Acquired myasthenia gravis in childhood
Acquired myasthenia gravis in childhood
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DOI：
10.1097/wco.0b013e32833c32af
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发表时间：
2010-10-01
影响因子：
4.8
通讯作者：
Evoli, Amelia
中科院分区：
文献类型：
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作者：
Evoli, Amelia
综述近年来有关儿童起病的重症肌无力(青少年重症肌无力)和新生儿重症肌无力的研究。在大多数患者中，青少年重症肌无力与乙酰胆碱受体(AChR)抗体有关。胸腺瘤很少见，但在儿童中通常是恶性的。不同国家的青少年重症肌无力伴肌肉特异性激酶(穆斯克)抗体的频率有显著差异；已描述了一些明显的特征。总的来说，青少年重症肌无力的治疗与成人重症肌无力的治疗没有什么不同。儿童胸腺切除的时机仍然存在争议。母体抗胎儿型AChR抗体可导致子代持续性局灶性无力，而与麝香抗体相关的新生儿重症肌无力通常是一种严重的、长期的、尽管是短暂的疾病。临床表现受抗体状况、种族和发病年龄的影响。治疗非常有效，但需要指导方针和对照试验。新生儿重症肌无力的风险明显受母体抗体亚类和抗原特异性的影响。母亲的适当治疗可以减少新生儿疾病的频率和严重程度。
Purpose of reviewThis review discusses recent studies on myasthenia gravis with onset in childhood (juvenile myasthenia gravis) and neonatal myasthenia gravis.Recent findingsThe occurrence of myasthenia gravis in childhood is strongly influenced by genetic and environmental factors. Juvenile myasthenia gravis is associated with antibodies to the acetylcholine receptor (AChR) in most patients. Thymoma is rare, but often malignant in children. The frequency of juvenile myasthenia gravis with antibodies to the muscle-specific kinase (MuSK) varies markedly in different countries; some distinct features have been described. Management of juvenile myasthenia gravis does not differ, on the whole, from that of adult myasthenia gravis. Timing of thymectomy in young children is still controversial. Maternal antifetal type AChR antibodies can cause persistent focal weakness in the offspring, while neonatal myasthenia gravis associated with MuSK antibodies is often a severe and protracted albeit transient disease.SummaryJuvenile myasthenia gravis, like its adult-onset counterpart, is a heterogeneous disease. Clinical presentation is influenced by antibody status, ethnicity and age of onset. Treatment is very effective, but guidelines and controlled trials are needed. The risk for neonatal myasthenia gravis appears to be markedly influenced by maternal antibody subclass and antigen specificity. Adequate treatment in mothers can reduce both frequency and severity of neonatal disease.