Myasthénie pédiatrique
Posté : 20 oct. 2017 08:05
Lu sur :https://www.ncbi.nlm.nih.gov/pubmed/28941526[ligne][/ligne]
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Pediatric Myasthenia Gravis.
Peragallo JH1.
Semin Pediatr Neurol. 2017 May;24(2):116-121. doi: 10.1016/j.spen.2017.04.003. Epub 2017 Apr 7.
Abstract
Myasthenia gravis is a disorder of neuromuscular transmission that leads to fatigue of skeletal muscles and fluctuating weakness. Myasthenia that affects children can be classified into the following 3 forms: transient neonatal myasthenia, congenital myasthenic syndromes, and juvenile myasthenia gravis (JMG).
JMG is an autoimmune disorder that has a tendency to affect the extraocular muscles, but can also affect all skeletal muscles leading to generalized weakness and fatigability.
Respiratory muscles may be involved leading to respiratory failure requiring ventilator support. Diagnosis should be suspected clinically, and confirmatory diagnostic testing be performed, including serum acetylcholine receptor antibodies, repetitive nerve stimulation, and electromyography. Treatment for JMG includes acetylcholinesterase inhibitors, immunosuppressive medications, plasma exchange, intravenous immunoglobulins, and thymectomy.
Children with myasthenia gravis require monitoring by a pediatric ophthalmologist for the development of amblyopia from ptosis or strabismus.
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