Neville H E, Ringel S P, Guggenheim M A, Wehling C A, Starcevich J M
Neurology. 1983 Apr;33(4):483-8. doi: 10.1212/wnl.33.4.483.
We report muscle biopsy abnormalities in four patients with a chronic cholestatic syndrome, low serum vitamin E levels, absent reflexes, mild limb weakness, ataxia, and sensory loss in arms and legs. Skeletal muscle fibers contained multiple autofluorescent inclusions that show strong acid phosphatase and esterase reactivity. By electronmicroscopy, the inclusions lying between myofibrils were membrane-bound dense bodies having characteristics of both lysosomes and lipopigment material. The material was similar to that observed in vitamin E-deficient animals and probably formed in response to disordered intracellular lipid peroxidation.
我们报告了4例患有慢性胆汁淤积综合征、血清维生素E水平低、反射消失、轻度肢体无力、共济失调以及手臂和腿部感觉丧失的患者的肌肉活检异常情况。骨骼肌纤维含有多个自发荧光包涵体，这些包涵体显示出强酸性磷酸酶和酯酶反应性。通过电子显微镜观察，位于肌原纤维之间的包涵体是膜结合的致密小体，具有溶酶体和脂色素物质的特征。该物质与在维生素E缺乏动物中观察到的物质相似，可能是对细胞内脂质过氧化紊乱的反应而形成的。
