eMedicine Specialties > Neurology > Neuromuscular Diseases

Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Follow-up

Author: Richard A Lewis, MD, Professor and Associate Chairman of Neurology, Department of Neurology, Wayne State University School of Medicine
Contributor Information and Disclosures

Updated: Jan 15, 2009

Follow-up

Further Inpatient Care

Most care is delivered on an outpatient basis, although patients may have to be admitted for a short stay for the initiation of plasmapheresis or IVIg treatment, because of complications of chronic inflammatory demyelinating polyradiculoneuropathy or treatment, or for inpatient physical therapy.

Further Outpatient Care

Outpatient care consists of visits to specialists such as neurologists and physiatrists and of treatment visits for IVIg infusions or to the plasmapheresis unit.

Inpatient & Outpatient Medications

Most medications are administered on an outpatient basis. IVIg can be administered as a home infusion or during an outpatient visit. An exception is plasmapheresis, which requires visits to a specialized pheresis center.

Complications

  • If the disease becomes severe, swallowing and breathing functions can be affected. Aspiration pneumonia, atelectasis, and respiratory failure can occur.
  • If autonomic function is involved, GI motility and bladder function can be abnormal. Orthostatic hypotension and cardiac conduction defects can occur.
  • As already discussed, complications of treatment also must be considered.

Prognosis

Some have suggested that patients with the relapsing disease have a better prognosis than patients with the chronic progressive course. Approximately 70% of patients are said to make relatively good recovery from their relapses, and close to 90% of patients respond to initial immunosuppressive therapy. Some patients do not respond to the usual treatments and accumulate significant disability. Some patients have only a short treatment effect and become treatment dependent.

Patient Education

Refer to physical and occupational therapists and to a physiatrist for optimal outpatient therapy, orthotic devices, and adaptation at home.

Miscellaneous

Medicolegal Pitfalls

Chronic inflammatory demyelinating polyradiculoneuropathy and its variants are uncommon disorders and can be difficult to diagnose and treat. Frequently, patients with this disorder require referral to a medical center specializing in neuropathic disorders.

Special Concerns

Associated conditions need to be excluded and treated appropriately.

 
Acknowledgments

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



More on Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Overview: Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Differential Diagnoses & Workup: Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Treatment & Medication: Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Follow-up: Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Multimedia: Chronic Inflammatory Demyelinating Polyradiculoneuropathy
References

References

  1. [Best Evidence] Hughes RA, Donofrio P, Bril V, Dalakas MC, Deng C, Hanna K, 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. Feb 2008;7(2):136-44. [Medline].

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

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

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

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

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

  7. 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. Jan 1998;21(1):55-62. [Medline].

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

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

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

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

  12. Dyck PJ, Lais AC, Ohta M, et al. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc. Nov 1975;50(11):621-37. [Medline].

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

  14. Erdmann PG, van Meeteren NL, Kalmijn S, et al. Functional health status of patients with chronic inflammatory neuropathies. J Peripher Nerv Syst. Jun 2005;10(2):181-9. [Medline].

  15. European Federation of Neurological Societies/Peripheral Nerve Society. 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 Periph. J Peripher Nerv Syst. Sep 2005;10(3):220-228. [Medline].

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

  17. 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. Feb 1997;48(2):321-8. [Medline].

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

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

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

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

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

  23. Mahattanakul W, Crawford TO, Griffin JW, et al. Treatment of chronic inflammatory demyelinating polyneuropathy with cyclosporin-A. J Neurol Neurosurg Psychiatry. Feb 1996;60(2):185-7. [Medline].

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

  25. Notermans NC, Franssen H, Eurelings M, et al. Diagnostic criteria for demyelinating polyneuropathy associated with monoclonal gammopathy. Muscle Nerve. Jan 2000;23(1):73-9. [Medline].

  26. Rotta FT, Sussman AT, Bradley WG, et al. The spectrum of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. Feb 15 2000;173(2):129-39. [Medline].

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

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

  29. Stamboulis E, Katsaros N, Koutsis G, et al. Clinical and subclinical autonomic dysfunction in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. Sep 23 2005;[Medline].

  30. 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. Apr 1999;110(4):705-11. [Medline].

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

Further Reading

Keywords

chronic inflammatory demyelinating polyneuropathy, CIDP, chronic acquired demyelinating polyneuropathies, CADP, acute inflammatory demyelinating polyradiculoneuropathy, AIDP, Guillain-Barre Syndrome #233; syndrome, GBS, chronic inflammatory demyelinating polyradiculoneuropathy, monoclonal gammopathies, multifocal motor neuropathy, MMN, acquired purely motor neuropathy, lower motor neuron-type deficits, multifocal sensorimotor neuropathy, sensorimotor mononeuropathy multiplex, Lewis-Sumner neuropathy, sensory predominant CADP, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, POEMS, CADP associated with diabetes mellitus, sensorimotor disorder

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 Association of Neuromuscular and Electrodiagnostic Medicine, American Society of Neuroimaging, and Medical Society of Virginia
Disclosure: Nothing to disclose.

Medical Editor

Dianna Quan, MD, Associate Professor of Neurology, Director, Electromyography Laboratory, University of Colorado Health Sciences Center
Dianna Quan, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa
Disclosure: e-medicine Honoraria Other

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Paraplegia Society, and National Multiple Sclerosis Society
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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