Leigh Syndrome: Serial MRI Findings

ABSTRACT

PURPOSE: The purpose of this study was to evaluate the effect of the temporal changes in brain lesions onserial MR images during the course of Leigh syndrome. MATERIALS AND METHODS: We retrospectively reviewed 11 MRimages in four patients diagnosed as suffering from Leigh syndrome on the basis of clinical features, MRIfindings, and biochemical data. Follow-up and earlier, MR images were compared and temporal changes in lesionswere 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), caudatenuclei (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 putaminal lesions, as seen on follow-up MRI, decreased in the short term (within two weeks); inone patient, strong contrast enhancement of the lesions was observed twelve days after initial MRI. Long termfollow-up MRI, over 7 - 19 months, showed newly developed lesions (2/4) and atrophy (2/4) or obliteration ofprevious lesions (3/4) in the basal ganglia, thalami, and brain stem. CONCLUSION: Serial MRI demonstratedtemporal changes in brain lesions during the course of Leigh syndrome. On follow-up MRI, the appearance ofbilateral lesions in basal ganglia and the brain stem, not present on initial MRI, may be helpful corroborativeevidence to support a diagnosis of this syndrome.