Diseases Involving Myelin

This chapter focuses on multiple sclerosis and the acquired and inherited peripheral neuropathies. The integrity of myelin sheaths is dependent upon the normal functioning of the myelin-forming oligodendrocytes in the central nervous system (CNS) and Schwann cells in the peripheral nervous system (PNS) as well as on the viability of the axons that they ensheath. Neuronal death inevitably leads to degeneration of axons and secondary degeneration of the myelin surrounding them. Failure of synthesis of normal myelin proteins or lipids is referred to as hypomyelination or dysmyelination. Primary demyelination involves the destruction of myelin with relative sparing of axons, whereas secondary demy­elination includes those disorders in which myelin is involved only after damage to neurons and axons occurs. The integrity of myelin sheaths is dependent upon the normal functioning of myelin-forming oligodendrocytes in the central nervous system (CNS) and Schwann cells in the peripheral nervous system (PNS) as well as on the viability of the axons that they ensheath.