Nonaka Takayuki, Ebisu Ikumi, Ohashi Ikkei, Shizukawa Hirohiko, Shimohama Shun
Department of Neurology, Sapporo-Kosei General Hospital.
Department of Neurology, Sapporo Medical University School of Medicine.
Rinsho Shinkeigaku. 2022 Aug 27;62(8):615-620. doi: 10.5692/clinicalneurol.cn-001729. Epub 2022 Jul 22.
A 51-year-old Japanese man presenting with a several-month history of parasomnia, orthostatic hypotension and generalized myokymia was admitted to our hospital. He had a past medical history of unresectable recurrent thymoma, but chemotherapy for thymoma was discontinued according to the patient's decision four years before this hospitalization, and the thymoma had enlarged. He exhibited symptoms of the peripheral nervous system (myokymia), central nervous system (parasomnia, short-term memory impairment), and autonomic nervous system (orthostatic hypotension), and his serum was positive for voltage-gated potassium channel (VGKC)-complex antibodies. Based on the above findings, Morvan syndrome was diagnosed. Resumption of chemotherapy for thymoma resulted in shrinkage of the thymoma accompanied by remission of Morvan syndrome. Subsequently, discontinuation of chemotherapy led to aggravation of thymoma with recurrence of Morvan syndrome. This clinical course suggests a strong correlation between the disease activity of thymoma and Morvan syndrome. In the present case of Morvan syndrome associated with unresectable thymoma, chemotherapy contributed to the remission of Morvan syndrome. Our patient suggests a possibility that chemotherapy for thymoma is a useful treatment for Morvan syndrome.
一名51岁的日本男性因存在数月的异态睡眠、直立性低血压和全身性肌束震颤病史而入住我院。他既往有不可切除的复发性胸腺瘤病史，但在此次住院前四年，根据患者的决定停止了胸腺瘤化疗，此后胸腺瘤增大。他出现了周围神经系统症状（肌束震颤）、中枢神经系统症状（异态睡眠、短期记忆障碍）和自主神经系统症状（直立性低血压），其血清电压门控钾通道（VGKC）复合物抗体呈阳性。基于上述发现，诊断为莫旺综合征。恢复胸腺瘤化疗后，胸腺瘤缩小，同时莫旺综合征缓解。随后，停止化疗导致胸腺瘤加重，莫旺综合征复发。这一临床过程提示胸腺瘤的疾病活动与莫旺综合征之间存在密切关联。在本例与不可切除胸腺瘤相关的莫旺综合征中，化疗有助于莫旺综合征的缓解。我们的患者提示胸腺瘤化疗可能是治疗莫旺综合征的一种有效方法。
