Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Immune checkpoint inhibitor-induced anti-striational antibodies in myasthenia gravis and myositis: a case report

Yukio Sugiyama, M.D., Ph.D.1), Yoshiki Esa, M.D.1), Akihiro Watanabe, M.D., Ph.D.1), Junya Kobayashi, M.D.1), Shigeaki Suzuki, M.D., Ph.D.2) and Daisuke Takahashi, M.D.1)

1) Department of Cerebrovascular Medicine, National Hospital Organization Osaka Minami Medical Center
2) Department of Neurology, Keio University School of Medicine

A 78-year-old man was treated with ipilimumab and nivolumab for advanced renal cell carcinoma with liver and lymph node metastasis. He developed diplopia, ptosis, dysphagia, and weakness of the limbs and neck, 1 month after treatment. Serum creatine kinase (CK) levels were elevated, and neck MRI revealed inflammation of the deep trunk muscles. Although anti-acetylcholine receptor antibody was negative, the edrophonium test was positive. Anti-striational antibodies such as the anti-titin and the anti-muscular voltage-gated potassium channel (Kv 1.4) antibodies (which serve as biomarkers of immune checkpoint inhibitors associated with myasthenia gravis and myositis) were positive (anti-titin antibody titer 11.51, normal <1 index; anti-Kv 1.4 antibody titer 15.13, normal <1 index). Intravenous methylprednisolone pulse therapy (1,000 mg/day for 3 days), plasmapheresis, and oral prednisolone (PSL) (20 mg/day) administration improved the patient's neurological function and normalized the serum CK levels. The PSL dosage was tapered without any worsening of clinical signs. The antibody titers decreased but remained positive (anti-titin antibody 5.00, anti-Kv 1.4 antibody 3.83) one year after the initial evaluation. Therefore, low-dose PSL (5 mg/day) administration was continued, and the patient was in remission.
Full Text of this Article in Japanese PDF (931K)

(CLINICA NEUROL, 61: 630−634, 2021)
key words: immune checkpoint inhibitor, immune-related adverse events, myasthenia gravis, myositis, anti-striational antibody

(Received: 12-Feb-21)