Juvenile Myasthenia Gravis: A Short Review

Volume 2 - Issue 1

Shubhankar Mishra*

Received: November 26, 2018;   Published: November 29, 2018

DOI: 10.32474/PAPN.2018.02.000127

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Abstract

Juvenile myasthenia gravis (JMG) is an autoimmune disorder of neuromuscular transmission caused by production of antibodies against components of the postsynaptic membrane of the neuromuscular junction. The patients present with a wide range of symptoms-from isolated intermittent ocular symptoms to general muscle weakness with or without respiratory insufficiency. Prepubertal children in particular have a higher prevalence of isolated ocular symptoms, lower frequency of acetylcholine receptor antibodies, and a higher probability of achieving remission. It must be differentiated from congenital myasthenia which is a channelopathy rather than autoimmune disease. Treatment commonly includes anticholinesterases, corticosteroids with or without steroid-sparing agents, and newer immune modulating agents. Plasma exchange and intravenous immunoglobulin (IVIG) are effective in preparation for surgery and in treatment of myasthenic crisis.

Keywords: Myasthenia; Ptosis; Pyridostigmine

Abbrevations: JMG: Juvenile Myasthenia Gravis; IVIG: Intravenous Immunoglobulin; MG: Myasthenia Gravis; Ab: Antibodies; AChR: Acetylcholine Receptor; MuSK: Muscle-Specific Kinase; LRP4: Leucine Rich Protein 4; RNS: Repetitive Stimulation Test; SFEMG: Single Fiber Electromyography; CT: Computed Tomography; MRI: Magnetic Resonance Imaging

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