Steven S. Scherer1 and John Svaren2 1Department of Neurology, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA 2Department of Comparative Biosciences, Waisman Center, University of Wisconsin-Madison, Madison, Wisconsin 53705, USA Correspondence: sschererpennmedicine.upenn.edu

This is a review of inherited and acquired causes of human demyelinating neuropathies and a subset of disorders that affect axon–Schwann cell interactions. Nearly all inherited demyelinating neuropathies are caused by mutations in genes that are expressed by myelinating Schwann cells, affecting diverse functions in a cell-autonomous manner. The most common acquired demyelinating neuropathies are Guillain–Barré syndrome and chronic, inflammatory demyelinating polyneuropathy, both of which are immune-mediated. An additional group of inherited and acquired disorders affect axon–Schwann cell interactions in the nodal region. Overall, these disorders affect the formation of myelin and its maintenance, with superimposed axonal loss that is clinically important.