Universität zu Köln

Lehmann, Helmar Christoph, Burke, David and Kuwabara, Satoshi ORCID: 0000-0002-4716-8578 (2019). Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment. J. Neurol. Neurosurg. Psychiatry, 90 (9). S. 981 - 988. LONDON: BMJ PUBLISHING GROUP. ISSN 1468-330X

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Abstract

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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Lehmann, Helmar Christoph UNSPECIFIED UNSPECIFIED UNSPECIFIED Burke, David UNSPECIFIED UNSPECIFIED UNSPECIFIED Kuwabara, Satoshi UNSPECIFIED orcid.org/0000-0002-4716-8578 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-142174
DOI:	10.1136/jnnp-2019-320314
Journal or Publication Title:	J. Neurol. Neurosurg. Psychiatry
Volume:	90
Number:	9
Page Range:	S. 981 - 988
Date:	2019
Publisher:	BMJ PUBLISHING GROUP
Place of Publication:	LONDON
ISSN:	1468-330X
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language TISSUE RAPID ACQUISITION; SUBCUTANEOUS IMMUNOGLOBULIN; MAINTENANCE TREATMENT; IGG4 ANTIBODIES; PLASMA-EXCHANGE; MR NEUROGRAPHY; POEMS SYNDROME; DOUBLE-BLIND; NEUROFASCIN; POLYRADICULONEUROPATHY Multiple languages Clinical Neurology; Psychiatry; Surgery Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/14217