Page 21 - Read Online
P. 21

Page 2 of 2                       Orsucci. Neuroimmunol Neuroinflammation 2019;6:3  I  http://dx.doi.org/10.20517/2347-8659.2019.05


               electrophysiological features, CSF analysis and sural nerve biopsy. Molecular studies for hereditary
               neuropathies were unremarkable. At age 33 he had a severe relapse leading to a subacute flaccid, areflexic
               tetraparesis unresponsive to IVIg. He was then successfully treated with plasma exchange and intravenous
               corticosteroids. Subsequently he became corticosteroid-dependent needing chronic treatment with oral
               prednisone (25 mg every other day). Unfortunately, he developed bilateral cataract, right hip osteonecrosis
               and cushingoid appearance. Therefore, we switched the treatment to pulsed intravenous methylprednisolone
               (1 g daily for three consecutive days every two months). After one year of this schedule, the neuropathy is
               excellently controlled (apart from mild distal leg weakness) and corticosteroid toxicity is minimized, with
               improvement of hip osteonecrosis and regression of the cushingoid features.


               In conclusion, corticosteroids are cheaper, easier to use, and much more widely available than IVIg. They
                                                                              [2]
               have been suggested to lead to long-term remission more often than IVIg . Furthermore, even if there are
               no significant differences in response and remission rate between these two regimens, pulsed intravenous
                                                                                        [2]
               corticosteroids have lower rates of serious adverse effects than long-term daily use . Therefore, in our
               opinion pulsed intravenous methylprednisolone should be considered in CIDP patients, especially in non-
               responders to IVIg. In fact, it may represent the therapy of choice in these patients.


               DECLARATIONS
               Authors’ contributions
               Made substantial contributions to conception and design of the study and performed data analysis and
               interpretation: Orsucci D


               Availability of data and materials
               Not applicable.

               Financial support and sponsorship
               None.

               Conflicts of interest
               The author declared that there are no conflicts of interest.

               Ethical approval and consent to participate
               Not applicable.

               Consent for publication
               Not applicable.


               Copyright
               © The Author(s) 2019.


               REFERENCES
               1.   Mathey EK, Park SB, Hughes RA, Pollard JD, Armati PJ, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from
                   pathology to phenotype. J Neurol Neurosurg Psychiatry 2015;86:973-85.
               2.   van Lieverloo GGA, Peric S, Doneddu PE, Gallia F, Nikolic A, et al. Corticosteroids in chronic inflammatory demyelinating
                   polyneuropathy: a retrospective, multicentre study, comparing efficacy and safety of daily prednisolone, pulsed dexamethasone, and
                   pulsed intravenous methylprednisolone. J Neurol 2018;265:2052-9.
               3.   Kuwabara S, Mori M, Misawa S, Suzuki M, Nishiyama K, et al. Intravenous immunoglobulin for maintenance treatment of chronic
                   inflammatory demyelinating polyneuropathy: a multicentre, open-label, 52-week phase III trial. J Neurol Neurosurg Psychiatry
                   2017;88:832-8.
               4.   Oaklander AL, Lunn MP, Hughes RA, van Schaik IN, Frost C, et al. Treatments for chronic inflammatory demyelinating
                   polyradiculoneuropathy (CIDP): an overview of systematic reviews. Cochrane Database Syst Rev 2017;1:CD010369.
   16   17   18   19   20   21   22   23   24   25   26