Leigh Syndrome: Serial MRI Findings

Abstract

Purpose : The purpose of this study was to evaluate the effect of the temporal changes in brain lesions on serial MR images during the course ofLeigh syndrome.Materials and Methods : We retrospectively reviewed 11 MR images in four patients diagnosed as suffering from Leigh syndrome on the basis of clinical features, MRI findings, and biochemical data. F이low-up and earlier, MR images were compared and temporal changes in lesions were analyzed, with particular attention to location, size, signal intensity, and contrast enhancement.Results : Initial MRI showed that in order of frequency, the following were involved : bilateral putamina( 4/4), caudate nuclei(2/4), the brain stem(2/4), medial thalamic nuclei(1 /4), and the cerebral cortex( 1/4). In two patients, the size of acute putaminallesions, as seen on fl 이low-up MRI, decreased in the short term(within two weeks); in one patient, strong contrast enhancement of the lesions was observed twelve days after initial MRI. Long term follow-up MRI, over 7 -19 months, showed newly developed lesions (2/4) and atrophy (2/4) or obliteration of previous lesions (3/4) in the basal ganglia, thalami, and brain stem.Conclusion : Serial MRI demonstrated temporal changes in brain lesions during the course of Leigh syndrome. On follow-up MRI, the appearance of bilateral lesions in basal ganglia and the brain stem, not present on initial MRI, may be helpful corroborative evidence to support a diagnosis ofthis syndrome.