Fujimura-Kiyono Chieko, Racz Gabor Z, Nishino Ichizo
Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4-1-1 Ogawahigashi-Cho Kodaira, Tokyo 187-8502, Japan.
Neurol India. 2008 Jul-Sep;56(3):325-32. doi: 10.4103/0028-3886.43451.
The term congenital myopathy is applied to muscle disorders presenting with generalized muscle weakness and hypotonia from early infancy with delayed developmental milestones. The congenital myopathies have been classified into various categories based on morphological findings on muscle biopsy. Although the clinical symptoms may seem homogenous, the genetic basis is remarkably variable. This review will focus on myotubular myopathy, centronuclear myopathy, central core disease, and congenital neuromuscular disease with uniform Type 1 fiber, myopathies that are subjects of our ongoing examinations.
先天性肌病这一术语适用于从婴儿早期就出现全身肌无力和肌张力减退且发育里程碑延迟的肌肉疾病。根据肌肉活检的形态学发现,先天性肌病已被分为不同类别。尽管临床症状可能看似相同,但其遗传基础却显著不同。本综述将聚焦于中央轴空病、核仁中央性肌病、中央核心疾病以及具有均匀1型纤维的先天性神经肌肉疾病,这些都是我们正在研究的肌病。