Morise Satoshi, Nakamura Masataka, Morita Jun-Ichi, Miyake Kousuke, Kunieda Takenobu, Kaneko Satoshi, Kusaka Hirofumi
Department of Neurology, Kansai Medical University, Japan.
Department of Neurology, Komatsu Hospital, Japan.
Intern Med. 2017;56(13):1733-1737. doi: 10.2169/internalmedicine.56.7979. Epub 2017 Jul 1.
We report a case of a 72-year-old woman who initially presented with symptoms of bulbar myasthenia and was positive for anti-acetylcholine receptor antibodies. She subsequently developed painful muscle spasms, myoclonus, and stiffness. Thymoma was detected, and both anti-glycine receptor and anti-glutamic acid decarboxylase antibodies were found. She was diagnosed with thymoma-associated progressive encephalomyelitis with rigidity and myoclonus (PERM). She experienced marked improvement after thymectomy followed by plasma exchange and intravenous immunoglobulin and prednisolone. This case suggests that thymectomy followed by sufficient immunosuppression may be useful in the treatment of thymoma-associated PERM. Myasthenia gravis may develop in thymoma-associated PERM patients.
我们报告一例72岁女性，最初表现为延髓型重症肌无力症状，抗乙酰胆碱受体抗体检测呈阳性。随后她出现了疼痛性肌肉痉挛、肌阵挛和僵硬。检测到胸腺瘤，同时发现了抗甘氨酸受体抗体和抗谷氨酸脱羧酶抗体。她被诊断为胸腺瘤相关的伴有僵硬和肌阵挛的进行性脑脊髓炎（PERM）。在胸腺切除术后，接着进行血浆置换、静脉注射免疫球蛋白和泼尼松龙治疗后，她病情显著改善。该病例表明，胸腺切除术后进行充分的免疫抑制治疗可能对胸腺瘤相关的PERM治疗有效。胸腺瘤相关的PERM患者可能会发生重症肌无力。
