Autoantibody profile in a Malaysian cohort of chronic inflammatory demyelinating polyneuropathy
Document Type
Article
Publication Date
3-1-2022
Abstract
We report on our cohort of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) who fulfilled the 2010 diagnostic criteria of CIDP. Patients were consecutively recruited and their demographics, clinical features and serological analysis of autoantibodies against neurofascin (NF)-155, NF-186, contactin-1 (CNTN1) and contactin-associated protein 1 were obtained. A total of 26 patients for which there was serologic testing were included: 22 typical CIDP, 3 distal CIDP and 1 multifocal CIDP. Of these, 2 patients had previously reported paranodal antibodies; one with autoantibodies IgG4 against NF155 and one with IgG4 against CNTN1. The patient with IgG4 anti-NF155 had young-onset, predominantly distal phenotype with associated tremor and sensory ataxia and poor response to intravenous immunoglobulin (IVIG). The patient with IgG4 anti-CNTN1 antibodies had a subacute onset, sensory ataxia, membranous nephropathy but responded poorly to IVIG. Autoimmune nodopathies represented 8% of our CIDP cohort. The clinical features and treatment response of patients with IgG4 anti-NF155 and anti-CNTN1 were similar to previous reports. Detecting the presence of autoimmune nodopathies was crucial in refining the diagnosis and determining the prognosis. (c) 2022 Elsevier B.V. All rights reserved.
Keywords
Autoimmune nodopathy, Chronic inflammatory demyelinating polyneuropathy, CIDP, Contactin, Neurofascin, Node of Ranvier
Divisions
fac_med,medicinedept
Funders
University of Malaya Department of Medicine Research Grant [Grant No: DOMRSF2021-01]
Publication Title
Neuromuscular Disorders
Volume
32
Issue
3
Publisher
Pergamon-Elsevier Science Ltd
Publisher Location
THE BOULEVARD, LANGFORD LANE, KIDLINGTON, OXFORD OX5 1GB, ENGLAND