The Studies On The Expression Of Anti-mitochondrial Antibody In The Brain And Muscle Fibers Of Patients With MELAS Syndrome

Background and Purpose:To investigate the special expression and diagnostic value of anti-mitochondrial antibody(AMA) in muscle tissues of those patients with MELAS.Methods:All cases including 10 cases of mitochondria encephalopathy,lactic acidosis and stroke-like episodes(MELAS),which were diagnosed definitely by clinical,pathological and/or DNA analysis,and 2 cases of normal control and 3 cases of necrotic muscle fibers, all of 15 cases were collected for muscle biopsies in order to carry out AMA staining.At the same time observe the original MGT staining.Results:A large number of ragged brown fibers(RBF) were observed under light microscopy in AMA immunohistochemical staining in the muscle tissues of patients with MELAS.But no RBF were observed in control groups.Conclusions:RBF might be the specific performance of paraplastic mitochondrial,which was similar to the ragged red fibers,so RBF might be a pathological index for evaluation of abnormal mitochondrial function and in diagnosing mitochondriopathy. Objective To investigate the pathological changes of MELAS syndrome by using the method of immunohistochemical staining in the brain biopsy specimens.with anti-mitochondrial antibody(AMA)Method We performed immunohistochemical staining in 3 definite MELAS patients' paraffins-imbed brain biopsy specimens.Results The small vessels hyperplasia and the uneven thickness of the wall were found in the 3 definite patients with lots of brown deposits,which were also noted in the neurons. There was no remarkable vessels hyperplasia in the other four controls,but the brown deposits located in some vascular smooth muscle,endothelial cell and neurons.Conclusion The main pathological changes of the MELAS brain biopsy immunohistochemical staining by anti-mitochondrial antibody(AMA) were the small vessels hyperplasia,indicating that the abnormal mitochondria accumulated in the vascular smooth muscle,endothelial cell and neurons of the diseased regions.These findings consistent with the electron microscopic discovery were valuable for the diagnosis of MELAS.