Pathophysiology of the autoimmune nodopathy based on pathological findings

Abstract

The pathophysiology of nodopathy is a disorder of node of Ranvier caused by autoantibodies. Autoantibodies act on nodes of Ranvier to disrupt sodium channels and cause conduction failure. Pathologic studies have revealed impaired Na channels, loss of transverse bands, loss of Schwann cell microvilli, elongation of node of Ranvier, and detachment of the Schwann cell terminal loop and axon. Based on imaging tests, the distribution of the lesions in patients with nodopathy is expected to be diffuse and patchy in peripheral nerves. The distal extremities are more affected because the number of nodes of Ranvier is length–dependent in nerve fibers.