Abstract
Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous disorder and both clinical course and response to treatment vary widely. Because of the propensity for relapse, CIDP requires maintenance therapy after the initial response to treatment. There is no consensus regarding this in the published literature.
Present report: A patient with CIDP was treated with oral prednisolone and cyclophosphamide pulse therapy but required repeated plasma exchange and intravenous immunoglobulin (IVIg). Treatment with ciclosporin freed the patient from repeated IVIg administration. Therapeutic responses in 14 subsequent cases including three patients who showed improvement with ciclosporin are also presented along with an algorithm of the authors' suggested protocol for treatment.
Conclusion: Ciclosporin should be considered for patients with intractable CIDP who require repeated IVIg.
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Selected References
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- Ahlmén J., Andersen O., Hallgren G., Peilot B. Positive effects of tacrolimus in a case of CIDP. Transplant Proc. 1998 Dec;30(8):4194–4194. doi: 10.1016/s0041-1345(98)01389-x. [DOI] [PubMed] [Google Scholar]
- Barnett M. H., Pollard J. D., Davies L., McLeod J. G. Cyclosporin A in resistant chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 1998 Apr;21(4):454–460. doi: 10.1002/(sici)1097-4598(199804)21:4<454::aid-mus3>3.0.co;2-8. [DOI] [PubMed] [Google Scholar]
- Bosboom W. M., Van den Berg L. H., De Boer L., Van Son M. J., Veldman H., Franssen H., Logtenberg T., Wokke J. H. The diagnostic value of sural nerve T cells in chronic inflammatory demyelinating polyneuropathy. Neurology. 1999 Sep 11;53(4):837–845. doi: 10.1212/wnl.53.4.837. [DOI] [PubMed] [Google Scholar]
- Dyck P. J., Daube J., O'Brien P., Pineda A., Low P. A., Windebank A. J., Swanson C. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. N Engl J Med. 1986 Feb 20;314(8):461–465. doi: 10.1056/NEJM198602203140801. [DOI] [PubMed] [Google Scholar]
- Dyck P. J., Litchy W. J., Kratz K. M., Suarez G. A., Low P. A., Pineda A. A., Windebank A. J., Karnes J. L., O'Brien P. C. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 1994 Dec;36(6):838–845. doi: 10.1002/ana.410360607. [DOI] [PubMed] [Google Scholar]
- Dyck P. J., O'Brien P. C., Oviatt K. F., Dinapoli R. P., Daube J. R., Bartleson J. D., Mokri B., Swift T., Low P. A., Windebank A. J. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol. 1982 Feb;11(2):136–141. doi: 10.1002/ana.410110205. [DOI] [PubMed] [Google Scholar]
- Good J. L., Chehrenama M., Mayer R. F., Koski C. L. Pulse cyclophosphamide therapy in chronic inflammatory demyelinating polyneuropathy. Neurology. 1998 Dec;51(6):1735–1738. doi: 10.1212/wnl.51.6.1735. [DOI] [PubMed] [Google Scholar]
- Gorson K. C., Ropper A. H., Clark B. D., Dew R. B., 3rd, Simovic D., Allam G. Treatment of chronic inflammatory demyelinating polyneuropathy with interferon-alpha 2a. Neurology. 1998 Jan;50(1):84–87. doi: 10.1212/wnl.50.1.84. [DOI] [PubMed] [Google Scholar]
- Hadden R. D., Sharrack B., Bensa S., Soudain S. E., Hughes R. A. Randomized trial of interferon beta-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 1999 Jul 13;53(1):57–61. doi: 10.1212/wnl.53.1.57. [DOI] [PubMed] [Google Scholar]
- Hahn A. F., Bolton C. F., Pillay N., Chalk C., Benstead T., Bril V., Shumak K., Vandervoort M. K., Feasby T. E. Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy. A double-blind, sham-controlled, cross-over study. Brain. 1996 Aug;119(Pt 4):1055–1066. doi: 10.1093/brain/119.4.1055. [DOI] [PubMed] [Google Scholar]
- Hahn A. F., Bolton C. F., Zochodne D., Feasby T. E. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy. A double-blind, placebo-controlled, cross-over study. Brain. 1996 Aug;119(Pt 4):1067–1077. doi: 10.1093/brain/119.4.1067. [DOI] [PubMed] [Google Scholar]
- Hefter H., Sprenger K. B., Arendt G., Hafner D. Treatment of chronic relapsing inflammatory demyelinating polyneuropathy by cyclosporin A and plasma exchange. A case report. J Neurol. 1990 Aug;237(5):320–323. doi: 10.1007/BF00314752. [DOI] [PubMed] [Google Scholar]
- Hess A. D., Tutschka P. J. Effect of cyclosporin A on human lymphocyte responses in vitro. I. CsA allows for the expression of alloantigen-activated suppressor cells while preferentially inhibiting the induction of cytolytic effector lymphocytes in MLR. J Immunol. 1980 Jun;124(6):2601–2608. [PubMed] [Google Scholar]
- Hughes R., Bensa S., Willison H., Van den Bergh P., Comi G., Illa I., Nobile-Orazio E., van Doorn P., Dalakas M., Bojar M. Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001 Aug;50(2):195–201. doi: 10.1002/ana.1088. [DOI] [PubMed] [Google Scholar]
- Kahan B. D. Cyclosporine. N Engl J Med. 1989 Dec 21;321(25):1725–1738. doi: 10.1056/NEJM198912213212507. [DOI] [PubMed] [Google Scholar]
- Katz J. S., Saperstein D. S., Gronseth G., Amato A. A., Barohn R. J. Distal acquired demyelinating symmetric neuropathy. Neurology. 2000 Feb 8;54(3):615–620. doi: 10.1212/wnl.54.3.615. [DOI] [PubMed] [Google Scholar]
- Mahattanakul W., Crawford T. O., Griffin J. W., Goldstein J. M., Cornblath D. R. Treatment of chronic inflammatory demyelinating polyneuropathy with cyclosporin-A. J Neurol Neurosurg Psychiatry. 1996 Feb;60(2):185–187. doi: 10.1136/jnnp.60.2.185. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Mendell J. R., Barohn R. J., Freimer M. L., Kissel J. T., King W., Nagaraja H. N., Rice R., Campbell W. W., Donofrio P. D., Jackson C. E. Randomized controlled trial of IVIg in untreated chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 2001 Feb 27;56(4):445–449. doi: 10.1212/wnl.56.4.445. [DOI] [PubMed] [Google Scholar]
- Mowzoon N., Sussman A., Bradley W. G. Mycophenolate (CellCept) treatment of myasthenia gravis, chronic inflammatory polyneuropathy and inclusion body myositis. J Neurol Sci. 2001 Apr 1;185(2):119–122. doi: 10.1016/s0022-510x(01)00478-6. [DOI] [PubMed] [Google Scholar]
- Oh S. J., Joy J. L., Kuruoglu R. "Chronic sensory demyelinating neuropathy": chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy. J Neurol Neurosurg Psychiatry. 1992 Aug;55(8):677–680. doi: 10.1136/jnnp.55.8.677. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Sabatelli M., Madia F., Mignogna T., Lippi G., Quaranta L., Tonali P. Pure motor chronic inflammatory demyelinating polyneuropathy. J Neurol. 2001 Sep;248(9):772–777. doi: 10.1007/s004150170093. [DOI] [PubMed] [Google Scholar]
- Saperstein D. S., Amato A. A., Wolfe G. I., Katz J. S., Nations S. P., Jackson C. E., Bryan W. W., Burns D. K., Barohn R. J. Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome. Muscle Nerve. 1999 May;22(5):560–566. doi: 10.1002/(sici)1097-4598(199905)22:5<560::aid-mus2>3.0.co;2-q. [DOI] [PubMed] [Google Scholar]
- Saperstein D. S., Katz J. S., Amato A. A., Barohn R. J. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve. 2001 Mar;24(3):311–324. doi: 10.1002/1097-4598(200103)24:3<311::aid-mus1001>3.0.co;2-a. [DOI] [PubMed] [Google Scholar]
- Saxon A., Stevens R. H., Ramer S. J., Clements P. J., Yu D. T. Glucocorticoids administered in vivo inhibit human suppressor T lymphocyte function and diminish B lymphocyte responsiveness in in vitro immunoglobulin synthesis. J Clin Invest. 1978 Apr;61(4):922–930. doi: 10.1172/JCI109017. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Schmidt B., Toyka K. V., Kiefer R., Full J., Hartung H. P., Pollard J. Inflammatory infiltrates in sural nerve biopsies in Guillain-Barre syndrome and chronic inflammatory demyelinating neuropathy. Muscle Nerve. 1996 Apr;19(4):474–487. doi: 10.1002/(SICI)1097-4598(199604)19:4<474::AID-MUS8>3.0.CO;2-9. [DOI] [PubMed] [Google Scholar]
- Thompson N., Choudhary P., Hughes R. A., Quinlivan R. M. A novel trial design to study the effect of intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol. 1996 Mar;243(3):280–285. doi: 10.1007/BF00868527. [DOI] [PubMed] [Google Scholar]
- Uncini A., Sabatelli M., Mignogna T., Lugaresi A., Liguori R., Montagna P. Chronic progressive steroid responsive axonal polyneuropathy: a CIDP vaariant or a primary axonal disorder? Muscle Nerve. 1996 Mar;19(3):365–371. doi: 10.1002/(SICI)1097-4598(199603)19:3<365::AID-MUS14>3.0.CO;2-R. [DOI] [PubMed] [Google Scholar]
- Van den Berg-Vos R. M., Van den Berg L. H., Franssen H., Vermeulen M., Witkamp T. D., Jansen G. H., van Es H. W., Kerkhoff H., Wokke J. H. Multifocal inflammatory demyelinating neuropathy: a distinct clinical entity? Neurology. 2000 Jan 11;54(1):26–32. doi: 10.1212/wnl.54.1.26. [DOI] [PubMed] [Google Scholar]
- Vermeulen M., Van Oers M. H. Successful autologous stem cell transplantation in a patient with chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 2002 Jan;72(1):127–128. doi: 10.1136/jnnp.72.1.127. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Wertman E., Argov Z., Abrmasky O. Chronic inflammatory demyelinating polyradiculoneuropathy: features and prognostic factors with corticosteroid therapy. Eur Neurol. 1988;28(4):199–204. doi: 10.1159/000116266. [DOI] [PubMed] [Google Scholar]
- Yan W. X., Archelos J. J., Hartung H. P., Pollard J. D. P0 protein is a target antigen in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001 Sep;50(3):286–292. doi: 10.1002/ana.1129. [DOI] [PubMed] [Google Scholar]
- van Doorn P. A., Brand A., Strengers P. F., Meulstee J., Vermeulen M. High-dose intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy: a double-blind, placebo-controlled, crossover study. Neurology. 1990 Feb;40(2):209–212. doi: 10.1212/wnl.40.2.209. [DOI] [PubMed] [Google Scholar]
- van Schaik Ivo N., Winer John B., de Haan Rob, Vermeulen Marinus. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review. Lancet Neurol. 2002 Dec;1(8):491–498. doi: 10.1016/s1474-4422(02)00222-3. [DOI] [PubMed] [Google Scholar]