Sugiyama Yukio, Esa Yoshiki, Watanabe Akihiro, Kobayashi Junya, Suzuki Shigeaki, Takahashi Daisuke
Department of Cerebrovascular Medicine, National Hospital Organization Osaka Minami Medical Center.
Department of Neurology, Keio University School of Medicine.
Rinsho Shinkeigaku. 2021 Sep 28;61(9):630-634. doi: 10.5692/clinicalneurol.cn-001604. Epub 2021 Aug 26.
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.
一名78岁男性因晚期肾细胞癌伴肝和淋巴结转移接受了伊匹单抗和纳武单抗治疗。治疗1个月后,他出现了复视、上睑下垂、吞咽困难以及四肢和颈部无力。血清肌酸激酶(CK)水平升高,颈部MRI显示深部躯干肌肉炎症。尽管抗乙酰胆碱受体抗体为阴性,但依酚氯铵试验呈阳性。抗肌动蛋白抗体如抗肌联蛋白抗体和抗肌肉电压门控钾通道(Kv 1.4)抗体(作为与重症肌无力和肌炎相关的免疫检查点抑制剂的生物标志物)呈阳性(抗肌联蛋白抗体滴度11.51,正常<1指数;抗Kv 1.4抗体滴度15.13,正常<1指数)。静脉注射甲泼尼龙冲击疗法(1000mg/天,共3天)、血浆置换和口服泼尼松龙(PSL)(20mg/天)改善了患者的神经功能,并使血清CK水平恢复正常。PSL剂量逐渐减少,临床症状未恶化。初次评估1年后,抗体滴度下降但仍为阳性(抗肌联蛋白抗体5.00,抗Kv 1.4抗体3.83)。因此,继续给予低剂量PSL(5mg/天),患者病情缓解。