Rituximab in severe seronegative juvenile myasthenia gravis

Review of the literature

Research output: Contribution to journalArticle

11 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)209-212
Number of pages4
JournalPediatric Neurology
Volume47
Issue number3
DOIs
Publication statusPublished - Sep 2012

Fingerprint

Myasthenia Gravis
Mycophenolic Acid
Plasmapheresis
Neuromuscular Junction
Intravenous Immunoglobulins
Cholinesterase Inhibitors
Azathioprine
Cholinergic Receptors
Therapeutics
Immunotherapy
Cyclosporine
Adrenal Cortex Hormones
Steroids
Quality of Life
Morbidity
Mortality
Rituximab
Antibodies

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Neurology

Cite this

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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.",
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