轴性肌病的研究进展

Research progress of axial myopathy

轴性肌病是一组累及中轴肌的肌病总称,主要指一组椎旁肌单独或显著受累的骨骼肌病,伴或不伴有全身骨骼肌受累。中轴肌无力在临床上常被忽视,目前文献对中轴肌受累的评价,主要是针对椎旁肌(即竖脊肌)的评估,椎旁肌无力临床表现为垂头综合征和躯干前屈征。体格检查和骨骼肌磁共振成像,尤其是全身肌肉磁共振成像扫描对中轴肌的评估至关重要。重视轴性肌病的诊断与鉴别有助于避免遗漏可治疗疾病,并可加强对相关肌病的理解和早期识别,具有重要临床意义。

Axial myopathy is a general term for a group of myopathy involving the axial muscles.It refers to a group of skeletal myopathy in which paraspinal muscles are individually or significantly affected,with or without the involvement of whole body skeletal muscles.Axial muscle weakness is often ignored in clinical practice.The evaluation of axial muscle is mainly the evaluation of the paraspinal muscles(erector spinae)in current literature.The clinical manifestations of paraspinal muscle weakness are dropped head syndrome and camptocormia.Physical examination and skeletal muscle magnetic resonance imaging,especially the whole body muscle magnetic resonance scan,are vital for the evaluation of axial muscle.It is of great clinical significance to increase attention to the diagnosis and differentiation of axial myopathy,which is helpful to avoid missing treatable diseases and improve the understanding and early recognition of associated myopath.