OCULAR findings often represent the first, and sometimes the sole manifestation of acquired MG.1,2 Ocular MG, with weakness restricted to the ocular muscles, is generally considered a mild subtype of MG. Nonetheless, the diagnosis and management of purely ocular MG may be particularly challenging and frustrating.3 Diplopia and ptosis, resulting from dysfunction of neuromuscular junctions (NMJs) of extraocular skeletal muscles and levator palpebral muscles in ocular MG, often impair vision sufficiently to interfere with work and quality of life. Whereas ocular symptoms are often the presenting symptoms of MG, they also tend to be the symptoms most refractory to treatment. Response to anticholinesterases is often incomplete,3 and pharmacological suppression of the NMJ immune attack or thymectomy is often required for resolution of symptoms.4-6 The judicious use of corticosteroids represents a key component of management of individual patients with ocular MG. A second consideration is that half the patients with MG, with weakness restricted to ocular muscles clinically, reveal decrement on repetitive nerve stimulation in extremity muscles.7 Furthermore, most patients with purely clinical ocular MG at the onset of disease develop generalized weakness, especially within the first year.2,8,9 This represents a subgroup of patients with ocular MG at risk for rapid deterioration.2,8 Myasthenic crisis is better prevented than treated. In this context, immune treatment is warranted in individual patients with ocular MG and electrophysiological evidence of generalized MG.
Agius MA. Treatment of Ocular Myasthenia With Corticosteroids: Yes. Arch Neurol. 2000;57(5):750–751. doi:10.1001/archneur.57.5.750
Coronavirus Resource Center
Customize your JAMA Network experience by selecting one or more topics from the list below.