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小儿重症肌无力

Pediatric Myasthenia Gravis.

作者信息

Peragallo Jason H

机构信息

Department of Ophthalmology, Emory University School of Medicine, Atlanta, GA; Department of Pediatrics, Emory University School of Medicine, Atlanta, GA.

出版信息

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.

摘要

重症肌无力是一种神经肌肉传递障碍性疾病,可导致骨骼肌疲劳和肌无力波动。影响儿童的重症肌无力可分为以下3种形式:短暂性新生儿重症肌无力、先天性重症肌无力综合征和青少年型重症肌无力(JMG)。JMG是一种自身免疫性疾病,倾向于累及眼外肌,但也可累及所有骨骼肌,导致全身无力和易疲劳。呼吸肌可能受累,导致呼吸衰竭,需要呼吸机支持。临床应怀疑诊断,并进行确诊性诊断检查,包括血清乙酰胆碱受体抗体、重复神经电刺激和肌电图检查。JMG的治疗包括乙酰胆碱酯酶抑制剂、免疫抑制药物、血浆置换、静脉注射免疫球蛋白和胸腺切除术。重症肌无力患儿需要由小儿眼科医生监测是否因上睑下垂或斜视而发生弱视。

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