Papazian O
Department of Neurology, Miami Children's Hospital, FL 33155.
J Child Neurol. 1992 Apr;7(2):135-41. doi: 10.1177/088307389200700202.
Transient neonatal myasthenia gravis is a postsynaptic neuromuscular transmission defect occurring in 21% of infants born to women with active (and, less commonly, in remission) acquired myasthenia gravis. Although passive-transfer acetylcholine receptor (AChR) antibodies are found in the majority of these newborns, their pathogenic role is questionable because only some infants are symptomatic. Pathogenesis in infants without AChR antibodies is unknown. There is still no biologic marker for prenatal identification of this subpopulation of newborns, although HLA typing may be a promising tool. Sucking, swallowing, and respiratory difficulties are the most common presenting signs in the first day of life. Final diagnosis is done when administration of acetylcholinesterase agents transiently corrects the neuromuscular transmission defect. Serum AChR antibody titers follow the same pattern as their mothers. Supportive management and anticholinesterase agents prior to feedings are necessary in about 80% of patients. In the majority of infants the condition resolves spontaneously.
短暂性新生儿重症肌无力是一种突触后神经肌肉传递缺陷，发生于患活动性（较少见的是缓解期）获得性重症肌无力母亲所生婴儿的21%。虽然在大多数此类新生儿中发现了被动转移的乙酰胆碱受体（AChR）抗体，但它们的致病作用存在疑问，因为只有部分婴儿出现症状。无AChR抗体婴儿的发病机制尚不清楚。虽然人类白细胞抗原（HLA）分型可能是一种有前景的工具，但目前仍没有用于产前识别这一亚群新生儿的生物学标志物。吸吮、吞咽和呼吸困难是出生第一天最常见的表现体征。当给予乙酰胆碱酯酶药物能短暂纠正神经肌肉传递缺陷时即可做出最终诊断。血清AChR抗体滴度与其母亲的情况一致。约80%的患者在喂奶前需要支持性治疗和使用抗胆碱酯酶药物。大多数婴儿的病情会自发缓解。
