Silvestri_2012_Semin.Neurol_32_215

Myasthenia gravis MG is the most common disorder of the neuromuscular junction NMJ with an estimated prevalence between 25 and 142 per million It characteristically presents with fatigable weakness often initially involving the ocular muscles and manifesting as intermittent ptosis and diplopia Ultimately the disease generalizes in two-thirds of patients leading to weakness of bulbar neck limb and respiratory muscles The majority of patients with generalized MG and roughly half of patients with purely ocular disease harbor antibodies to skeletal muscle nicotinic acetylcholine receptors A subset of patients with generalized disease have antibodies to muscle-specific receptor tyrosine kinase MuSK Acetylcholinesterase inhibitors are often the first modality of therapy for MG As an immune-mediated disorder MG can respond to several immunosuppressive agents such as corticosteroids azathioprine mycophenolate mofetil and cyclosporin Thymectomy is a key component of management in appropriately chosen MG patients and those with thymoma Newer or alternative immunotherapies including tacrolimus rituximab methotrexate and complement inhibiting agents are an area of active investigation.