Abstract
ObjectiveTo examine clinical severity, cognitive impairment, and MRI changes in patients with MELAS syndrome. MethodsCognitive-mnestic functions, brain MRI (lesion load, cella media index) and clinical severity of ten patients with MELAS syndrome were examined. All patients carried the m.3243A>G mutation. ResultsThe detailed neuropsychological assessment revealed cognitive deficits in attention, executive function, visuoperception, and -construction. There were significant correlations between these cognitive changes, lesion load in MRI, disturbances in everyday life (clinical scale), and high scores in NMDAS. ConclusionPatients with MELAS syndrome showed no global neuropsychological deficit, but rather distinct cognitive deficits.
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