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Review
. 2020 Dec 8:2:42.
doi: 10.1186/s42466-020-00088-8. eCollection 2020.

Comprehensive approaches for diagnosis, monitoring and treatment of chronic inflammatory demyelinating polyneuropathy

Anna Lena Fisse #  1   2 , Jeremias Motte #  1   2 , Thomas Grüter  1   2 , Melissa Sgodzai  1   2 , Kalliopi Pitarokoili  1   2 , Ralf Gold  1   2
Affiliations
Review

Comprehensive approaches for diagnosis, monitoring and treatment of chronic inflammatory demyelinating polyneuropathy

Anna Lena Fisse et al. Neurol Res Pract. .

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common chronic inflammatory neuropathy. CIDP is diagnosed according to the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria, which combine clinical features with the electrophysiological evidence of demyelination. However, firstly, diagnosis is challenging, as some patients e.g. with severe early axonal damage do not fulfil the criteria. Secondly, objective and reliable tools to monitor the disease course are lacking. Thirdly, about 25% of CIDP patients do not respond to evidence-based first-line therapy. Recognition of these patients is difficult and treatment beyond first-line therapy is based on observational studies and case series only. Individualized immunomodulatory treatment does not exist due to the lack of understanding of essential aspects of the underlying pathophysiology. Novel diagnostic imaging techniques and molecular approaches can help to solve these problems but do not find enough implementation. This review gives a comprehensive overview of novel diagnostic techniques and monitoring approaches for CIDP and how these can lead to individualized treatment and better understanding of pathophysiology.

Keywords: Biobank; Chronic inflammatory demyelinating polyneuropathy; Diagnosis; Imaging; Inflammatory neuropathies; Monitoring; Pathophysiology; Register; Treatment.

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Conflict of interest statement

Competing interestsThe authors declare that they have no competing interest.

Figures

Fig. 1
Fig. 1
Overview of inflammatory neuropathies with focus on CIDP, subtypes and distinct disease entities
Fig. 2
Fig. 2
a Normal median nerve at the middle of the forearm between the flexor digitorum profundus and superficialis muscles with a normal CSA of 6.95 mm2 and normal fascicular structure. b Significantly enlarged median nerve at the forearm with a CSA of 31.5 mm2 in a patient with CIDP. Some swollen fascicles and a hypoechoic structure can be depicted. Ultrasound stetting except focus are the same in both images
Fig. 3
Fig. 3
Corneal confocal microscopy showing nerve fiber reduction and immune cell infiltration in a CIDP patient with progressive disease course (b) compared to a healthy person (a). (With the kind permission of Professor Martin Tegenthoff, Bochum, and Dr. Dietrich Sturm, Wuppertal, 2020)
Fig. 4
Fig. 4
Overview of comprehensive approaches for diagnosis, monitoring and treatment of CIDP
See this image and copyright information in PMC

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