ABSTRACT
Myasthenia gravis (MG) is a rare, progressive autoimmune disorder that causes muscle weakness, fatigue, and locomotor dysfunction when antibodies are produced against the acetylcholine receptor (AChR), muscle-specific kinase (MuSK), lipoprotein-related protein 4 (LRP4), or agrin; all this takes place in the postsynaptic membrane located at the neuromuscular junction. Clinical manifestations of MG affect bulbar muscles, as well as limb musculature, in about two-thirds of patients. Until now, the exact etiology of MG has not been clear; however, it is known that the presence of antibodies plays a pivotal role against the nicotinic acetylcholine receptor (nAChR) in its pathological process. In about 85% of patients, IgG antibodies are found against postsynaptic nAChR. The therapeutic paradigm for this disease is based on a supportive, immunosuppressive, symptomatic approach. As of now, various conventional drugs are available to take care of this situation, but these drugs are associated with serious side effects. Hence, recent focus has shifted toward target-based therapy that could offer a safe and effective treatment option. Thus, in this chapter, we discuss the key aspects of molecular signaling in the pathogenesis, as well as various novel therapeutic options and future perspectives in the management of MG. Moreover, an insight into the various pathways involved in the initial triggering of MG will help in better understanding the disease progression.
