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Autonomic nervous system involvement in chronic inflammatory demyelinating polyradiculoneuropathy: a literature review

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Abstract

Background and aims

Although dysautonomia is a well-recognized complication of acute demyelinating polyradiculoneuropathy, it is rarely reported and evaluated in chronic demyelinating neuropathies. The purpose of this review is to search and synthesize the current literature on the prevalence and type of autonomic dysfunction (AD) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Methods

PubMed and Web of Science were searched for studies reporting AD in CIDP.

Results

Twelve studies, including 346 patients with CIDP, were found eligible for the review. Seven studies used autonomic tests only as an additional component of the comprehensive clinical evaluation, and found that dysautonomia in CIDP may indicate the presence of a comorbid disease (e.g., diabetes) and facilitate the differentiation of CIDP from other neuropathies (e.g., amyloid neuropathy). Five studies performed quantitative assessment of autonomic function in CIDP as a primary goal. Two studies have used the Composite Autonomic Severity Score (CASS) to assess severity and distribution of dysautonomia. The reported prevalence of dysautonomia in CIDP during quantitative assessment of autonomic function ranged from 25 to 89%, depending on the battery of tests used, with CASS not exceeding 4 points. The abnormalities in autonomic tests indicated both sympathetic and parasympathetic dysfunction and did not correlate with the duration, severity and variant of CIDP.

Conclusions

Clinical or subclinical involvement of the ANS has been shown to be common and relatively mild in CIDP. The impact of autonomic impairment on disability and of its possible response to therapy in CIDP needs to be further investigated.

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Data Availability

The datasets generated during and/or analyzed during the current review are available from the corresponding author on reasonable request.

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Authors and Affiliations

  1. Department of Neurology, 10th Military Research Hospital and Polyclinic, Bydgoszcz, Poland

    Łukasz Rzepiński

  2. Sanitas-Neurology Outpatient Clinic, Bydgoszcz, Poland

    Łukasz Rzepiński

  3. Neuromuscular Diseases and Neuroimmunology Service, IRCCS Humanitas Clinical and Research Institute, Rozzano, Italy

    Pietro Emiliano Doneddu, Claudia Cutellè & Eduardo Nobile-Orazio

  4. Department of Biomedical Sciences, Humanitas University, Milan, Italy

    Pietro Emiliano Doneddu

  5. Department of Human Physiology, Nicolaus Copernicus University Ludwik Rydygier Collegium Medicum in Bydgoszcz, Bydgoszcz, Poland

    Monika Zawadka-Kunikowska

  6. Department of Medical Biotechnology and Translational Medicine, Milan University, Milan, Italy

    Eduardo Nobile-Orazio

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  1. Łukasz Rzepiński
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  2. Pietro Emiliano Doneddu
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Contributions

Łukasz Rzepiński: Developed the methodology; database search; analyzed and interpreted the data, wrote the first draft; read and agreed to the final version of the manuscript. Pietro Emiliano Doneddu: Conceived the study; developed the methodology, critically revised successive drafts of the manuscript; supervised the project; read and agreed to the final version of the manuscript. Claudia Cutellè: critically revised successive drafts of the manuscript; read and agreed to the final version of the manuscript. Monika Zawadka-Kunikowska: Developed the methodology; database search; analysed and interpreted the data; read and agreed to the final version of the manuscript. Eduardo Nobile-Orazio: Conceived the study; developed the methodology, critically revised successive drafts of the manuscript; supervised the project; read and agreed to the final version of the manuscript.

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Correspondence to Łukasz Rzepiński.

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Conflict of interest

Pietro Emiliano Doneddu has received travel grants to attend scientific meetings from CSL Behring and Kedrion. Eduardo Nobile Orazio reports personal fees for Advisory or Scientific Board from Argenx, Belgium; Takeda, USA; CSL Behring, Switzerland; Janssen, USA; Kedrion, Italy; LFB, France; Roche, Switzerland and Honorarium for Lectures or travel Grants from Takeda, USA; CSL Behring, Italy; Kedrion, Italy.

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Rzepiński, Ł., Doneddu, P.E., Cutellè, C. et al. Autonomic nervous system involvement in chronic inflammatory demyelinating polyradiculoneuropathy: a literature review. Neurol Sci 44, 3071–3082 (2023). https://doi.org/10.1007/s10072-023-06802-z

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