Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment.

Authors:
Helmar Christoph Lehmann
Helmar Christoph Lehmann
University of Cologne
David Burke
David Burke
Institute of Clinical Neurosciences
Australia
Satoshi Kuwabara
Satoshi Kuwabara
Graduate School of Medicine
Knoxville | United States

J Neurol Neurosurg Psychiatry 2019 Apr 16. Epub 2019 Apr 16.

Neurology, Chiba University School of Medicine, Chiba, Japan.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy typically characterised by symmetrical involvement, and proximal as well as distal muscle weakness (typical CIDP). However, there are several 'atypical' subtypes, such as multifocal acquired demyelinating sensory and motor neuropathy (Lewis-Sumner syndrome) and 'distal acquired demyelinating symmetric neuropathy', possibly having different immunopathogenesis and treatment responses. In the absence of diagnostic and pathogenetic biomarkers, diagnosis and treatment may be difficult, but recent progress has been made in the application of neuroimaging tools demonstrating nerve hypertrophy and in identifying subgroups of patients who harbour antibodies against nodal proteins such as neurofascin and contactin-1. Despite its relative rarity, CIDP represents a significant economic burden, mostly due to costly treatment with immunoglobulin. Recent studies have demonstrated the efficacy of subcutaneous as well as intravenous immunoglobulin as maintenance therapy, and newer immunomodulating drugs can be used in refractory cases. This review provides an overview focusing on advances over the past several years.

Download full-text PDF

Source
http://dx.doi.org/10.1136/jnnp-2019-320314DOI Listing
April 2019

Publication Analysis

Top Keywords

acquired demyelinating
8
immunopathogenesis treatment
8
inflammatory demyelinating
8
demyelinating polyneuropathy
8
chronic inflammatory
8
hypertrophy identifying
4
nerve hypertrophy
4
neuroimaging tools
4
identifying subgroups
4
demonstrating nerve
4
tools demonstrating
4
patients harbour
4
proteins neurofascin
4
neurofascin contactin-1
4
nodal proteins
4
antibodies nodal
4
application neuroimaging
4
harbour antibodies
4
subgroups patients
4
diagnosis treatment
4

Similar Publications