Thymoma-Related Stiff-Person Syndrome with Successfully Treated by Surgery

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...

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Detalles Bibliográficos
Autores principales: Sasaki, Akihiro, Kato, Tatsuya, Ujiie, Hideki, Wakasa, Satoru, Otake, Setsuyuki, Kikuchi, Keisuke, Ohno, Koichi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Editorial Committee of Annals of Thoracic and Cardiovascular Surgery 2021
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9763711/
https://www.ncbi.nlm.nih.gov/pubmed/34275989
http://dx.doi.org/10.5761/atcs.cr.21-00052
Descripción
Sumario: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.

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