Silver M M, Hudson A J, Vilos G A, Banerjee D
Med Hypotheses. 1985 Mar;16(3):207-20. doi: 10.1016/0306-9877(85)90002-7.
An environmental factor acting on the fetus is thought to cause a neonatal syndrome characterized by marked muscular hypotonia, lack of respiratory drive and feeding difficulties, in some infants born to mothers with myotonic dystrophy. Mortality is high, especially amongst those babies born prematurely, but muscle strength and tone improve rapidly in survivors. Nevertheless, most survivors have physical deformities and mental retardation and are thought to develop myotonic dystrophy later. We propose that alterations in maternal insulin secretion (usual in myotonic dystrophy subjects) alter fetal blood glucose and amino acid levels and retard growth and maturation of fetal skeletal muscle. This leads to severe muscular hypotonia in affected infants. Also, we suggest that infants who die during the perinatal period may not have inherited the defective autosomal dominant gene that causes myotonic dystrophy.
有一种环境因素作用于胎儿，被认为会导致一种新生儿综合征，其特征为明显的肌肉张力减退、呼吸驱动力缺乏和喂养困难，在患有强直性肌营养不良的母亲所生的一些婴儿中出现。死亡率很高，尤其是在那些早产的婴儿中，但幸存者的肌肉力量和张力会迅速改善。然而，大多数幸存者有身体畸形和智力迟钝，并且被认为后来会发展为强直性肌营养不良。我们提出，母亲胰岛素分泌的改变（强直性肌营养不良患者中常见）会改变胎儿血糖和氨基酸水平，并延缓胎儿骨骼肌的生长和成熟。这会导致受影响婴儿出现严重的肌肉张力减退。此外，我们认为在围产期死亡的婴儿可能没有遗传导致强直性肌营养不良的有缺陷的常染色体显性基因。
