The clinical and pathological character analysis of limb-girdle muscular dystrophy type 2B misdiagnosed to polymyositis / 中国医师进修杂志

ABSTRACT

Objective To clarify the differential diagnosis between limb-girdle muscular dystrophy type 2B (LGMD2B) and polymyositis (PM). Methods Analyzed the character of clinical, histochemical and immunohistochemical pathologic with biopsied skeletal muscle in 5 patients with LGMD2B and the relationship with PM. Results Both LGMD2B and PM showed the muscle weakness in proximal limbs and elevated creatinkinase level and electromyogram showed myogenic damage. Histochemical stains it was similar to PM, that was the muscle fibers degenerating, regenerating and necrotic and the infiltrating inflammatory cells in different extent in 5 patients. Immunohistoehemical stains anfi-Dysferlin monoelonal antibody showed that Dysferlin was deficient on sarcolemma in LGMD2B, but it was normal in PM. Conclusions It is resemble on the clinical and histoehemieal pathologic characters in both LGMD2B and PM. It is key to antidiastole LGMD2B and PM by the immunohistochemieal stain of anti-Dysferlin monoelonal antibody.