Bruni J, Bilbao J M, Pritzker P H
Can J Neurol Sci. 1977 Feb;4(1):77-80.
A 38-year-old patient with the clinical picture of a progressive myopathy resembling limb girdle muscular dystrophy is presented. Muscle biopsy showed amyloid deposits in the walls of small endomysial blood vessels. There was no clinical or physiological evidence of peripheral nerve involvement, no plasma cell dyscrasia and no generalized amyloidosis. There was no muscle fiber hypertrophy, inflammation or neurogenic change. Theere was no response to steroid therapy. The etiopathogenesis of this amyloid angiopathy is undetermined. The extensive vessel involvement with amyloid deposition and the absence of changes indicative of muscular dystropyy or inflammatory myopathy leads us to favor an ischemic basis for this patient's myopathy.
本文报告了一名38岁患者，其临床表现为进行性肌病，类似肢带型肌营养不良。肌肉活检显示肌内膜小血管壁有淀粉样沉积物。没有外周神经受累的临床或生理学证据，没有浆细胞异常增生，也没有全身性淀粉样变性。没有肌纤维肥大、炎症或神经源性改变。类固醇治疗无效。这种淀粉样血管病的病因尚未确定。淀粉样沉积物广泛累及血管，且无提示肌营养不良或炎性肌病的改变，这使我们倾向于认为该患者肌病的基础是缺血。
