\/j.jstrokecerebrovasdis.2015.11.038.","journal-title":"J Stroke Cerebrovasc Dis"},{"key":"2639_CR8","doi-asserted-by":"publisher","first-page":"243","DOI":"10.1016\/B978-0-444-64012-3.00014-9","volume":"165","author":"L Nguyen","year":"2019","unstructured":"Nguyen L, Matsumoto RR. The psychopharmacology of pseudobulbar affect. Handb Clin Neurol. 2019;165:243\u201351. https:\/\/doi.org\/10.1016\/B978-0-444-64012-3.00014-9 PMID: 31727215.","journal-title":"Handb Clin Neurol"},{"key":"2639_CR9","doi-asserted-by":"publisher","first-page":"23","DOI":"10.2147\/DNND.S34160","volume":"3","author":"RR King","year":"2013","unstructured":"King RR, Reiss J. The epidemiology and pathophysiology of pseudobulbar affect and its association with neurodegeneration. Degener Neurol Neuromuscul Dis. 2013;3:23\u201331. https:\/\/doi.org\/10.2147\/DNND.S34160.","journal-title":"Degener Neurol Neuromuscul Dis"}],"container-title":["BMC Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12883-022-02639-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12883-022-02639-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12883-022-02639-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,25]],"date-time":"2022-04-25T11:05:12Z","timestamp":1650884712000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcneurol.biomedcentral.com\/articles\/10.1186\/s12883-022-02639-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,25]]},"references-count":9,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2022,12]]}},"alternative-id":["2639"],"URL":"https:\/\/doi.org\/10.1186\/s12883-022-02639-9","relation":{},"ISSN":["1471-2377"],"issn-type":[{"type":"electronic","value":"1471-2377"}],"subject":[],"published":{"date-parts":[[2022,4,25]]},"assertion":[{"value":"30 June 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 March 2022","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 April 2022","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The case report is approved by USD\/Sanford health ethics committee. A case report is waived.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"All authors have nothing to declare. No financial or non-financial competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"155"}}