Demyelination due to mitochondrial disease (Q41152)

Mitochondrial disorders can cause multifocal and relapsing central nervous system syndromes. Mitochondrial disorders which can cause such syndromes include Mitochondrial Encephalopathy with Lactic Acidosis and Stroke (MELAS), and Leigh's disease. MELAS is a progressive neurodegenerative disorder associated with headache, treatment resistant partial seizures, short stature, muscle weakness, exercise intolerance, deafness, diabetes, and slow progressive dementia. Leigh syndrome or subacute necrotizing encephalomyelopathy is the prototype mitochondrial disease, with hallmark neuroimaging findings.