常染色体隐性肢带肌营养不良（ＬＧＭＳ）是一组遗传异质性肌肉疾病，其特征为进行性近端肢肌无力。已定位６个不同基因位点，并且在文献中已证明编码肌肉聚多糖复合物组成成分（α－，β－，γ－和σ－肉聚多糖）基因的致病性突变。原发性“肌肉聚多糖病”受累的ＬＧＭＤ... Autosomal recessive limb muscular dystrophy (LGMS) is a group of hereditary heterogeneous muscular diseases characterized by progressive proximal limb muscle weakness. Six different genetic loci have been mapped and causative mutations in the genes encoding the muscle polysaccharide complex components (alpha-, beta-, gamma- and sigma-polysaccharides) have been demonstrated in the literature. Primary “muscle polysaccharide disease” involvement of LGMD ...