Update on Nodopathies of the Peripheral Nerve

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 characteris...

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Bibliographic Details
Published inCurrent treatment options in neurology Vol. 23; no. 8
Main Authors Vizcarra, Joaquin A., Harrison, Taylor B., Garcia-Santibanez, Rocio
Format Journal Article
LanguageEnglish
Published New York Springer US 01.08.2021
Subjects
Critical Care Medicine
Diabetes
Family Medicine
General Practice
Intensive
Internal Medicine
Medicine
Medicine & Public Health
Neurology
Neuromuscular Disorders (C Fournier
Ophthalmology
Section Editor
Topical Collection on Neuromuscular Disorders
Nodopathies
Peripheral nerve
neurofascin
Treatment
CIDP
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Summary: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.
ISSN:1092-8480
1534-3138
DOI:10.1007/s11940-021-00683-3