Abstract:Objective: To investigate the imaging characteristics of MELAS syndrome and assess the diagnostic value of MRI. Methods: The clinical and imaging findings of 6 patients with MELAS syndrome were analyzed retrospectively. Result: Abnormal calcification on CT was not found in 3 patients. 10 stroke-like episodes presented long T1 long T2 signals in the cortex and subcortical white matter of temporal, parietal, and occipital lobes, and 2 presented high signals in diffusion weighted images in periods of attacks. In convalescent periods, local atrophy and laminar necrosis were showed. Muscle biopsy showed ragged red fibers in 5 patients. Conclusion: Recurrent lesions in the cortex and subcortical white matter of temporal, parietal, and occipital lobe on MRI might provide clues for diagnosis of MELAS syndrome. DWI might show lesions earlier than T2WI in MELAS syndrome similar to cerebral infarction.