- Acronym: mitochondrial encephalopathy, lactic acidosis, stroke-like episodes
- Due to mitochondrial DNA mutation
- Stroke like symptoms, encephalopathy, seizures, myopathy
- Lactic acid in CSF
- Ragged red fibres on muscle biopsy
- Cortico-subcortical T2 hyperintensities in parietal, temporal and occipital lobes
- Topography does not correspond to single vascular territory
- Cortical and leptomeningeal contrast enhancement
- DWI – restriction or increased diffusion depending on phase of lesions
- Spectroscopy – lactate peak in lesions, in normal appearing brain areas and in CSF of lateral ventricles
- Bilateral basal ganglia calcifications
Cheon, Jung-Eun, et al. “Leukodystrophy in children: a pictorial review of MR imaging features.” Radiographics 22.3 (2002): 461-476.
Saenz, Rocky C. “The disappearing basal ganglia sign.” Radiology 234.1 (2005): 242-243.