Nodopathy: chronic inflammatory demyelinating polyneuropathy with anti-neurofascin 155 antibodies

Anti-neurofascin 155 antibodies cause myelin detachment and ‘nodopathy’ in a subgroup of patients diagnosed with chronic inflammatory demyelinating polyneuropathy While peripheral neuropathies have conventionally been classified into demyelinating or axonal, recent studies have proposed a novel concept, namely a ‘nodopathy’, whereby microstructural changes restricted to the nodal and paranodal regions may induce significant nerve dysfunction.1 The nodes of Ranvier are the sites where sodium channels are clustered at their highest density and therefore remain the critically important region for the generation of action potentials. At the paranodes, the terminal myelin loops are tightly connected to the axolemma by adhesion molecules, such as contactin-1, contactin-associated protein (Caspr) and neurofascin-155. Detachment of the myelin terminal loops may lead to massive leaks of driving current, with resultant substantial reduction of the safety factor for impulse transmission, and consequent conduction failure.1 The term nodopathy focuses attention to the...

Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Kuwabara, S., Misawa, S., Mori, M. Tags: Editorial commentaries Source Type: research