Acquired neuromyotonia: a new autoantibody-mediated neuronal potassium channelopathy

Neuromyotonia (Isaacs syndrome) is a rare and heterogenous syndrome of continuous motor unit activity of peripheral nerve origin that manifests as various combinations of muscle stiffness, cramps, twitching, weakness, and delayed muscle relaxation. Although neuromyotonia may accompany an assortment of inherited diseases, most cases are acquired. The observation that the acquired form is often associated with an autoimmune disorder, and the demonstration that some cases improve after plasma exchange, has led to a search for an immune-mediated etiology. This review summarizes the recent immunological and electrophysiological evidence that autoantibodies to voltage-gated potassium channels produce the peripheral motor nerve hyperexcitability that characterizes neuromyotonia and thus establishes acquired neuromyotonia as an autoantibody-mediated disorder. In the nervous system, ion channels and neurotransmitter receptors that function as ligand-gated ion channels seem to be favored targets for autoantibody attack, and neuromyotonia can now be added to the growing list of autoimmune channelopathies.