A study on the expression of anti-mitochondriai antibody in the brain of patients with MELAS syndrome

VernacularTitle:抗线粒体抗体在MELAS综合征脑活检组织的表达观察
Author: Xiaokun QI ; Sheng YAO ; Haiyan WANG ; Yueshan PIAO ; Dehong LU ; Yun YUAN
Publication Type:Journal Article
Keywords: Biopsy; Mitochondrial myopathies; Mitochondrial encephalopathy lactic acidosis strok-like episodes
From: Chinese Journal of Internal Medicine 2009;48(4):274-276
CountryChina
Language:Chinese
Abstract: Objective To investigate the pathological changes and pathogenesis of the MELAS syndrome (mitochondrial encephalopathy lactic acidosis strok-like episodes ) by using the method of immunohistochemical staining in the brain biopsy specimens with anti-mitochondrial antibody (AMA).Methods We performed immunohistochemical staining in 3 confirmed MELAS patients' paraffin-imbed brain biopsy specimens.Results Small vessel proliferation and the uneven thickness of the wall were found in the 3 MELAS patients.A lot of brown deposits was shown in the wall of small vessels and also noted in neurons.Conclusions The main pathological change in the MELAS brain biopsy immunohistochemical staining with AMA was the small vessel proliferation,indicating that abnormal mitochondria accumulated in the vascular smooth muscle,endothelial cell and neurons of the lesion sites.This finding was consistent with the electron microscopic discovery and valuable for the diagnosis of MELAS.