Argov Z, Gardner-Medwin D, Johnson M A, Mastaglia F L
Arch Neurol. 1980 Nov;37(11):693-6. doi: 10.1001/archneur.1980.00500600041006.
Histometric data on muscle fiber types were studied in two cases of congenital myotonic dystrophy (CMD); one underwent biopsy at the age of 5 months and the other at the age of 10 years. A previously unreported severe deficiency of type IIB fibers were found in both cases. In addition, in the first case, there was type I fiber preponderance and hypotrophy as described in cases of congenital fiber type disproportion (CFTD). It is suggested that an abnormality of motor unit maturation may be common to CMD and to CFTD, and that this results from a disorder of neural trophic influences during muscle development.
对两例先天性肌强直营养不良（CMD）患者的肌纤维类型进行了组织计量学数据研究；一例在5个月大时接受活检，另一例在10岁时接受活检。在这两例患者中均发现了此前未报道的IIB型纤维严重缺乏的情况。此外，在第一例患者中，存在如先天性纤维类型比例失调（CFTD）病例中所描述的I型纤维优势和萎缩。提示运动单位成熟异常可能是CMD和CFTD所共有的，并且这是由肌肉发育过程中神经营养影响紊乱所致。
