Ocular myasthenia: a protean disorder.

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.