Santiago Gonçalves Catarina, Nunes Marques M Ines, Antunes Sónia, Serrano Ana
Pediatrics, Hospital do Espírito Santo de Évora, Évora, PRT.
Cureus. 2021 Dec 22;13(12):e20592. doi: 10.7759/cureus.20592. eCollection 2021 Dec.
Myasthenia gravis (MG) in the neonate is usually due to placentally transferred antibodies to the acetylcholine receptor (AChR), resulting in impaired neuromuscular transmission. It occurs in 10%-15% of newborns born to women with MG. We present a male newborn admitted to the neonatal intensive care unit (NICU) 38 hours after birth due to feeding difficulties and choking episodes. He was born to a mother with MG after an uneventful, well-followed pregnancy. Physical examination revealed a weak cry, persistent inability to fully close his eyelids, weak facial mimic, and a mouth that was always held open with swallowing and sucking difficulties. He assumed a frog leg position and showed generalized hypotonia with marked head lag. No respiratory distress was present. Laboratory evaluation showed an elevated anti-acetylcholine receptor antibody concentration (36.30 nmol/L; normal: <0.25 nmol/L). Transient neonatal myasthenia gravis (TNMG) was admitted, and an anticholinesterase agent was initiated. Given that he showed only a mild clinical improvement, two doses of immunoglobulin were administered on the eighth and ninth days of life. Anticholinesterase agents were progressively reduced and suspended on day 31 of life with clinical improvement. He was discharged home at one month of life clinically asymptomatic. He was evaluated one month later and was doing well. A positive history of MG in the mother associated with a suggestive physical examination may be sufficient to make the diagnosis of transient neonatal MG, emphasizing the importance of good medical history. With prompt diagnosis and appropriate management, most newborns experience spontaneous remission after a period of weeks to months.
新生儿重症肌无力(MG)通常是由于胎盘传递的抗乙酰胆碱受体(AChR)抗体导致神经肌肉传递受损。它发生在患有MG的女性所生的10%-15%的新生儿中。我们报告一例男性新生儿,出生后38小时因喂养困难和呛咳发作入住新生儿重症监护病房(NICU)。他的母亲患有MG,孕期顺利且产检良好。体格检查发现哭声微弱、持续无法完全闭合眼睑、面部表情肌无力,嘴巴总是张开,伴有吞咽和吸吮困难。他呈蛙腿姿势,表现为全身肌张力低下,头部明显滞后。无呼吸窘迫。实验室检查显示抗乙酰胆碱受体抗体浓度升高(36.30 nmol/L;正常:<0.25 nmol/L)。诊断为短暂性新生儿重症肌无力(TNMG),并开始使用抗胆碱酯酶药物。鉴于他仅表现出轻微的临床改善,在出生后第八天和第九天给予了两剂免疫球蛋白。随着临床改善,抗胆碱酯酶药物逐渐减量并在出生后第31天停用。他在出生一个月时临床无症状出院回家。一个月后对他进行评估,情况良好。母亲有MG的阳性病史且体格检查有提示意义,可能足以诊断短暂性新生儿MG,这强调了详细病史的重要性。通过及时诊断和适当管理,大多数新生儿在数周至数月后会自发缓解。