Impairment of central and peripheral myelin in mitochondrial diseases

Clinical or sub-clinical impairment of central and peripheral myelin is of ten part of the overlapping multisystem disorders associated with a variety of mitochondrial (mt)DNA abnormalities. Suboptimal energy metabolism of the oligodendrocytes and Schwann cells carrying mitochondrial defects may cause insufficient production of myelin. Further, edema, vascular and toxic factors may directly damage myelin. The recognition that certain mtDNA point mutations are associated with inflammatory demyelination of the central nervous system suggests that additional mechanisms besides degeneration need to be considered in the development of some forms of myelin damage.