IMPROVEMENT OF LESIONS SHOWN ON MRI AND CT SCAN BY ADMINISTRATION OF DICHLOROACETATE IN PATIENTS WITH LEIGH-SYNDROME

Brain lesions exhibited on MRI and CT scan in 2 patients with mitochon drial encephalomyelopathy representing Leigh syndrome were improved by administration of dichloroacetate (DCA). One patient had pyruvic acid dehydrogenase complex (PDHC) deficiency, the other had complex I defi ciency. The efficacy of DCA was transient in the patient with the PDHC deficiency, lasting for about 2.5 months. The patient died at the age of 6, about 2 years after the initiation of DCA treatment. DCA admini stration was started in the patient with complex I deficiency when he was 15 months old and it is still effective at his present age of 24 m onths, His motor ability is developing, and he could walk without supp ort at the age of 19 months. DCA administration should be tried in pat ients with mitochondrial diseases.