{"title":"Thymoma-Related Stiff-Person Syndrome with Successfully Treated by Surgery.","authors":"Akihiro Sasaki, Tatsuya Kato, Hideki Ujiie, Satoru Wakasa, Setsuyuki Otake, Keisuke Kikuchi, Koichi Ohno","doi":"10.5761/atcs.cr.21-00052","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder. Paraneoplastic SPS associated with malignant tumors such as thymoma occurs in approximately 5% of all SPS cases. We present a rare case of thymoma accompanied by SPS successfully treated using surgery.</p><p><strong>Presentation of case: </strong>A 26-year-old woman presented with lower limbs convulsions and gait disturbance and complained of leg pain. Cerebrospinal fluid and blood test results showed a high level of anti-glutamic acid decarboxylase (GAD) antibodies. Computed tomography showed anterior mediastinal tumor suggestive of a thymoma. She underwent extended thymectomy, and her symptoms gradually improved after surgery. No evidence of recurrent thymoma and SPS has been observed over 44 months.</p><p><strong>Conclusion: </strong>Surgical treatment would be effective for patients with SPS and thymoma.</p>","PeriodicalId":8037,"journal":{"name":"Annals of Thoracic and Cardiovascular Surgery","volume":null,"pages":null},"PeriodicalIF":1.1000,"publicationDate":"2022-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6b/77/atcs-28-448.PMC9763711.pdf","citationCount":"3","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Annals of Thoracic and Cardiovascular Surgery","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.5761/atcs.cr.21-00052","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
引用次数: 3
Abstract
Introduction: Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder. Paraneoplastic SPS associated with malignant tumors such as thymoma occurs in approximately 5% of all SPS cases. We present a rare case of thymoma accompanied by SPS successfully treated using surgery.
Presentation of case: A 26-year-old woman presented with lower limbs convulsions and gait disturbance and complained of leg pain. Cerebrospinal fluid and blood test results showed a high level of anti-glutamic acid decarboxylase (GAD) antibodies. Computed tomography showed anterior mediastinal tumor suggestive of a thymoma. She underwent extended thymectomy, and her symptoms gradually improved after surgery. No evidence of recurrent thymoma and SPS has been observed over 44 months.
Conclusion: Surgical treatment would be effective for patients with SPS and thymoma.