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Orsucci. Neuroimmunol Neuroinflammation 2019;6:3              Neuroimmunology and
               DOI: 10.20517/2347-8659.2019.05                                   Neuroinflammation




               Editorial                                                                     Open Access


               Pulsed intravenous corticosteroids in chronic
               inflammatory demyelinating polyneuropathy: why
               not?


               Daniele Orsucci

               Unit of Neurology, San Luca Hospital, Via Lippi-Francesconi, Lucca 55100, Italy.

               Correspondence to: Dr. Daniele Orsucci, Unit of Neurology, San Luca Hospital, Via Lippi-Francesconi, Lucca 55100, Italy.
               E-mail: orsuccid@gmail.com
               How to cite this article: Orsucci D. Pulsed intravenous corticosteroids in chronic inflammatory demyelinating polyneuropathy: why
               not? Neuroimmunol Neuroinflammation 2019;6:3. http://dx.doi.org/10.20517/2347-8659.2019.05
               Received: 11 Feb 2019     Accepted: 12 Feb 2019      Published: 24 Feb 2019

               Science Editor: Athanassios P. Kyritsis    Copy Editor: Cai-Hong Wang    Production Editor: Huan-Liang Wu


               Chronic inflammatory demyelinating polyneuropathy (CIDP) is a remitting/relapsing and/or chronic
               autoimmune disorder, characterized by symmetrical, sensorimotor neuropathic involvement and a slowly
               progressive onset. There are many clinical variants, suggesting that this disorder may not be an unique
                                       [1]
               entity but rather a spectrum . CIDP diagnostic criteria combine clinical and electrophysiological features.
                                                                            [1]
               Supportive data include increased cerebrospinal fluid (CSF) protein levels .
               Many, but not all, patients may be successfully treated with therapies aimed at arresting immunological
                                                                                      [3]
                                               [2]
               mechanisms, such as corticosteroids  and intravenous immunoglobulins (IVIg) . A systematic review
               concluded that there was no clear short-term difference with IVIg when compared with intravenous
               methylprednisolone and likely no improvement when compared with either oral prednisolone or plasma
                       [4]
                                                              [4]
               exchange . More randomised trials are strongly needed .
               Recently, a multicentre retrospective study compared safety and efficacy of daily prednisolone, pulsed
               dexamethasone, and pulsed intravenous methylprednisolone. Interestingly, corticosteroids led to
                                                                                  [2]
               improvement in 60% of subjects and to clinical remission in 61% of responders . There were no significant
                                                   [2]
               differences in terms of safety and efficacy . A therapeutic protocol with corticosteroids, with IVIg as an
               adjunctive treatment in case corticosteroid treatment was insufficient, could lead to improvement in 90% of
                           [2]
               CIDP patients .

               Some patients may not respond to IVIg. For instance, we follow a 37-year-old male patient diagnosed
               with CIDP at age 7, based on a sensory ataxic phenotype. The diagnosis was supported by typical

                           © The Author(s) 2019. Open Access This article is licensed under a Creative Commons Attribution 4.0
                           International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use,
                sharing, adaptation, distribution and reproduction in any medium or format, for any purpose, even commercially, as long
                as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license,
                and indicate if changes were made.


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