Thymoma-associated Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM) with Myasthenia Gravis.

Thymoma-associated Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM) with Myasthenia Gravis.
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DOI:
10.2169/internalmedicine.56.7979
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发表时间:
2017
期刊:
Internal medicine (Tokyo, Japan)
影响因子:
--
通讯作者:
Kusaka H
Kusaka H
中科院分区:
其他
文献类型:
--
作者:
Morise S;Nakamura M;Morita JI;Miyake K;Kunieda T;Kaneko S;Kusaka H

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我们报告一例72岁的妇女谁最初提出的症状球肌无力和阳性抗乙酰胆碱受体抗体。她随后出现疼痛性肌肉痉挛、肌阵挛和僵硬。检查发现胸腺瘤,并发现抗甘氨酸受体和抗谷氨酸脱羧酶抗体。她被诊断为胸腺瘤相关的进行性脑脊髓炎伴强直和肌阵挛(PERM)。她经历了明显改善后,胸腺切除术后,血浆置换和静脉注射免疫球蛋白和泼尼松龙。本病例提示,胸腺切除术后充分的免疫抑制可能有助于治疗胸腺瘤相关的PERM。胸腺瘤相关的PERM患者可能发生重症肌无力。
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.
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