MELAS syndrome in a child: CT and MR findings.
10.3348/jkrs.1993.29.1.160
- Author:
Hye Young CHOI
;
Soo Jong HONG
;
Jeong Hee CHO
;
Dae Chul SUH
;
Chang Yee HONG
- Publication Type:Original Article
- MeSH:
Acidosis, Lactic;
Basal Ganglia;
Brain Diseases;
Child*;
Humans;
Infarction;
Magnetic Resonance Imaging;
Male;
MELAS Syndrome*;
Mitochondria;
Mitochondrial Encephalomyopathies;
Muscular Diseases;
Rare Diseases;
Respiration
- From:Journal of the Korean Radiological Society
1993;29(1):160-164
- CountryRepublic of Korea
- Language:English
-
Abstract:
MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) is one of the mitochondrial encephalomyopathy, A rare disease caused by a disturbance of the mitochondrial chain of respiration. MELAS is confirmed by typical light and electron microscopic findings : "ragged red fibers" by modified Gomori trichrome stain on light microscope and numerous abormal mitochondria on electron microscope. We experienced a boy with the characteristic clinical and pathologic findings of MELAS. Our patient demonstrated bilateral basal ganglia calcifications and infarction at right parieto-occipital and thalamic areas on CT and MR We found that MRI was more sensitive and represented the infarcted lesions better than CT. Detection of cerebral insults of MELAS by MRI is important in making decision on patient treatment and also in predicion of the patient prognosis.
