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. 2019 Jan:44:53-57.
doi: 10.1016/j.mito.2017.12.012. Epub 2017 Dec 29.

Cognitive impairment, clinical severity and MRI changes in MELAS syndrome

Torsten Kraya  1 , Lena Neumann  2 , Yvonne Paelecke-Habermann  3 , Marcus Deschauer  4 , Dietrich Stoevesandt  5 , Stephan Zierz  2 , Stefan Watzke  6
Affiliations

Cognitive impairment, clinical severity and MRI changes in MELAS syndrome

Torsten Kraya et al. Mitochondrion. 2019 Jan.

Abstract

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.

Keywords: Cognitive impairment; Dementia; MELAS; MRI; Mitochondrial disorders.

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