eMedicine Specialties > Neurology > Neuromuscular Diseases

Acute Inflammatory Demyelinating Polyradiculoneuropathy: Follow-up

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
Coauthor(s): Richard A Sater, MD, PhD, Consulting Staff, High Point Neurological Associates
Contributor Information and Disclosures

Updated: Jan 15, 2009

Follow-up

Further Inpatient Care

Based on the severity of symptoms, patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) may require further inpatient services.

  • Patients should have cardiac monitoring to confirm and treat arrhythmias.
  • Pulmonary function tests such as FVC and negative inspiratory pressure should be performed 3-4 times a day until a patient has reached a plateau for several days.
  • Transfer to an ICU is recommended for patients with worsening respiratory effort (ie, FVC <20 mL/kg) or cardiac arrhythmias.
  • Physical therapy should be initiated early to help increase patient activity and mobility. Patients who do not recover quickly benefit by transfer to an inpatient rehabilitation center before returning home.

Further Outpatient Care

  • Generally, all patients in whom AIDP is suspected should be admitted for further monitoring and treatment.
  • Patients who present with mild neurologic impairment after already reaching a plateau can be treated as outpatients with close supervision.
  • Upon discharge, patients require several follow-up visits to ensure that relapses do not occur and to help coordinate home-health services if necessary. Physical and occupational therapy, either in a long-term rehabilitation unit or at home, help many patients return more rapidly to their baseline level of activity.
  • Relapses occur (10-20%) following completion of plasma exchange, and these relapses frequently respond to a second course of treatment. Similarly, relapses that follow IVIG therapy also respond to a second course.

Transfer

  • To the ICU when respiratory failure is impending or when cardiac arrhythmias are occurring
  • To regional or tertiary hospitals if a community hospital does not have an ICU or is unable to provide IVIg or plasmapheresis therapy

Complications

Critically ill patients are susceptible to the same complications as other intubated patients, including pneumonia, sepsis, skin decubiti, deep venous thrombosis, and urinary tract infections. Patients with AIDP have some unique complications that may cause significant morbidity, the most common being pain, labile blood pressure, and increased sensitivity to cardiac medications.

Prognosis

  • About 75% of patients have an excellent recovery and regain their premorbid condition.
    • Some of these patients experience easy fatigability for many years.
    • Almost all of the remaining patients have mild or moderately severe impairment but remain independent in most functions. Residual complaints include dysesthesias, foot drop, and intrinsic hand muscle weakness.
    • Severe disability occurs in fewer than 5% of patients, who do not recover full independence. Patients with residual deficits are usually those who required mechanical intubation. Improvement is usually complete by 6 months. In more serious cases, recovery may continue for 18-24 months.
  • Death occurs in only 2-6% of patients and is usually due to cardiac arrest, ARDS, pulmonary embolism, severe bronchospasm, pneumonia, or sepsis.
  • About 10% of patients have a relapse 1-6 weeks after completing immunomodulatory therapy. These patients can be treated with a second course of immunomodulation.
  • Fewer than 1% of patients have AIDP 1 or more years after onset of symptoms. In some cases, the recurrence follows immunization. This recurrence differs from CIDP.
  • Sporadic cases of recurrent Guillain-Barré syndrome13 and rare cases of recurrent Guillain-Barré syndrome after a long asymptomatic period14 have been reported. Some authors consider recurrent Guillain-Barré syndrome a variant of CIDP, while others maintain that they are 2 different entities.Martic et al describe a patient who developed Guillain-Barré syndrome as a child and experienced a full relapse after 19 years with another innocuous episode 10 years later.15
  • Several prognostic factors have been identified.
    • In general, younger patients have a better prognosis than older patients. Those patients with more severe weakness and those who are intubated have a worse prognosis than those with milder weakness.
    • Diarrhea as an antecedent association often is associated with C jejuni infection. These patients may have a more prolonged recovery.
    • Early improvement in strength during treatment is associated with a more rapid recovery. Low compound muscle action potential (CMAP) amplitudes (<20% of normal) are considered a bad prognostic indicator.
  • In spite of therapy with plasma exchange or IVIG, the decrease in mortality has often been attributed to improved aggressive supportive treatment than to any drug treatment. This has included close monitoring with the avoidance of hypoxia, pain, and arrhythmogenic stimuli.
    • In the presence of dysautonomia, hypoxia can trigger cardiac arrhythmias. Tracheal suction can also at times result in cardiac arrhythmias. Ideally, these patients should be given extra oxygen before tracheal toilet.
    • Subcutaneous heparin to avoid venous thromboembolism, treatment of pain with analgesics including narcotics, treatment of hypotension and hypertension, as the case be and treatment of severe bradyarrhythmia all go a long way in decreasing mortality. Carbamazepine and gabapentin may help.
    • Persistent fatigue following Guillain-Barré syndrome is common and may be helped by a graded exercise program. C jejuni is often treated with a course of erythromycin.
    • Hyponatremia is due to inappropriate antidiuretic hormone secretion (SIADH) is best managed by fluid restriction coupled by the avoidance of hyponatremic fluids. Need for immunization should be reviewed on an individual basis.

Patient Education

Guillain-Barré Syndrome Foundation International

Miscellaneous

Medicolegal Pitfalls

The main medicolegal pitfalls involve not anticipating progression of symptoms.

  • This may occur when a patient who has not reached a plateau is not admitted to a hospital.
  • Similarly, admitted patients require cardiac monitoring and FVC and negative inspiratory pressure testing at least 3 times daily.

Special Concerns

  • Plasmapheresis and IVIg have been used in pregnant patients; however, the risks are not well documented.
  • In elderly patients, arrhythmias are the most common cause of death. In addition, elderly patients have a higher complication rate.
  • The Miller-Fisher variant is more common in children than in adults, representing as many as 20% of cases.
  • To assess the impact of new strategies of therapy on outcomes and hospitalization charges among patients with Guillain-Barré syndrome requiring mechanical ventilation, Souayah et al compared pertinent variables between nationally representative data derived from 1992-2002.16 Their findings reveal that improvements in therapeutic strategies over that decade are not reflected in mortality, length of hospitalization, or hospital charges in the current study. Thus, this outcome may be more reflective of changing patterns of hospitalization rather than relative futility of new treatments.
  • Frenzen found that the estimated annual cost of Guillain-Barré syndrome was $1.7 billion (95% CI, $1.6-1.9 billion), including $0.2 billion (14%) in direct medical costs and $1.5 billion (86%) in indirect costs. Most of the medical costs were for community hospital admissions. Most of the indirect costs were due to premature deaths. The mean cost per patient with Guillain-Barré syndrome was $318,966 (95% CI, $278,378-$359,554).17
 


More on Acute Inflammatory Demyelinating Polyradiculoneuropathy

Overview: Acute Inflammatory Demyelinating Polyradiculoneuropathy
Differential Diagnoses & Workup: Acute Inflammatory Demyelinating Polyradiculoneuropathy
Treatment & Medication: Acute Inflammatory Demyelinating Polyradiculoneuropathy
Follow-up: Acute Inflammatory Demyelinating Polyradiculoneuropathy
References
[ CLOSE WINDOW ]

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. 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].

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

  9. 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].

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

  11. 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].

  12. 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].

  13. 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].

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

  15. 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].

  16. 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.

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

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

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

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

  21. 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].

  22. 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].

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

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

  25. 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].

  26. 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].

  27. 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.

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

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

  30. 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].

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

  32. 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].

  33. 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].

  34. 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].

  35. 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].

  36. 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].

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

  38. 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].

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

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

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

  42. 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].

  43. 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].

  44. 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].

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

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

  47. 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].

  48. 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].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

acute idiopathic polyneuritis, acute inflammatory demyelinating polyneuropathy, ascending paralysis, Guillain-Barré syndrome, Guillain-Barré-Strohl syndrome, AIDP, acute inflammatory demyelinating polyradiculoneuropathy

[ CLOSE WINDOW ]

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  Honoraria Consulting; Teva Marion Honoraria Consulting; 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.

Medical Editor

Donald B Sanders, MD, EMG Laboratory Director, Professor of Medicine (Neurology), Division of Neurology, Duke University Medical Center
Donald B Sanders, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Neurological Association, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Pharmacy Editor

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

Managing Editor

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, 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: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
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.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
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.