Lee E K, Maselli R A, Ellis W G, Agius M A
Department of Neurology, University of California, Davis Medical Center, Sacramento, USA.
J Neurol Neurosurg Psychiatry. 1998 Dec;65(6):857-62. doi: 10.1136/jnnp.65.6.857.
Morvan's fibrillary chorea is a rare disease characterised by symptoms which include neuromyotonia, cramping, weakness, pruritus, hyperhidrosis, insomnia, and delirium. The first case of Morvan's fibrillary chorea to be associated with clinical manifestations of myasthenia gravis with thymoma, psoriasis, and atopic dermatitis is reported. Muscle histopathology disclosed chronic denervation and myopathic changes and in vitro electrophysiology demonstrated both presynaptic and postsynaptic defects in neuromuscular transmission. Serum antibodies to acetylcholine receptors, titin, N-type calcium channels, and voltage gated potassium channels were detected. Plasmapheresis, thymectomy, and long term immunosuppression induced a dramatic resolution of symptoms. The association of thymoma with other autoimmune disorders and autoantibodies, and prolonged and sustained remission with chronic immunosuppression, place Morvan's fibrillary chorea on the range of neurological diseases arising as a paraneoplastic complication of cortical thymomas.
莫旺氏纤维性舞蹈病是一种罕见疾病，其症状包括神经肌强直、痉挛、无力、瘙痒、多汗、失眠和谵妄。本文报告了首例与重症肌无力合并胸腺瘤、银屑病和特应性皮炎临床表现相关的莫旺氏纤维性舞蹈病病例。肌肉组织病理学显示慢性去神经支配和肌病性改变，体外电生理学表明神经肌肉传递存在突触前和突触后缺陷。检测到抗乙酰胆碱受体、肌联蛋白、N型钙通道和电压门控钾通道的血清抗体。血浆置换、胸腺切除术和长期免疫抑制使症状显著缓解。胸腺瘤与其他自身免疫性疾病和自身抗体的关联，以及慢性免疫抑制导致的长期持续缓解，将莫旺氏纤维性舞蹈病归入作为皮质胸腺瘤副肿瘤并发症出现的神经系统疾病范畴。
