Myasthenia gravis: past, present, and future

Abstract

Pathogenesis of MG: current state of the artStructure and function of the NMJ. The terminal arborization of α-motor neuron axons from the ventral horns of the spinal cord and brainstem provides the nerve terminals that form the NMJ (Figure 1). These myelinated axons reach the muscles through peripheral nerves; then each axon divides into branches that innervate many individual muscle fibers. As it approaches its target fiber, each branch loses the myelin sheath and further subdivides into many presynaptic boutons, which contain ACh-loaded synaptic vesicles and face the surface of the muscle fiber at the NMJ ( Figure 1). The synaptic bouton and the muscle surface are separated by the synaptic cleft, a 20 nm-thick space that contains acetylcholinesterase (AChE) and other proteins and proteoglycans involved in

Refractory myasthenia gravis, dysphagia and malnutrition: A case report to suggest disease-specific nutritional issues

“…Myesthenia grave is a rare (prevalence 1-2 in 5000) autoimmune disease of the neuromuscular junction due to the presence of acetylcholine receptor-specific or musclespecific tyrosine kinase autoantibodies, which translates to the typical weakness and fatigue [1][2][3].…”

“…Her clinical history included Graves-Basedow disease, uterine fibromyoma causing chronic anemia, anxious-depressive syndrome, and other different neoplastic diseases (squamous cell carcinoma of the soft palate, left-foot melanoma, vocal chord polypus) that were treated with a surgical procedure. MG (presence of acetylcholine receptor-specific autoantibodies and a primary postsynaptic neuromuscular junctional disorder by electrophysiologic testing) [1][2][3] was first diagnosed in August 2005. Computed tomogram of the chest revealed the presence of mediastinal thymic residuals.…”

CCL2 recruitment of IL‐6‐producing CD11b+ monocytes to the draining lymph nodes during the initiation of Th17‐dependent B cell‐mediated autoimmunity

“…Here we tested the possibility that CCL2-mediated homing of monocytes may affect Th17 cells in a mouse model of human myasthenia gravis (MG), the experimental autoimmune MG (EAMG) model. In MG and EAMG, the cardinal sign of weakness is mediated by circulating IgG autoantibodies to the acetylcholine receptor (AChR) or to other muscular antigens at the neuromuscular junction [14,15].…”

Autoantibody to dihydropyridine receptor in myasthenia gravis

“…Myasthenia gravis (MG) is an autoimmune disease with impaired neuromuscular transmission mainly due to the effect of autoantibodies to acetylcholine receptor (AChR) (Conti-Fine et al, 2006). Recently, dysregulation of sarcoplasmic reticulum (SR) Ca 2+ relaease has been associated with muscle fatigue (Bellinger et al, 2008).…”