Changes of enzyme histochemistry and ultrastructure in mitochondrial myopathy and encephalomyopathy / 中华神经科杂志

ABSTRACT

Objective To investigate the pathological features and clinical characteristics of primary mitochondrial myopathy and encephalomyopathy. Methods 11 cases of mitochondrial myopathy and encephalomyopathy were analyzed on clinical features, histochemical changes and ultrastructure observations of muscles. Results RRFs were found in all 11 cases by muscle staining of MGT under light microscope. 7 cases were only afflicated with muscle weakness, 4 cases also having impairment of central nervous system. The proportion of RRFs was 6.4% to 10.3%. We observed these cases under electronic microscope. In 9 cases,the ultrastructural alterations included subsarcolemmal accumulation of mitochondria,increases of the number,abnormal shape,disarrangement of cristae and paracrystaline inclusion bodies. In 2 cases the increase of mitochondrial number is only the alteration. Conclusions Typical RRF is valuable for diagnoses of mitochondrial myopathy and encephalomyopathy under light microscope. The accumulation of abnormal mitochondrials beneath sarcolemal,especially the paracrystaline inclusion bodies are significant in making a definite diagnosis of this disease under electronic microscope.