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Mitochondrial myopathy

Encephalopathy

Lactic

Acidosis

Stroke-like symptoms

Inherited disorder of intracellular energy production

Mitochondrial Disorder:

Point mutation in mitochondrial DNA

Imaging Findings:

Best Diagnostic Clue:

Stroke-like cortical lesions

Classic shifting spread (appearance, disappearance, reappearance elsewhere)

Lesions cross vascular territories

Parieto-Occipital > Temporo-Parietal, Basal Ganglia

Multiple lesions are common

Gyriform swelling often spares underlying white matter

Chronic: Atrophy

MRA: Normal

Leigh’s Disease (most commonly bilateral putaminal involvement)

Other Mitochondrial disorders

Carbon Monoxide poisoning (most commonly globus palllidus)

Myoclonic epilepsy with ragged-red fibers