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Update on Nodopathies of the Peripheral Nerve

  • Neuromuscular Disorders (C Fournier, Section Editor)
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Current Treatment Options in Neurology Aims and scope Submit manuscript

Abstract

Purpose of review

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.

Recent findings

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.

Summary

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.

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Authors and Affiliations

  1. Department of Neurology, Emory University, 12 Executive Park Drive NE, Atlanta, GA, 30329, USA

    Joaquin A. Vizcarra MD, Taylor B. Harrison MD & Rocio Garcia-Santibanez MD

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Correspondence to Rocio Garcia-Santibanez MD.

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Vizcarra, J.A., Harrison, T.B. & Garcia-Santibanez, R. Update on Nodopathies of the Peripheral Nerve. Curr Treat Options Neurol 23, 25 (2021). https://doi.org/10.1007/s11940-021-00683-3

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