Special expression of anti-mitochondrial antibody in the muscle fibers of patients with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes
- VernacularTitle:抗线粒体抗体在线粒体脑肌病伴高乳酸血症和脑卒中样发作综合征患者肌纤维中的免疫组织化学表达
- Author:
Haiyan WANG
;
Xiaokun QI
;
Sheng YAO
;
Xia LEI
;
Liping LI
;
Yun YUAN
- Publication Type:Journal Article
- Keywords:
MELAS syndrome;
Mitochandria;
Autoantibodies;
Immunohistochemistry
- From:
Chinese Journal of Neurology
2008;41(11):760-762
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the special expression and diagnostic value of anti-mitochondrial antibody (AMA) in the muscle tissues of patients with mitochondria encephalopathy, lactic acidosis and stroke-like episodes (MELAS). Methods All cases including 10 cases of MELAS, which were diagnosed definitely by clinical, pathological and DNA analysis, and 2 cases of normal control and 3 cases of necrotic muscle fibers, underwent muscle biopsies in order to carry out AMA staining. At the same time the original MGT staining was observed. Results A large number of ragged brown fibers (RBF) were observed under light microscopy with AMA immunohistochemical staining in the muscle tissues of patients with MELAS. But no RBF were observed in control groups. Conclusion RBF might be the specific performance of paraplastic mitochondrial, which was similar to the ragged red fibers, so RBF might be a pathological index in evaluating abnormal mitochondrial function and in diagnosing mitochondriopathy.