Acute Inflammatory Demyelinating Polyradiculoneuropathy Treatment & Management

  • Author: Tarakad S Ramachandran, MBBS, FRCP(C), FACP; Chief Editor: Nicholas Lorenzo, MD   more...
 
Updated: Feb 6, 2012
 

Medical Care

Advances in supportive medical care have resulted in improved survival rates in acute inflammatory demyelinating polyneuropathy (AIDP).

  • Mechanical ventilatory assistance is required in about one third of patients with AIDP and lasts for an average of 49 days. Intubation should be performed when FVC drops to less than 15 mL/kg or negative inspiratory pressure is worse than -25 cm H2 O. Tracheostomy is usually recommended if mechanical ventilation will be required for more than 2-3 weeks. Bedridden patients need prophylaxis against thromboembolism. Subcutaneous heparin is the most common agent. Some may also need GI prophylaxis with an H2-blocker (or similar agent).
  • Enteric nutrition is necessary for patients on mechanical ventilation. Nasogastric tubes or Dubhoff tubes can be used initially. Those requiring more than 2 or 3 weeks or enteric nutrition may require gastrostomy or jejunostomy tube feedings.
  • Cardiac monitoring is necessary. Chronic sinus tachycardia often responds to beta-blockers or calcium channel blockers. Bradycardia requires atropine treatment, if symptomatic. Heart block may require temporary pacing. Hypertension responds well to beta-blockers. These treatments should be administered cautiously under the direction of a cardiologist or critical care specialist, since one of the main causes of death is iatrogenic hypotension, especially in patients with autonomic failure.
  • Constipation is common in intubated patients with AIDP, and a bowel regimen is usually necessary. Some patients may also require enemas. Ileus is rare. If it occurs, bowel rest is usually necessary and parenteral nutrition can be used during that time.
  • Skilled nursing care of intubated patients is necessary to avoid skin breakdown. Special mattresses are available in most intensive care or step-down units. Communication difficulties can lead to frustration and exacerbate depression. Involvement of speech therapy, physical therapy, and occupational therapy is highly recommended. Many patients may require a rehabilitation unit after being weaned off a ventilator.
  • Conventional immunosuppressant treatments with corticosteroids have failed to show benefit. But immunomodulation with IVIg and plasmapheresis has led to faster recovery, relatively mild disability, and shorter hospital stays. IV steroid therapy alone is not indicated for the treatment of AIDP. Treatment is less likely to be effective if initiated more than 2 weeks after the onset of symptoms. Some patients with mild weakness, especially those presenting during the plateau, may not require immunomodulatory therapy. Plasmapheresis had shown to cut the respirator time and time to independent ambulation, by about half when treatment was given during the first week of the disease.
  • In their study of immunotherapy in Guillain-Barr é syndrome, Alshekhlee et al found an increasing use of IVIg over plasma exchange (PE). Older population and those with pulmonary or sepsis complications were likely treated with PE. The mortality rate was higher in patients treated with PE.[14]
Next

Surgical Care

Tracheostomy is necessary in many intubated patients. Those requiring long-term enteral nutrition typically require a gastrostomy or jejunostomy.

Previous
Next

Consultations

  • Neurology: For patients on general medicine or other services, neurological consultation is indicated to manage diagnostic studies and to help determine appropriate treatment.
  • Critical care: About one third of patients require mechanical ventilation. Any intubated patient or patient who is transferred to an ICU for monitoring should be monitored by a critical care or pulmonary specialist.
  • Surgery: Some patients may require tracheostomy or a feeding tube for parenteral nutrition.
  • Cardiology: Patients with arrhythmias in addition to sinus tachycardia or major cardiac rhythm abnormalities should be evaluated by a cardiologist.
  • Physical medicine and rehabilitation
Previous
Next

Diet

No special diet is required.

Previous
Next

Activity

Keep patients ambulatory if they are able to walk without assistance. Most patients who are admitted to the hospital require bedrest.

Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Tarakad S Ramachandran, MBBS, FRCP(C), FACP  Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, FRCP(C), FACP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine

Disclosure: Abbott Labs None None; Teva Marion None None; Boeringer-Ingelheim Honoraria Speaking and teaching

Coauthor(s)

Richard A Sater, MD, PhD  Consulting Staff, High Point Neurological Associates

Richard A Sater, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Medical Association, and American Society of Neuroradiology

Disclosure: Nothing to disclose.

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: Medscape Salary Employment

Glenn Lopate, MD  Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Director of Neurology Clinic, St Louis ConnectCare; Consulting Staff, Department of Neurology, Barnes-Jewish Hospital

Glenn Lopate, 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: Baxter Grant/research funds Other; Amgen Grant/research funds None

Chief Editor

Nicholas Lorenzo, MD  Consulting Staff, Neurology Specialists and Consultants

Nicholas Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and American College of Physician Executives

Disclosure: Nothing to disclose.

References
  1. HAYMAKER WE, KERNOHAN JW. The Landry-Guillain-Barré syndrome; a clinicopathologic report of 50 fatal cases and a critique of the literature. Medicine (Baltimore). Feb 1949;28(1):59-141. [Medline].

  2. Krucke W. Die primar-entzundliche polyneuritis unbekannter ursache. Handbuch des speziellen pathologischen anatomie und histologie. 1955;Berlin, Springer-Verlag.

  3. WAKSMAN BH, ADAMS RD. Allergic neuritis: an experimental disease of rabbits induced by the injection of peripheral nervous tissue and adjuvants. J Exp Med. Aug 1 1955;102(2):213-36. [Medline].

  4. Nachamkin I, Barbosa PA, Ung H, Lobato C, Rivera AG, Rodriguez P. Patterns of Guillain-Barre syndrome in children: results from a Mexican population. Neurology. Oct 23 2007;69(17):1665-71. [Medline].

  5. Ropper AH. Unusual clinical variants and signs in Guillain-Barre syndrome. Arch Neurol. Nov 1986;43(11):1150-2. [Medline].

  6. Nagashima T, Koga M, Odaka M, Hirata K, Yuki N. Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. Arch Neurol. Oct 2007;64(10):1519-23. [Medline].

  7. Tse AC, Cheung RT, Ho SL, Chan KH. Guillain-Barré syndrome associated with acute hepatitis E infection. J Clin Neurosci. Jan 26 2012;[Medline].

  8. Wagner JC, Bromberg MB. HIV infection presenting with motor axonal variant of Guillain-Barré Syndrome. J Clin Neuromuscul Dis. Dec 2007;9(2):303-5. [Medline].

  9. Willison HJ. The immunobiology of Guillain-Barre syndromes. J Peripher Nerv Syst. Jun 2005;10(2):94-112. [Medline].

  10. Park SJ, Pai KS, Kim JH, Shin JI. The role of interleukin 6 in the pathogenesis of hyponatremia associated with Guillain-Barré syndrome. Nefrologia. Jan 27 2012;32(1):114. [Medline].

  11. Kaida K, Kamakura K, Ogawa G, Ueda M, Motoyoshi K, Arita M. GD1b-specific antibody induces ataxia in Guillain-Barre syndrome. Neurology. Jul 15 2008;71(3):196-201. [Medline].

  12. Cornblath DR. Electrophysiology in Guillain-Barré syndrome. Ann Neurol. 1990;27 Suppl:S17-20. [Medline].

  13. Jin K, Takeda A, Shiga Y, Sato S, Ohnuma A, Nomura H. CSF tau protein: a new prognostic marker for Guillain-Barré syndrome. Neurology. Oct 24 2006;67(8):1470-2. [Medline].

  14. Alshekhlee A, Hussain Z, Sultan B, Katirji B. Immunotherapy for Guillain-Barré syndrome in the US hospitals. J Clin Neuromuscul Dis. Sep 2008;10(1):4-10. [Medline].

  15. Grand'Maison F, Feasby TE, Hahn AF, Koopman WJ. Recurrent Guillain-Barré syndrome. Clinical and laboratory features. Brain. Aug 1992;115 ( Pt 4):1093-106. [Medline].

  16. Wijdicks EF, Ropper AH. Acute relapsing Guillain-Barré syndrome after long asymptomatic intervals. Arch Neurol. Jan 1990;47(1):82-4. [Medline].

  17. Martic V, Lepic T. Recurrence of childhood Guillain-Barré syndrome after a long asymptomatic interval: a case report. J Clin Neuromuscul Dis. Sep 2007;9(1):256-61. [Medline].

  18. Souayah N, Nasar A, Suri MFK, Qureshi A. National Trends in Hospital Outcomes Among Patients with Guillain-Barre Syndrome Requiring Mechanical Ventilation. Journal of Clinical Neuromuscular Disease. 2008;10(1):24-28.

  19. Frenzen PD. Economic cost of Guillain-Barré syndrome in the United States. Neurology. Jul 1 2008;71(1):21-7. [Medline].

  20. Asbury AK. Diagnostic considerations in Guillain-Barre syndrome. Ann Neurol. 1981;9 Suppl:1-5. [Medline].

  21. Ascherio A, Bermudez CS, Garcia D. Outbreak of buckthorn paralysis in Nicaragua. J Trop Pediatr. Apr 1992;38(2):87-9. [Medline].

  22. Berlit P, Rakicky J. The Miller Fisher syndrome. Review of the literature. J Clin Neuroophthalmol. Mar 1992;12(1):57-63. [Medline].

  23. Chiba A, Kusunoki S, Obata H. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barre syndrome: clinical and immunohistochemical studies. Neurology. Oct 1993;43(10):1911-7. [Medline].

  24. Crino PB, Zimmerman R, Laskowitz D. Magnetic resonance imaging of the cauda equina in Guillain-Barre syndrome. Neurology. Jul 1994;44(7):1334-6. [Medline].

  25. Dwyer JM. Manipulating the immune system with immune globulin. N Engl J Med. Jan 9 1992;326(2):107-16. [Medline].

  26. Feasby TE. Axonal Guillain-Barre syndrome. Muscle Nerve. Jun 1994;17(6):678-9. [Medline].

  27. FISHER M. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med. Jul 12 1956;255(2):57-65. [Medline].

  28. French Cooperative Group on Plasma Exchange in Guillain-Barre syndrome. Efficiency of plasma exchange in Guillain-Barre syndrome: role of replacement fluids. French Cooperative Group on Plasma Exchange in Guillain-Barre syndrome. Ann Neurol. Dec 1987;22(6):753-61. [Medline].

  29. Guillain G, Barre JA, Strohl A. Sur un syndrome de radiculo-nevrite avec hyperalbuminose du liquide cephalo-rachidien sans reaction cellulaire. Bulletins et memories de la Societe Medicale des Hopitaux de Paris. 1916;40:1462.

  30. Guillain-Barré Syndrome Study Group. Plasmapheresis and acute Guillain-Barre syndrome. Neurology. Aug 1985;35(8):1096-104. [Medline].

  31. Hughes RAC. Guillain-Barre Syndrome. 1990.

  32. Irani DN, Cornblath DR, Chaudhry V. Relapse in Guillain-Barre syndrome after treatment with human immune globulin. Neurology. May 1993;43(5):872-5. [Medline].

  33. Landry O. Note sur la paralysis ascendante aigue. Gazette Hebdomadaire. 1859;6:472.

  34. Leong H, Stachnik J, Bonk ME, Matuszewski KA. Unlabeled uses of intravenous immune globulin. Am J Health Syst Pharm. Oct 1 2008;65(19):1815-24. [Medline].

  35. McGrogan A, Madle GC, Seaman HE, de Vries CS. The Epidemiology of Guillain-Barré Syndrome Worldwide. A Systematic Literature Review. Neuroepidemiology. Dec 17 2008;32(2):150-163. [Medline].

  36. McKhann GM, Cornblath DR, Griffin JW. Acute motor axonal neuropathy: a frequent cause of acute flaccid paralysis in China. Ann Neurol. Apr 1993;33(4):333-42. [Medline].

  37. Osterman PO, Fagius J, Safwenberg J. Early relapse of acute inflammatory polyradiculoneuropathy after successful treatment with plasma exchange. Acta Neurol Scand. Apr 1988;77(4):273-7. [Medline].

  38. Plasma Exchange/Sandoglobulin Guillain-Barre Syndrome Trial Group. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barre syndrome. Lancet. Jan 25 1997;349(9047):225-30. [Medline].

  39. Prineas JW. Acute idiopathic polyneuritis. An electron microscope study. Lab Invest. Feb 1972;26(2):133-47. [Medline].

  40. Ropper AH. Further regional variants of acute immune polyneuropathy. Bifacial weakness or sixth nerve paresis with paresthesias, lumbar polyradiculopathy, and ataxia with pharyngeal-cervical-brachial weakness. Arch Neurol. Jul 1994;51(7):671-5. [Medline].

  41. Ropper AH, Wijdicks EFM, Truax BT. Guillain-Barre Syndrome. Contemporary Neurology Series. 1991.

  42. Rostami AM, Sater RA. Guillain-Barre Syndrome. Neuroimmunology for the Clinician. 1997;205-228.

  43. Sater RA, Rostami A. Treatment of Guillain-Barre syndrome with intravenous immunoglobulin. Neurology. Dec 1998;51(6 Suppl 5):S9-15. [Medline].

  44. van der Meche FG, Schmitz PI. A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barre syndrome. Dutch Guillain-Barre Study Group. N Engl J Med. Apr 23 1992;326(17):1123-9. [Medline].

  45. Vriesendorp FJ, Mishu B, Blaser MJ. Serum antibodies to GM1, GD1b, peripheral nerve myelin, and Campylobacter jejuni in patients with Guillain-Barre syndrome and controls: correlation and prognosis. Ann Neurol. Aug 1993;34(2):130-5. [Medline].

  46. Vriesendorp FJ, Triggs WJ, Mayer RF. Electrophysiological studies in Guillain-Barre syndrome: correlation with antibodies to GM1, GD1B and Campylobacter jejuni. J Neurol. Jul 1995;242(7):460-5. [Medline].

  47. Willison HJ, Winer JB. Clinical evaluation and investigation of neuropathy. J Neurol Neurosurg Psychiatry. Jun 2003;74 Suppl 2:ii3-ii8. [Medline].

  48. Young RR, Asbury AK, Corbett JL. Pure pandysautonomia with recovery. Description and discussion of diagnostic criteria. Brain. Dec 1975;98(4):613-36. [Medline].

  49. Yuki N, Taki T, Inagaki F. A bacterium lipopolysaccharide that elicits Guillain-Barre syndrome has a GM1 ganglioside-like structure. J Exp Med. Nov 1 1993;178(5):1771-5. [Medline].

  50. Yuki N, Taki T, Takahashi M, Saito K, Yoshino H, Tai T, et al. Molecular mimicry between GQ1b ganglioside and lipopolysaccharides of Campylobacter jejuni isolated from patients with Fisher's syndrome. Ann Neurol. Nov 1994;36(5):791-3. [Medline].

Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.