Cognitive impairment, clinical severity and MRI changes in MELAS syndrome

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

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.

MeSH terms

  • Adult
  • Brain / diagnostic imaging
  • Brain / pathology*
  • Brain / physiopathology*
  • Cognitive Dysfunction*
  • Female
  • Humans
  • MELAS Syndrome / diagnostic imaging
  • MELAS Syndrome / pathology*
  • MELAS Syndrome / physiopathology*
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Young Adult