Medscape is available in 5 Language Editions – Choose your Edition here.


Chronic Inflammatory Demyelinating Polyradiculoneuropathy Clinical Presentation

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


Chronic inflammatory demyelinating polyradiculoneuropathy most frequently starts insidiously and evolves slowly, either in a slowly progressive (more than 60% of patients) or relapsing manner (approximately one third of patients), with partial or complete recovery between recurrences.

  • Periods of worsening and improvement usually last weeks or months. Patients with a younger age of onset are said to have a higher frequency of relapsing course.
  • Because of the insidious onset of CIDP, documenting precipitating illnesses or events is very difficult. However, preceding infection has been reported infrequently. Both respiratory and gastrointestinal infections have been cited, but no causative organism has been identified.
  • Initial symptoms include weakness of the limbs, both proximal and distal, with proximal muscles affected at least as severely as distal.
  • Sensory symptoms are common, such as tingling and numbness of hands and feet, but usually motor symptoms predominate.
  • Only a small proportion of patients (approximately 16%) have a relatively acute or subacute onset of symptoms, with subsequent steadily progressive or fluctuating course.
  • Children usually have a more precipitous onset of symptoms.
  • Most experts consider the necessary duration of symptoms to be greater than 8 weeks for the diagnosis of CIDP to be made.
  • Autonomic system dysfunction can occur; in such a case, the patient would complain of orthostatic dizziness, problems with bowel and bladder functions, and cardiac problems.


Pertinent findings are limited to the nervous system, except for cases of CADP associated with other diseases, as already mentioned. Depending on the associated systemic disorder, abnormalities on physical examination may be found in multiple organ systems. Patients should be examined in detail for signs of autoimmune, inflammatory, and neoplastic conditions.

  • Cranial nerves
    • Cranial nerves may be involved, particularly CN VII, with paralysis of both upper and lower facial muscles. Diplopia can occur with the involvement of CN III, IV, or VI. Rarely, bulbar muscles (eg, palate, tongue) can be affected.
    • Papilledema with pseudotumor cerebri syndrome (eg, headaches, transient visual obscurations, pulsatile tinnitus, visual field defects) are observed rarely in patients with CIDP and are due to a very high CSF protein level (usually >1000 mg/mL).
  • Gait frequently is abnormal.
  • Type of gait depends on location of weakness and degree of proprioceptive loss. With significant weakness in the lower extremities, patients may walk with steppage (ie, high elevation of both feet to compensate for weakness of foot dorsiflexors) or a slapping gait (ie, due to deficit of proprioception in the feet).
  • Children are said to have more profound gait abnormalities than adults.
  • Motor system
    • Usually relatively symmetric weakness of both proximal and distal muscles is present in upper and lower extremities.
    • Muscle tone can be normal or decreased. Hypotonia, atrophy, and fasciculations may be present.
  • Deep tendon reflexes: Reflexes characteristically are diminished or absent even in regions with only mild weakness.
  • Sensory system
    • Large-diameter, heavily myelinated fibers are affected most severely, leading to proprioceptive and vibratory deficits.
    • Loss or decrease of pain (ie, pinprick) and temperature sensations is less common.
    • Stocking-glove distribution of sensory deficits is typical.
    • Neuropathic pain in affected extremities may occur.
  • Coordination: Patients may have sensory ataxia with positive Romberg sign due to damage to the large nerve fibers that convey proprioception.
  • Pathological reflexes: Pathological reflexes (eg, Babinski, Chaddock, Oppenheim) usually are not observed in patients with CIDP.


Chronic inflammatory demyelinating polyradiculoneuropathy is most frequently an idiopathic illness, but it has been known to occur with several conditions. In those cases, the associated condition is included in the main diagnosis (eg, CIDP with systemic lupus erythematosus, CIDP with HIV infection, CIDP associated with hepatitis B or C) to separate those cases from the idiopathic variety. Most reported conditions associated with CIDP are listed below.

  • CIDP associated with other disorders cannot be distinguished clinically from isolated CIDP except where mentioned in this section. The disease mechanisms for all these disorders are not known. They appear to be immunologic; in some instances, antibody-mediated mechanisms have been shown to play a role.
  • Vasculitis does not seem to be involved; physiologic and pathologic differences can distinguish the multifocal variants of CADP from vasculitic mononeuritis multiplex. Vasculitic neuropathies cause wallerian degeneration with minimal signs, if any, of segmental demyelination on biopsy and electromyographic studies (EMG). The multifocal variants of CADP have prominent conduction block and slowing—hallmarks of segmental demyelination.
  • HIV infection: In these patients, mild lymphocytic pleocytosis and increased gamma globulin level in the CSF are seen frequently. CIDP has been observed with early disease and later on in the course of AIDS.
  • Hodgkin lymphoma: Neuropathy associated with Hodgkin lymphoma is not caused by direct infiltration of the peripheral nerves but is a consequence of the autoimmune cascade that occurs with this disease; the mechanism is not completely clear.
  • Paraproteinemias and/or plasma cell dyscrasias
    • CADP is seen with monoclonal gammopathies (eg, monoclonal gammopathy of unknown significance [MGUS]), most frequently gammopathy of IgM. Evidence exists to suggest that CADP with IgM MGUS has specific clinical and electrophysiologic characteristics. Patients usually have predominance of distal sensory symptoms that are greater than motor symptoms. Conduction slowing on nerve conduction testing is accentuated in distal nerve segments. Fifty percent of patients with IgM-associated neuropathies have antibodies directed against MAG, a protein found in noncompact myelin of peripheral nerves. Whether any clinical difference exists between patients with IgM gammopathy without anti-MAG antibodies and those with anti-MAG antibodies is not clear. In both cases, the response to immunosuppressive and/or immunomodulatory treatment is poor. However, a number of reports, including a double-blind controlled trial, describe a response to rituximab, a monoclonal antibody directed against B cells.
    • Some paraproteinemias occur as isolated phenomena and some are by-products of malignant cells, as in the cases of Waldenström macroglobulinemia and myeloma. In myeloma-associated neuropathy, the abnormal paraprotein usually consists mostly of lambda light chain component. Combination of osteosclerotic myeloma, organomegaly, endocrinopathy, M protein, sensorimotor neuropathy, and pigmentary skin changes is referred to as POEMS syndrome. In POEMS syndrome, the M protein is typically immunoglobulin G (IgG).
    • The association of CIDP with IgG or immunoglobulin A (IgA) gammopathy is less clear. IgG paraproteins can occur in 5% of the population and it is unclear that the incidence of IgG paraproteins is excessive in patients with demyelinating neuropathy. Patients with CIDP and IgG or IgA paraproteins have identical clinical and electrophysiologic features to patients with CIDP and no paraprotein. Response to treatment is also the same.
  • Multiple sclerosis: Reports describe CNS white matter changes in patients with CIDP. Whether a true association exists between CIDP and multiple sclerosis remains unclear.
  • Chronic active hepatitis (B or C): CIDP associated with hepatitis should be differentiated from cryoglobulinemic vasculitis. The latter causes either symmetric distal sensorimotor polyneuropathy or mononeuropathy multiplex but on pathologic examination shows wallerian degeneration and not the segmental demyelination seen in CIDP. Patients can respond to standard CIDP treatments, but in some patients, treatment of the hepatitis infection is associated with remission of the CIDP.
  • Inflammatory bowel disease (IBD): CIDP has been described in association with Crohn disease and other inflammatory bowel conditions, although no direct correlation between the 2 afflictions is known. The mechanism of development of CIDP is presumed to be an autoimmune abnormality that is also causing the primary problem in inflammatory bowel disease, although the details are not known. The situation has become complicated with the recognition that TNF-alpha therapies used for IBD can cause CIDP.
  • TNF-alpha blockers: CIDP, Lewis-Sumner syndrome, and multifocal motor neuropathy have been described in patients with rheumatoid arthritis and IBD who are treated with TNF-alpha blockers, particularly etanercept and infliximab. In some cases, discontinuation of the medication was associated with remission of the inflammatory neuropathy.
  • Diabetes mellitus: Whether an increased incidence of CIDP occurs in patients with diabetes mellitus remains unclear. The most recent literature has not corroborated earlier reports of an association.
  • Pregnancy: Pregnancy is known to exacerbate CIDP. Worsening usually occurs in the third trimester or in the postpartum period.
Contributor Information and Disclosures

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.


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.

  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].

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.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.