Korényi-Both A, Korényi-Both I
Laboratory Services, 348th General Hospital, USAR, Philadelphia, PA.
J Med. 1987;18(2):93-107.
This addition to the classification of myopathies is a result of the improved diagnostic techniques in muscle pathology in recent years, largely through the application of electronmicroscopy and histochemistry. After summarizing the clinical picture, the authors call for classification: (1) Congenital myopathies due to developmental arrest: myotubular myopathy, congenital fiber type disproportion. (2) Congenital myopathies due to persistent organellar regression: focal loss of cross striation, myopathy with lysis of myofibrils, nemaline body myopathy, zebra body myopathy, spheroid body myopathy, myopathy with tubular aggregates, satellite cell myopathy. (3) Congenital myopathies due to metabolic errors: mitochondrial myopathy, mitochondria-lipid-glycogen disease. (4) Congenital myopathies due to the lack of the trophic influence of innervation: central core disease, multicore, minicore disease. These congenital myopathies have been taken from the ill-defined heterogeneous conglomeration known as amyotonia congenita or floppy infant syndrome and given a place of their own.
近年来，肌肉病理学诊断技术的改进，很大程度上得益于电子显微镜和组织化学的应用，这使得对肌病分类有了新进展。在总结临床表现后，作者呼吁进行如下分类：（1）因发育停滞导致的先天性肌病：肌管性肌病、先天性纤维类型不均一症。（2）因细胞器持续退化导致的先天性肌病：横纹局部缺失、伴有肌原纤维溶解的肌病、杆状体肌病、斑马体肌病、球状体肌病、伴有管状聚集物的肌病、卫星细胞肌病。（3）因代谢错误导致的先天性肌病：线粒体肌病、线粒体 - 脂质 - 糖原病。（4）因缺乏神经支配的营养影响导致的先天性肌病：中央轴空病、多核、微小核病。这些先天性肌病已从定义不明确的异质性集合体（即先天性肌张力缺失或松软婴儿综合征）中分离出来，并拥有了自己的分类地位。
