Yuki Natsuko, Yoshioka Akira, Isayama Reina, Koizumi Hidetaka, Nakagawa Masanori
Department of Neurology, Maizuru Medical Center, Maizuru.
Intern Med. 2008;47(14):1355-7. doi: 10.2169/internalmedicine.47.0965. Epub 2008 Jul 15.
A 56-year-old man with anti-acetylcholine receptor antibody-mediated myasthenia gravis had bilateral facial muscular atrophy and had noticed blepharoptosis 15 years earlier. From 45 to 51 years of age, 5-10 mg prednisolone and 180 mg pyridostigmine daily relieved his symptoms. Subsequently, these treatments no longer improved the facial weakness, though blepharoptosis was absent. At 56 years of age, the edrophonium test and repetitive supramaximal stimulation testing of the orbicularis oris were negative. Frontalis muscle needle electromyography showed low amplitude polyphasic units and an incomplete interference pattern. Facial muscle atrophy, caused by disuse atrophy from neuromuscular junction depletion, contributed to this patient's facial weakness.
一名56岁的男性，患有抗乙酰胆碱受体抗体介导的重症肌无力，出现双侧面部肌肉萎缩，15年前就已注意到上睑下垂。在45至51岁期间，每日服用5 - 10毫克泼尼松龙和180毫克吡啶斯的明可缓解其症状。随后，尽管上睑下垂已不存在，但这些治疗不再能改善面部无力的症状。56岁时，依酚氯铵试验和口轮匝肌重复超强刺激试验均为阴性。额肌针极肌电图显示多相波单位波幅低且干扰型不完全。由神经肌肉接头耗竭导致的废用性萎缩引起的面部肌肉萎缩，是该患者面部无力的原因。
