Chronic Inflammatory Demyelinating Polyradiculoneuropathy Questions & Answers

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. [QxMD MEDLINE Link].

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. [QxMD MEDLINE Link].

3. Oaklander AL, Lunn MP, Hughes RA, van Schaik IN, Frost C, Chalk CH. Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews. Cochrane Database Syst Rev. 2017 Jan 13. 1:CD010369. [QxMD MEDLINE Link].

4. 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. [QxMD MEDLINE Link].

5. 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. [QxMD MEDLINE Link].

6. 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. [QxMD MEDLINE Link].

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

8. 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. [QxMD MEDLINE Link].

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

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

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

12. 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. [QxMD MEDLINE Link].

13. Chaudhry V. Multifocal motor neuropathy. Semin Neurol. 1998. 18(1):73-81. [QxMD MEDLINE Link].

14. 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. [QxMD MEDLINE Link].

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

16. 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. [QxMD MEDLINE Link].

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

18. 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. [QxMD MEDLINE Link].

19. 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. [QxMD MEDLINE Link].

20. 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. [QxMD MEDLINE Link].

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

22. 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. [QxMD MEDLINE Link].

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

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

25. 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. [QxMD MEDLINE Link].

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

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

28. 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. [QxMD MEDLINE Link]. [Full Text].

29. 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. [QxMD MEDLINE Link].

30. 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. [QxMD MEDLINE Link].

31. 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. [QxMD MEDLINE Link].

32. 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. [QxMD MEDLINE Link].

33. 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. [QxMD MEDLINE Link].

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

35. 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. [QxMD MEDLINE Link].

36. 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. [QxMD MEDLINE Link].

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

38. van Schaik IN, van Geloven N, Bril V, Hartung HP, Lewis RA, Sobue G, et al. Subcutaneous immunoglobulin for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (The PATH Study): study protocol for a randomized controlled trial. Trials. 2016 Jul 25. 17 (1):345. [QxMD MEDLINE Link]. [Full Text].

39. Léger JM, De Bleecker JL, Sommer C, Robberecht W, Saarela M, Kamienowski J, et al. Efficacy and safety of Privigen(®) in patients with chronic inflammatory demyelinating polyneuropathy: results of a prospective, single-arm, open-label Phase III study (the PRIMA study). J Peripher Nerv Syst. 2013 Jun. 18 (2):130-40. [QxMD MEDLINE Link]. [Full Text].

40. van Schaik IN, et al. Subcutaneous immunoglobulin for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (PATH): a randomised, double-blind, placebo-controlled, phase 3 trial. The Lancet Neurology. 2018. 17 (1):35-46. [Full Text].

Richard A Lewis, MD Professor of Neurology, Director of Electromyography Laboratory, Cedars-Sinai Medical Center

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

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.

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, MA, MS Chair, Neuroscience Institute and Department of Neurology, Director, Hereditary Neuropathy Center, Co-Director, Center for Memory Loss and Brain Health, Co-Director, ALS Center, Hackensack University Medical Center; Associate Dean of Faculty, Founding Chair and Professor, Department of Neurology, Hackensack Meridian School of Medicine

Florian P Thomas, MD, PhD, 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, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Nicholas Lorenzo, MD, CPE, MHCM, FAAPL Co-Founder and Former Chief Publishing Officer, eMedicine and eMedicine Health, Founding Editor-in-Chief, eMedicine Neurology; Founder and Former Chairman and CEO, Pearlsreview; Founder and CEO/CMO, PHLT Consultants; Former Chief Medical Officer, MeMD Inc

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

Disclosure: Nothing to disclose.

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: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Association of Neuromuscular and Electrodiagnostic Medicine<br/>Received research grant from: Alnylam; Pfizer; Ionis; Cytokinetics; Momenta; Viela Bio; .

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.

• Print this section
• Print the entire contents of
• Print the entire contents of article