Eymard B, Morel E, Dulac O, Moutard-Codou M L, Jeannot E, Harpey J P, Rondot P, Bach J F
Service de Neurologie, Hôpital de la Salpêtrière, Paris.
Rev Neurol (Paris). 1989;145(10):696-701.
Forty-two pregnancies in 39 myasthenic mothers were studied between 1978 and 1987. In 4 cases myasthenia gravis began during pregnancy and for 20 patients the clinical condition exacerbated in 15 cases, usually during the first 3 months, or during the postpartum. Except for 1 case, clinical exacerbation was controlled by anticholinesterase drug adjustment. Obstetrical problems were uncommon: abortion in 1 case and premature delivery in 4 cases. In 2 babies with severe fetal involvement polyhydramnios was present. Twenty-one babies had neonatal myasthenia gravis (NMG). In 17 cases, transient symptoms were present at delivery or shortly afterwards and full recovery occurred from a few days to 4 months. In 4 babies the clinical presentation was atypical: 1 showed a long evolution (15 months) and residual facial bilateral weakness, and three others presented fetal involvement (arthrogryposis). In these latter cases, presentation was severe with polyhydramnios, respiratory failure and long standing evolution in 2 cases (4 months and 1 year). Antiacetylcholine receptor antibodies (anti-AChR Ab) were found in all myasthenic babies and in 19 out of 20 asymptomatic babies. Maternal antibody titers were usually slightly higher than umbilical cord titers. There was a good correlation between maternal titer and onset or severity of disorder in baby. Among the 15 high titer mothers (greater than 60 nM), 13 had a myasthenic baby, 6 of them with serious disease. Conversely all low titer mothers (less than 10 nM) had a symptom-free baby. Therefore, anti-AChR Ab titration in the mother is predictive for NMG onset. Mother's myasthenia gravis severity and treatment were not correlated to the clinical condition of the newborns.
1978年至1987年间,对39例重症肌无力母亲的42次妊娠情况进行了研究。4例重症肌无力在孕期发病,20例患者的病情在15例中加重,通常在孕早期3个月内或产后。除1例患者外,临床症状加重通过调整抗胆碱酯酶药物得到控制。产科问题并不常见:1例流产,4例早产。2例胎儿严重受累的婴儿出现羊水过多。21例婴儿患有新生儿重症肌无力(NMG)。17例在分娩时或产后不久出现短暂症状,数天至4个月内完全恢复。4例婴儿临床表现不典型:1例病程较长(15个月)且遗留双侧面部肌无力,另外3例出现胎儿受累(关节挛缩)。在这些后一种情况下,病情严重,2例出现羊水过多、呼吸衰竭且病程较长(4个月和1年)。所有患重症肌无力的婴儿及20例无症状婴儿中的19例均检测到抗乙酰胆碱受体抗体(抗AChR Ab)。母亲抗体滴度通常略高于脐带血滴度。母亲抗体滴度与婴儿疾病的发病或严重程度之间存在良好相关性。在15例高滴度母亲(大于60 nM)中,13例婴儿患有重症肌无力,其中6例病情严重。相反,所有低滴度母亲(小于10 nM)的婴儿均无症状。因此,母亲抗AChR Ab滴度测定可预测NMG的发病。母亲重症肌无力的严重程度及治疗与新生儿的临床状况无关。