Update on Nodopathies of the Peripheral Nerve

Abstract

The purpose of this paper is to provide an up-to-date review of the current knowledge on immune-mediated neuropathies secondary to antibodies against nodal and paranodal proteins. Antibodies against neurofascins and contactin have recently been linked to characteristic neuropathy presentations including a more acute/subacute presentation, involvement of cranial nerves, presence of tremor, and refractory to typical first-line treatments for immune-mediated neuropathies. Nodopathies are a group of disorders associated with humoral autoimmunity to antigens of the node, paranode, and juxtaparanode. The clinical syndromes most commonly associated with these antibodies include acute inflammatory demyelinating polyneuropathy, chronic inflammatory demyelinating polyneuropathy, and combined central and peripheral demyelination. Treatment response is variable, and though most data reported is on NF155, there appears to be a better response to plasma exchange and rituximab followed by prednisone and then IVIG.