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

Ann Thorac Cardiovasc Surg. 2022 Dec 20;28(6):448-452. doi: 10.5761/atcs.cr.21-00052. Epub 2021 Jul 16.

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.

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

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Female
  • Humans
  • Neoplasm Recurrence, Local / complications
  • Stiff-Person Syndrome* / diagnosis
  • Stiff-Person Syndrome* / diagnostic imaging
  • Thymoma* / complications
  • Thymoma* / diagnostic imaging
  • Thymoma* / surgery
  • Thymus Neoplasms* / complications
  • Thymus Neoplasms* / diagnostic imaging
  • Thymus Neoplasms* / surgery
  • Treatment Outcome