Page 947 - Clinical Immunology_ Principles and Practice ( PDFDrive )
P. 947

914          Part Seven  Organ-Specific Inflammatory Disease



        MULTIFOCAL MOTOR NEUROPATHY                            Please check your eBook at https://expertconsult.inkling.com/
                                                               for self-assessment questions. See inside cover for registration
        MMN responds very well only to IVIG, which is the treatment   details.
        of choice based on controlled trials. In difficult cases, cyclophos-
        phamide or rituximab may be promising, but no controlled   REFERENCES
        studies have been carried out. 2,3,21
                                                                1.  Yuki N, Hartung H-P. Guillain-Barre syndrome. N Engl J Med
        Paraneoplastic Anti-Hu Neuropathy                         2012;366:2294–304.
        Anecdotally, some of these patients have responded to plasma   2.  Dalakas MC. Pathophysiology of autoimmune polyneuropathies. Presse
        exchange or IVIG, but overall this neuropathy has not been   Med 2013;42(6 Pt 2):e181–92.
        consistently responsive to available therapies.         3.  Dalakas MC. Pathogenesis of autoimmune neuropathies. Biochim
                                                                  Biophys Acta 2015;1852:658–66.
        Vasculitic Neuropathies                                 4.  Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet
                                                                  2016 Feb 29. pii: S0140-6736(16).
        For isolated peripheral nerve vasculitis, a combination of pred-  5.  Kuwabara S, Yuki N. Axonal Guillain-Barre syndrome: concepts and
        nisone 1.5 mg/kg/day with cyclophosphamide 2 mg/kg/day orally,   controversies. Lancet Neurol 2013; 12; 1180-8.
               2
        or 1 g/m  intravenously monthly for 6 months, are the treatments   6.  Dalakas MC, Quarles RH, editors. Autoimmune ataxic neuropathies
        of choice; specific treatment protocol may vary from patient to   (sensory ganglionopathies): are glycolipids the responsible autoantigens?
        patient. The administration of cyclophosphamide may not be   Ann Neurol 1996;39:419–22.
        necessary for >6 months, as in systemic vasculitis. Plasmapheresis   7.  Illa I, Ortiz N, Gallard E, et al. Acute axonal Guillain–Barré syndrome
        has been tried in cryoglobulinemic neuropathies, with variable   with IgG antibodies against motor axons following parenteral
        results.                                                  gangliosides. Ann Neurol 1995;38:218–24.
                                                                8.  Cao-Lormeau V-M, et al. Guillain-Barre Syndrome outbreak associated
        Human Immunodeficiency Virus Neuropathies                 with Zika virus infection in French Polynesia: a case controlled study.
                                                                  Lancet 2016;387(10027):1531–9.
        The two demyelinating neuropathies, GBS and CIDP, are treated   9.  Uncini A, Kuwabara S. Nodopathies of the peripheral nerve: an emerging
        with the same immunomodulatory therapies as used in HIV-  concept. J Neurol Neurosurg Psychiatry 2015;86:1186–95.
        negative patients with IVIG being the preferable choice. Ganci-  10.  Stathopoulos P, Alexopoulos H, Dalakas MC. Autoimmune antigenic
        clovir may be helpful in CMV-related polyradiculoneuropathy.   targets at the node of Ranvier in demyelinating disorders. Nat Rev Neurol
        Painful sensory neuropathy can be quite disabling because of   2015;11:143–56.
        intractable pain. Tricyclic antidepressants (TCAs), nonsteroidal   11.  Dalakas MC. Advances in the diagnosis, pathogenesis and treatment of
        antiinflammatory drugs (NSAIDs), anticonvulsants (carbam-  CIDP. Nat Rev Neurol 2011;7(9):507–17.
        azepine, gabapentin, topiramate), and topical capsaicin in various   12.  Koller H, Kieseier BC, Jander S, et al. Chronic inflammatory
        combinations provide some relief from neuropathic pain.   demyelinating polyneuropathy. N Engl J Med 2005;352:1343–56.
                                                               13.  Schneider-Hohendorf T, Schwab N, Uceyler K, et al. T cell immunity in
                                                                  CIDP. Neurology 2012;78:402–8.
                                                               14.  Murata K, Dalakas MC. Expression of the co-stimulatory molecule BB-1,
                                                                  the ligands CTLA-4 and CD28 and their mRNAs in chronic inflammatory
            On tHe HOrIZOn                                        demyelinating polyneuropathy. Brain 2000;123:1660–6.
                                                               15.  Salomon B, Rhee L, Bour-Jordan H, et al. Development of spontaneous
          •  Identification of common causes that break tolerance, either in con-
           nection with the gut microbiome or exogenous agents, and expansion   autoimmune peripheral polyneuropathy in B7-2-deficient NOD mice.
           of the current limited data on molecular mimicry between nerve   J Exp Med 2001;194:677–84.
           glycoproteins and viral or bacterial antigens       16.  Dalakas M, Engel WK. Immunoglobulin deposits in chronic relapsing
          •  Development of neuroimaging capabilities to image peripheral nerves   polyneuropathies. Arch Neurol 1980;37:637–40.
           and dorsal roots in vivo and quantify inflammation, demyelination and   17.  Devaux JJ, Miura Y, Fukami Y, et al. Neurofascin-155 IgG4 in chronic
           axonal degeneration, providing an accessible and reliable tool for   inflammatory demyelinating polyneuropathy. Neurology 2016;86:800–7.
           diagnosis and monitoring response to therapies, in a model similar to   18.  Miura Y, Devaux JJ, Fukami Y, et al. Contactin 1 IgG4 associates to
           magnetic resonance imaging (MRI) in multiple sclerosis  chronic inflammatory demyelinating polyneuropathy with sensory ataxia.
          •  Performance of proteome studies aimed to identify biomarkers of   Brain 2015;138:1–8.
           nerve autoimmunity that may lead to specific therapies  19.  Dalakas MC, Gooch C. Close to the node but still far: What antibodies
          •  Trials with target-specific therapies for acute and chronic demyelinating   tell us about CIDP and its therapies. Neurology 2016;86:796–7.
           neuropathies, applied in combination with the existing therapies, target-  20.  Weiss MD, Luciano CA, Semino-Mora C, et al. Molecular mimicry in
           ing early in the disease process complement activation, regulatory T   chronic inflammatory demyelinating polyneuropathy and melanoma.
           cell (Treg) functions, key proinflammatory cytokines, and B cells  Neurology 1998;51:1738–41.
          •  Identification of agents and trophic factors that could prevent axonal   21.  Van Asseldonk JT, Franssen H, Van den Berg-Vos RM, et al. Multifocal
           degeneration from the outset, promote remyelination, prevent axonal   motor neuropathy. Lancet Neurol 2005;4:309–19.
           loss, or trigger axonal regeneration and reverse, early in the disease   22.  Kyle RA, Therneau TM, Rajkumar SV, et al. Prevalence of monoclonal
           process, a seemingly “permanent” clinical deficit
                                                                  gammopathy of undetermined significance. N Engl J Med
                                                                  2006;354:1362–9.
                                                               23.  Kelly JJ, Kyle RA, O’Brien PC, et al. The prevalence of monoclonal
                                                                  gammopathy in peripheral neuropathy. Neurology 1981;31:1480–3.
        ACKNOWLEDGMENTS                                        24.  Latov N, Hays A, Sherman WH. Peripheral neuropathy and anti-MAG
                                                                  antibodies. Crit Rev Neurobiol 1988;3:301–32.
        The author thanks the various Neurology Fellows who provided   25.  Dalakas MC. Pathogenesis and treatment of anti-MAG neuropathy. Curr
        excellent care to his patients; and Drs. Amjad Ilyas and Richard   Treat Options Neurol 2010;12:71–83.
        Quarles for help with immunochemistry of MAG and SGPG   26.  Dalakas MC, Engel WK. Polyneuropathy and monoclonal gammopathy:
        and gangliosides.                                         studies of 11 patients. Ann Neurol 1981;10:45–52.
   942   943   944   945   946   947   948   949   950   951   952