Inclusion-body myositis - Clinical, diagnostic, and pathologic aspects

Inclusion-body myositis - Clinical, diagnostic, and pathologic aspects
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DOI:
10.1212/01.wnl.0000192260.33106.bb
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发表时间:
2006-01-01
期刊:
影响因子:
9.9
通讯作者:
Askanas, V
Askanas, V
中科院分区:
医学1区
文献类型:
--
作者:
Engel, WK;Askanas, V

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散发性包涵体肌炎(s-IBM)的诊断方面,并提出了一些意见,我们自己的方法来治疗,以促进本次研讨会的目标,这是有组织的,以激发新的想法有关的原因和治疗这一目前无法解决的疾病。s-IBM是最常见的、进行性的、使人衰弱的肌肉疾病,开始于50岁以上的人,并且在男性中更常见。诊断参数审查的临床,肌肉活检组织化学,电生理和CSF评价。总的来说,s-IBM肌纤维中的退行性现象似乎是进行性的、不可阻挡的虚弱的主要原因,而不是淋巴细胞炎症。现有的治疗方法仅对一些s-IBM患者有轻微的、暂时的益处,这表明迫切需要确定的治疗方法。
The diagnostic aspects of sporadic inclusion-body myositis (s-IBM), and a few comments on our own approach to its treatment, are presented to foster the goals of this symposium, which was organized to provoke new ideas concerning the cause and treatment of this currently unsolvable disease. s-IBM is the most common, progressive, debilitating muscle disease beginning in persons over age 50 years, and it is more common in men. Diagnostic parameters reviewed are clinical, muscle-biopsy histochemistry, electrophysiologic and CSF evaluations. Overall, the degenerative phenomena in s-IBM muscle fibers seem to be the major cause of the progressive, unstoppable weakness, rather than the lymphocytic inflammation. Available treatments are of only slight, temporary benefit for only some s-IBM patients, indicating a desperate need for definitive therapies.