Juvenile Myasthenia Gravis: A Short Review
Journal Title: Progressing Aspects in Pediatrics and Neonatology - Year 2018, Vol 2, Issue 1
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.The first description of myasthenia gravis (MG) with onset in childhood originates from Erb in 1879 [1]. The definition of juvenile myasthenia gravis (JMG) includes infants, children, and adolescents aged 0 to 19 years [2]. JMG patients are subdivided according to the occurrence of the first symptoms as prepubertal (first symptoms before the age of 12 years) and postpubertal (first symptoms after the age of 12 years) [3]. The main action of disease is production of antibodies (Ab) against the components of postsynaptic membrane, predominantly the binding acetylcholine receptor (AChR). MG is divided into five groups: ocular myasthenia (grade I), mild (grade IIa), moderate (grade IIb), severe weakness other than ocular (grade III), and very severe weakness defined by intubation with or without mechanical ventilation (grade IV) [4]. It is a rare disorder of the childhood which accounts for less than 10- 15% with an incidence of 1-5 per million per year.
Authors and Affiliations
Shubhankar Mishra
Keywords
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- EP ID EP583268
- DOI 10.32474/PAPN.2018.02.000127
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