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Chronic Inflammatory Demyelinating Polyradiculoneuropathy Treatment & Management

  • Author: Richard A Lewis, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
 
Updated: May 06, 2016
 

Medical Care

Untreated, chronic inflammatory demyelinating polyradiculoneuropathy is characterized by accumulating disability that requires physical and occupational therapy, orthotic devices, and long-term treatment. Close follow-up care with a physician knowledgeable in the field is necessary to adjust treatment.

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Surgical Care

Surgical and orthopedic consultation may be required for sural nerve biopsy or in severe disease with joint deformities that require corrective surgery.

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Consultations

See the list below:

  • Consult a neurologist for evaluation, diagnosis, and treatment of CIDP and possible associated diseases.
  • Consult a physical medicine and rehabilitation specialist for physical and occupational therapy and evaluation for orthotic devices.
  • Other consultations (eg, internist, hematologist, oncologist, rheumatologist) may be necessary if associated diseases are present. Some treatments may be best accomplished with the assistance of a hematologist or rheumatologist.
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Diet

No specific diet has been proven beneficial in CIDP.

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Activity

Activity generally depends on the level of disability. Encourage physical therapy and an active lifestyle.

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Contributor Information and Disclosures
Author

Richard A Lewis, MD Professor and Associate Chairman of Neurology, Department of Neurology, Wayne State University School of Medicine

Richard A Lewis, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, Peripheral Nerve Society, American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Received grant/research funds from GBS/CIDP FI for other; Received consulting fee from Baxter for consulting; Received grant/research funds from Baxter for none; Received consulting fee from CSL Behring for consulting.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Florian P Thomas, MD, PhD, Drmed, MA, MS Director, National MS Society Multiple Sclerosis Center; Professor and Director, Clinical Research Unit, Department of Neurology, Adjunct Professor of Physical Therapy, Associate Professor, Institute for Molecular Virology, St Louis University School of Medicine; Editor-in-Chief, Journal of Spinal Cord Medicine

Florian P Thomas, MD, PhD, Drmed, MA, MS is a member of the following medical societies: Academy of Spinal Cord Injury Professionals, American Academy of Neurology, American Neurological Association, Consortium of Multiple Sclerosis Centers, National Multiple Sclerosis Society, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Nicholas Lorenzo, MD, MHA, CPE Founding Editor-in-Chief, eMedicine Neurology; Founder and CEO/CMO, PHLT Consultants; Chief Medical Officer, MeMD Inc

Nicholas Lorenzo, MD, MHA, CPE is a member of the following medical societies: Alpha Omega Alpha, American Association for Physician Leadership, American Academy of Neurology

Disclosure: Nothing to disclose.

Additional Contributors

Dianna Quan, MD Professor of Neurology, Director of Electromyography Laboratory, University of Colorado School of Medicine

Dianna Quan, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Neurological Association

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Marina Zvartau-Hind, MD, PhD, to the development and writing of this article.

References
  1. Rajabally YA, Simpson BS, Beri S, Bankart J, Gosalakkal JA. Epidemiologic variability of chronic inflammatory demyelinating polyneuropathy with different diagnostic criteria: study of a UK population. Muscle Nerve. 2009 Apr. 39(4):432-8. [Medline].

  2. Press R, Hiew FL, Rajabally YA. Steroids for chronic inflammatory demyelinating polyradiculoneuropathy: evidence base and clinical practice. Acta Neurol Scand. 2016 Apr. 133 (4):228-38. [Medline].

  3. Hughes RA, Donofrio P, Bril V, et al. Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo-controlled trial. Lancet Neurol. 2008 Feb. 7(2):136-44. [Medline].

  4. Gorson KC, van Schaik IN, Merkies IS, et al. Chronic inflammatory demyelinating polyneuropathy disease activity status: recommendations for clinical research standards and use in clinical practice. J Peripher Nerv Syst. 2010 Dec. 15(4):326-33. [Medline].

  5. Eftimov F, Vermeulen M, van Doorn PA, Brusse E, van Schaik IN. Long-term remission of CIDP after pulsed dexamethasone or short-term prednisolone treatment. Neurology. 2012 Apr 3. 78(14):1079-84. [Medline].

  6. Barnett MH, Pollard JD, Davies L, McLeod JG. Cyclosporin A in resistant chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 1998 Apr. 21(4):454-60. [Medline].

  7. Barohn RJ, Kissel JT, Warmolts JR, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol. 1989 Aug. 46(8):878-84. [Medline].

  8. Barohn RJ, Saperstein DS. Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy. Semin Neurol. 1998. 18(1):49-61. [Medline].

  9. Bouchard C, Lacroix C, Plante V, et al. Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology. 1999 Feb. 52(3):498-503. [Medline].

  10. Bromberg MB. Comparison of electrodiagnostic criteria for primary demyelination in chronic polyneuropathy. Muscle Nerve. 1991 Oct. 14(10):968-76. [Medline].

  11. Chassande B, Leger JM, Younes-Chennoufi AB, et al. Peripheral neuropathy associated with IgM monoclonal gammopathy: correlations between M-protein antibody activity and clinical/electrophysiological features in 40 cases. Muscle Nerve. 1998 Jan. 21(1):55-62. [Medline].

  12. Chaudhry V. Multifocal motor neuropathy. Semin Neurol. 1998. 18(1):73-81. [Medline].

  13. Dalakas MC, Quarles RH, Farrer RG, et al. A controlled study of intravenous immunoglobulin in demyelinating neuropathy with IgM gammopathy. Ann Neurol. 1996 Nov. 40(5):792-5. [Medline].

  14. Donofrio PD. Chronic inflammatory demyelinating polyradiculoneuropathy: new views and guidelines. J Peripher Nerv Syst. 2005 Sep. 10(3):217-9. [Medline].

  15. Dyck PJ, Daube J, O'Brien P, et al. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. N Engl J Med. 1986 Feb 20. 314(8):461-5. [Medline].

  16. Dyck PJ, Lais AC, Ohta M, Bastron JA, Okazaki H, Groover RV. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc. 1975 Nov. 50(11):621-37. [Medline].

  17. Dyck PJ, Litchy WJ, Kratz KM, et al. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 1994 Dec. 36(6):838-45. [Medline].

  18. Erdmann PG, van Meeteren NL, Kalmijn S, Wokke JH, Helders PJ, van den Berg LH. Functional health status of patients with chronic inflammatory neuropathies. J Peripher Nerv Syst. 2005 Jun. 10(2):181-9. [Medline].

  19. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. J Peripher Nerv Syst. 2005 Sep. 10(3):220-8. [Medline].

  20. Good JL, Chehrenama M, Mayer RF, Koski CL. Pulse cyclophosphamide therapy in chronic inflammatory demyelinating polyneuropathy. Neurology. 1998 Dec. 51(6):1735-8. [Medline].

  21. Gorson KC, Allam G, Ropper AH. Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology. 1997 Feb. 48(2):321-8. [Medline].

  22. Hadden RD, Hughes RA. Treatment of immune-mediated inflammatory neuropathies. Curr Opin Neurol. 1999 Oct. 12(5):573-9. [Medline].

  23. Hobson-Webb LD, Donofrio PD. Inflammatory neuropathies: an update on evaluation and treatment. Curr Rheumatol Rep. 2005 Oct. 7(5):348-55. [Medline].

  24. Jann S, Beretta S, Bramerio MA. Different types of chronic inflammatory demyelinating polyneuropathy have a different clinical course and response to treatment. Muscle Nerve. 2005 Sep. 32(3):351-6. [Medline].

  25. Korinthenberg R. Chronic inflammatory demyelinating polyradiculoneuropathy in children and their response to treatment. Neuropediatrics. 1999 Aug. 30(4):190-6. [Medline].

  26. Léger JM, Behin A. Multifocal motor neuropathy. Curr Opin Neurol. 2005 Oct. 18(5):567-73. [Medline].

  27. Mahattanakul W, Crawford TO, Griffin JW, Goldstein JM, Cornblath DR. Treatment of chronic inflammatory demyelinating polyneuropathy with cyclosporin-A. J Neurol Neurosurg Psychiatry. 1996 Feb. 60(2):185-7. [Medline]. [Full Text].

  28. Markvardsen LH, Debost JC, Harbo T, Sindrup SH, Andersen H, Christiansen I, et al. Subcutaneous immunoglobulin in responders to intravenous therapy with chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol. 2013 Jan 7. [Medline].

  29. Mendell JR, Barohn RJ, Freimer ML, et al. Randomized controlled trial of IVIg in untreated chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 2001 Feb 27. 56(4):445-9. [Medline].

  30. Notermans NC, Franssen H, Eurelings M, Van der Graaf Y, Wokke JH. Diagnostic criteria for demyelinating polyneuropathy associated with monoclonal gammopathy. Muscle Nerve. 2000 Jan. 23(1):73-9. [Medline].

  31. Rotta FT, Sussman AT, Bradley WG, Ram Ayyar D, Sharma KR, Shebert RT. The spectrum of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2000 Feb 15. 173(2):129-39. [Medline].

  32. Saperstein DS, Amato AA, Wolfe GI, et al. Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome. Muscle Nerve. 1999 May. 22(5):560-6. [Medline].

  33. Saperstein DS, Katz JS, Amato AA, Barohn RJ. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve. 2001 Mar. 24(3):311-24. [Medline].

  34. Stamboulis E, Katsaros N, Koutsis G, Iakovidou H, Giannakopoulou A, Simintzi I. Clinical and subclinical autonomic dysfunction in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 2006 Jan. 33(1):78-84. [Medline].

  35. Uncini A, De Angelis MV, Di Muzio A, et al. Chronic inflammatory demyelinating polyneuropathy in diabetics: motor conductions are important in the differential diagnosis with diabetic polyneuropathy. Clin Neurophysiol. 1999 Apr. 110(4):705-11. [Medline].

  36. Visudtibhan A, Chiemchanya S, Visudhiphan P. Cyclosporine in chronic inflammatory demyelinating polyradiculoneuropathy. Pediatr Neurol. 2005 Nov. 33(5):368-72. [Medline].

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Electromyography of a patient with chronic inflammatory demyelinating polyradiculoneuropathy illustrating conduction block, temporal dispersion of compound muscle action potential, prolonged distal latencies, and slowed conduction.
Prolonged F wave latencies (normal is < 31).
Electron micrograph of the peripheral nerve of a patient with chronic inflammatory demyelinating polyradiculoneuropathy. Note "onion bulb" formation in the myelin sheath of the nerve fibers due to continuous demyelination and remyelination. Courtesy of A. Sima, MD, Department of Pathology, Wayne State University.
 
 
 
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