包涵体肌炎
肌炎
医学
病理
肌病
多发性肌炎
免疫染色
免疫组织化学
作者
Narayanappa Gayathri,Anisya-Vasanth,M Veerendra Kumar,Suman Das,Vani Santosh,T C Yasha,Y Ramamohan,AB Taly,M Gourie‐Devi,S. K. Shankar
出处
期刊:PubMed
日期:2000-04-25
卷期号:19 (1): 13-20
被引量:4
摘要
Clinical, histological, immunohistochemical and ultrastructural features of 5 cases of inclusion body myositis -4 sporadic (s-IBM) and one hereditary (h-IBM) form are described. These patients (3 men, 2 women) had chronic progressive weakness of varying severity in all 4 extremities with sparing of cranial muscles. Elevation of CPK was noted in 2 patients. Electromyography revealed features of myopathy in 4 and additional neurogenic changes in 2 subjects. Clinical diagnosis was often other than inclusion body myositis. Presence of characteristic eosinophilic inclusions within the vacuoles established the diagnosis. The inclusions were congophilic and showed positivity to ubiquitin, beta-amyloid and SMI-31 in the sporadic cases while congophila was absent in the hereditary form. Immunostaining to hyperphosphorylated-tau was negative in both s-IBM and h-IBM. Membraneous whorls were observed at ultrastructural level. None of the patients improved with steroids and trial with other immunosuppressants was unsuccessful.
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