Cognitive impairment, clinical severity and MRI changes in MELAS syndrome.

OBJECTIVE

To examine clinical severity, cognitive impairment, and MRI changes in patients with MELAS syndrome.

METHODS

Cognitive-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.

RESULTS

The 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.

CONCLUSION

Patients with MELAS syndrome showed no global neuropsychological deficit, but rather distinct cognitive deficits.