Weinberg D A, Lesser R L, Vollmer T L
Neuro-Ophthalmology Service, Wills Eye Hospital, Philadelphia, Pennsylvania.
Surv Ophthalmol. 1994 Nov-Dec;39(3):169-210. doi: 10.1016/0039-6257(94)90194-5.
Ocular myasthenia is a localized form of myasthenia clinically involving only the extraocular, levator palpebrae superioris, and/or orbicularis oculi muscles. Ocular manifestations can masquerade as a variety of ocular motility disorders, including cranial nerve and gaze palsies. A history of variable and fatiguable muscle weakness suggests this diagnosis, which may be confirmed by the edrophonium (Tensilon) test and acetylcholine receptor antibody titer. Anticholinesterases, corticosteroids and other immunosuppressive agents, and other therapeutic modalities, including thymectomy and plasmapheresis, are used in treatment. As the pathophysiology of myasthenia has been elucidated in recent years, newer treatment strategies have evolved, resulting in a much more favorable prognosis than several decades ago. This review provides historical background, pathophysiology, immuno-genetics, diagnostic testing, and treatment options for ocular myasthenia, as well as a discussion of drug-induced myasthenic syndromes.
眼肌型重症肌无力是重症肌无力的一种局限性形式，临床上仅累及眼外肌、提上睑肌和/或眼轮匝肌。眼部表现可伪装成多种眼球运动障碍，包括颅神经麻痹和凝视麻痹。可变且易疲劳的肌肉无力病史提示该诊断，可通过依酚氯铵（腾喜龙）试验和乙酰胆碱受体抗体滴度来确诊。抗胆碱酯酶药物、皮质类固醇和其他免疫抑制剂以及其他治疗方式，包括胸腺切除术和血浆置换，都用于治疗。近年来，随着重症肌无力病理生理学的阐明，新的治疗策略不断发展，预后比几十年前要好得多。本文综述了眼肌型重症肌无力的历史背景、病理生理学、免疫遗传学、诊断测试和治疗选择，并讨论了药物性肌无力综合征。
