Clinical Neurophyisology, Somerset NHS Foundation Trust, Taunton, UK.
Neurology, Kettering General Hospital NHS Foundation Trust, Kettering, UK.
BMJ Case Rep. 2023 Nov 17;16(11):e256407. doi: 10.1136/bcr-2023-256407.
A man in his mid-60s presented with a 3-month history of progressive muscle twitching, agitation, cognitive impairment, insomnia, hyperhidrosis and lower limb pain. He had fasciculations, myokymia, myoclonus, exaggerated startle response and significant postural hypotension. Electrophysiological studies showed evidence of peripheral nerve hyperexcitability with neuromyotonia. Contactin-associated protein-like 2 antibodies (CASPR2) were strongly positive. A diagnosis of Morvan syndrome was made. CT of the chest, abdomen and pelvis was undertaken to identify any occult malignancy, and a large bowel carcinoma in situ was identified and resected. His central nervous system and autonomic symptoms significantly improved following surgery, but neuromyotonia persisted, and this was treated with intravenous immunoglobulins and steroids. Early detection of bowel cancer in this patient enabled curative treatment.
一位 60 多岁的男性,出现进行性肌肉抽搐、激越、认知障碍、失眠、多汗和下肢疼痛 3 个月。他有肌束颤动、肌纤维震颤、肌阵挛、惊跳反应过度和明显的体位性低血压。电生理研究显示有周围神经兴奋性过高和神经肌强直的证据。接触蛋白相关蛋白样 2 抗体(CASPR2)强阳性。诊断为莫旺综合征。进行了胸部、腹部和骨盆 CT 检查以确定任何隐匿性恶性肿瘤,发现并切除了一处大肠原位癌。手术治疗后,他的中枢神经系统和自主神经症状显著改善,但神经肌强直持续存在,使用静脉注射免疫球蛋白和类固醇治疗。该患者的结直肠癌早期发现使治疗成为可能。