Mitochondrial Encephalomyopathy in Children:MRI Appearances / 实用放射学杂志

ABSTRACT

Objective To study MR findings in 20 children aged from 10 months to 14 years with mitochondrial encephalomyopathy.Methods MRI evaluations were done in 20 children with mitochondrial encephalomyopathy proved by muscle biopsy and biochemical laboratory examinations.Results Brain parenchymal lesions in all cases were low signal intensity on T 1-weighted and high signal intensity on T 2-weighted images.Brain atrophy was showed in different degrees in 8 children.18 patients had involvement of gray matter,10 had only the deep gray matter involved and 4 cases had both the deep gray matter and the cerebral cortex involved simultaneously.4 patients showed deep gray matter abnormalities and cerebral infarction with involvement of cortex and subcortical white matter.2 patients had exclusively involvement of white matter,which were nonspecific white matter changes of the trigonal area.Conclusion MRI findings in mitochondrial encephalomyopathy are varied when gray matter especially deep gray matter involved,brain atrophy,untypical infarction and involvement of peripheral white matter are showed on MR,and associated with a variety of neuromuscular symptoms in children mitochondrial encephalomyopathy should be consider.