title: Rituximab in severe seronegative juvenile myasthenia gravis: review of the literature.
abstract: Myasthenia gravis is an autoimmune neuromuscular disorder caused by circulating antibodies that block acetylcholine receptors at the postsynaptic neuromuscular junction. A wide range of symptomatic therapy with acetylcholinesterase inhibitors and immunotherapy such as corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, plasmapheresis, and intravenous immunoglobulin has been used in the treatment of myasthenia gravis, with variable responses. However, most modalities of treatment involve delayed onset of action. We describe a child with severe, life-threatening seronegative myasthenia gravis who repeatedly failed extubation and responded dramatically to rituximab. She achieved complete and sustained remission for more than 9 months, with gradual reduction in steroid dose without any side effects. Advances in the treatment of myasthenia gravis have reduced mortality and morbidity and improved the quality of life in these patients.
authors.affiliation: Department of Child Health, Sultan Qaboos University Hospital, Muscat, Oman.
authors.forename: Roshan
authors.initials: R
authors.lastname: Koul
authors.forename: Amna
authors.initials: A
authors.lastname: Al Futaisi
authors.forename: Reem
authors.initials: R
authors.lastname: Abdwani
mesh_terms: D060666:Airway Extubation; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000009:adverse effects / Q000627:therapeutic use*; D018941:Antigens, CD19 / Q000032:analysis; D018501:Antirheumatic Agents / Q000009:adverse effects / Q000627:therapeutic use*; D001763:Blepharoptosis / Q000209:etiology; D002675:Child, Preschool; D004172:Diplopia / Q000209:etiology; D005260:Female; D006801:Humans; D018655:Lymphocyte Count; D009157:Myasthenia Gravis / Q000188:drug therapy*; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use; D012121:Respiration, Artificial; D000069283:Rituximab
chemical_list: D000893:Anti-Inflammatory Agents; D058846:Antibodies, Monoclonal, Murine-Derived; D018941:Antigens, CD19; D018501:Antirheumatic Agents; D000069283:Rituximab; D011241:Prednisone