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

Akihiro Sasaki; Tatsuya Kato; Hideki Ujiie; Satoru Wakasa; Setsuyuki Otake; Keisuke Kikuchi; Koichi Ohno

doi:10.5761/atcs.cr.21-00052

Case Reports

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

Akihiro Sasaki, Tatsuya Kato, Hideki Ujiie, Satoru Wakasa, Setsuyuki Otake, Keisuke Kikuchi, Koichi Ohno

Author information

Akihiro Sasaki
Department of Surgery, Obihiro Kosei General Hospital, Obihiro, Hokkaido, Japan
Department of Cardiovascular and Thoracic Surgery, Hokkaido University Faculty and School of Medicine, Sapporo, Hokkaido, Japan
Tatsuya Kato
Department of Cardiovascular and Thoracic Surgery, Hokkaido University Faculty and School of Medicine, Sapporo, Hokkaido, Japan
Hideki Ujiie
Department of Cardiovascular and Thoracic Surgery, Hokkaido University Faculty and School of Medicine, Sapporo, Hokkaido, Japan
Satoru Wakasa
Department of Cardiovascular and Thoracic Surgery, Hokkaido University Faculty and School of Medicine, Sapporo, Hokkaido, Japan
Setsuyuki Otake
Department of Surgery, Obihiro Kosei General Hospital, Obihiro, Hokkaido, Japan
Keisuke Kikuchi
Department of Pathology, Obihiro Kosei General Hospital, Obihiro, Hokkaido, Japan
Koichi Ohno
Department of Surgery, Obihiro Kosei General Hospital, Obihiro, Hokkaido, Japan

Keywords: stiff-person syndrome, paraneoplastic stiff-person syndrome, thymoma, anti-acetylcholine receptor antibody, myasthenia gravis

JOURNAL OPEN ACCESS

2022 Volume 28 Issue 6 Pages 448-452

DOI https://doi.org/10.5761/atcs.cr.21-00052

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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.

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