eMedicine Specialties > Emergency Medicine > Neurology

Guillain-Barre Syndrome: Differential Diagnoses & Workup

Author: Andrew C Miller, MD, Chief Resident and Clinical Assistant Instructor, Departments of Emergency Medicine and Internal Medicine, State University of New York Downstate Medical Center, Kings County Hospital Center
Coauthor(s): Razi M Rashid, MD, MPH, Intern, Department of Internal Medicine, St John's Hospital and Medical Center, Detroit, Michigan; Richard H Sinert, DO, Associate Professor of Emergency Medicine, Clinical Assistant Professor of Medicine, Research Director, State University of New York College of Medicine; Consulting Staff, Department of Emergency Medicine, Kings County Hospital Center
Contributor Information and Disclosures

Updated: Jul 1, 2009

Differential Diagnoses

Cauda Equina Syndrome
Polymyositis
CBRNE - Botulism
Spinal Cord Infections
Diphtheria
Spinal Cord Injuries
Encephalitis
Systemic Lupus Erythematosus
Hyperkalemia
Tick-Borne Diseases, Lyme
Hypokalemia
Toxicity, Alcohols
Hypophosphatemia
Toxicity, Heavy Metals
Meningitis
Toxicity, Organophosphate and Carbamate
Multiple Sclerosis
Myasthenia Gravis

Other Problems to Be Considered

West Nile encephalitis
Basilar artery occlusion
Chronic inflammatory demyelinating polyneuropathy
Folate deficiency
Hereditary neuropathies
Neoplasia
Neurotoxic fish poisoning
Poliomyelitis
Porphyria
Sarcoid meningitis
Spinal cord compression
Spinal cord syndromes, particularly postinfection
Tick paralysis
Transverse myelitis
Vitamin B-12 deficiency
Vitamin B-6
HIV peripheral neuropathy
Thiamine deficiency

Workup

Laboratory Studies

  • Diagnosis of Guillain-Barré syndrome usually is made on clinical grounds. Laboratory studies are useful to rule out other diagnoses and to better assess functional status and prognosis.
  • Lumbar puncture and spinal fluid analysis
    • Elevated or rising protein levels on serial lumbar punctures and 10 or fewer mononuclear cells/mm3 strongly support the diagnosis.
    • Most, but not all, patients have an elevated CSF protein level (>400 mg/L), with normal CSF cell counts.
    • Normal CSF protein level does not rule out Guillain-Barré syndrome as the level may remain normal in 10% of patients, and a rise in the CSF protein level may not be observed until 1-2 weeks after the onset of weakness.
    • CSF pleocytosis is well recognized in HIV-associated Guillain-Barré syndrome.
  • Biochemical screening
    • Biochemical screening includes electrolyte levels; liver function tests (LFTs); CPK level; erythrocyte sedimentation rate (ESR); antiganglioside antibodies; and antibodies to C jejuni, cytomegalovirus, Ebstein-Barr virus, herpes simplex virus (HSV), HIV, and M pneumoniae.
    • Syndrome of inappropriate antidiuretic hormone (SIADH) occurs in some patients with Guillain-Barré syndrome.
    • LFT results are elevated in up to one third of patients.
    • CPK and ESR may be elevated with myopathies or systemic inflammatory conditions.
    • Patients with the Miller-Fisher variant may have anti-GQ1b antibodies.
    • Patients who have antibody subtype GM1 may have worse prognoses.
  • Stool culture for C jejuni
  • Pregnancy test

Imaging Studies

  • MRI
    • MRI is a sensitive but nonspecific test.
    • Spinal nerve root enhancement with gadolinium is a nonspecific feature seen in inflammatory conditions and is caused by disruption of the blood-nerve barrier.
    • Selective anterior nerve root enhancement appears to be strongly suggestive of GBS.
    • The cauda equine nerve roots are enhanced in 83% of patients.

Other Tests

  • Forced vital capacity29
    • Forced vital capacity (FVC) is very helpful in guiding disposition and therapy.
    • Patients with an FVC less than 15-20 mL/kg, maximum inspiratory pressure less than 30 cm H2 O, or a maximum expiratory pressure less than 40 cm H2 O generally progress to require prophylactic intubation and mechanical ventilation.
  • Nerve conduction studies2
    • A delay in F waves is present, implying nerve root demyelination.
    • Nerve motor action potentials may be decreased. This is technically difficult to determine until the abnormality is severe.
    • Compound muscle action potential (CMAP) amplitude may be decreased.
    • Frequently, patients show evidence of conduction block or dispersion of responses at sites of natural nerve compression. The extent of decreased action potentials correlates with prognosis.
    • Prolonged distal latencies may be present.
    • Rarely neurophysiologic testing is normal in patients with Guillain-Barré syndrome. This is believed to be due to the location of demyelinating lesions in proximal sites not amenable to study.
  • Muscle biopsy may help to distinguish Guillain-Barré syndrome from a primary myopathy in unclear cases.
  • Many different abnormalities may be seen on ECG, including second-degree and third-degree atrioventricular (AV) block, T-wave abnormalities, ST depression, QRS widening, and a variety of rhythm disturbances.

Procedures

  • Lumbar puncture and spinal fluid analysis

More on Guillain-Barre Syndrome

Overview: Guillain-Barre Syndrome
Differential Diagnoses & Workup: Guillain-Barre Syndrome
Treatment & Medication: Guillain-Barre Syndrome
Follow-up: Guillain-Barre Syndrome
References

References

  1. Seneviratne U. Guillain-Barre syndrome. Postgrad Med J. Dec 2000;76(902):774-82. [Medline].

  2. Winer JB. Guillain Barre syndrome. Mol Pathol. Dec 2001;54(6):381-5. [Medline].

  3. Kimoto K, Koga M, Odaka M, et al. Relationship of bacterial strains to clinical syndromes of Campylobacter-associated neuropathies. Neurology. Nov 28 2006;67(10):1837-43. [Medline].

  4. Geleijns K, Roos A, Houwing-Duistermaat JJ, et al. Mannose-binding lectin contributes to the severity of Guillain-Barre syndrome. J Immunol. Sep 15 2006;177(6):4211-7. [Medline].

  5. Winer JB. Treatment of Guillain-Barré syndrome. QJM. Nov 2002;95(11):717-21. [Medline].

  6. Li H, Yuan J. Miller Fisher syndrome: toward a more comprehensive understanding. Chin Med J (Engl). Mar 2001;114(3):235-9. [Medline].

  7. Van Koningsveld R, Van Doorn PA. Steroids in the Guillain-Barre syndrome: is there a therapeutic window?. Neurologia. Mar 2005;20(2):53-7. [Medline].

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

  9. Barzegar M, Alizadeh A, Toopchizadeh V, Dastgiri S, Majidi J. Association of Campylobacter jejuni infection and GuillainBarré syndrome: a cohort study in the northwest of Iran. Turk J Pediatr. 2008;50(5):433-8. [Medline].

  10. Bersano A, Carpo M, Allaria S. Long term disability and social status change after Guillain-Barre syndrome. J Neurol. 2006;253(2):214-8. [Medline].

  11. Das A, Kalita J, Misra UK. Recurrent Guillain Barre' syndrome. Electromyogr Clin Neurophysiol. Mar 2004;44(2):95-102. [Medline].

  12. Jiang GX, de Pedro-Cuesta J, Strigård K, Olsson T, Link H. Pregnancy and Guillain-Barré syndrome: a nationwide register cohort study. Neuroepidemiology. 1996;15(4):192-200. [Medline].

  13. Evans OB, Vedanarayanan V. Guillain-Barre syndrome. Pediatr Rev. Jan 1997;18(1):10-6. [Medline].

  14. Lo YL. Clinical and immunological spectrum of the Miller Fisher syndrome. Muscle Nerve. Nov 2007;36(5):615-27. [Medline].

  15. Lasky T, Terracciano GJ, Magder L, Koski CL, Ballesteros M, Nash D, et al. The Guillain-Barré syndrome and the 1992-1993 and 1993-1994 influenza vaccines. N Engl J Med. Dec 17 1998;339(25):1797-1802. [Medline].

  16. Kinnunen E, Junttila O, Haukka J, Hovi T. Nationwide oral poliovirus vaccination campaign and the incidence of Guillain-Barré Syndrome. Am J Epidemiol. Jan 1 1998;147(1):69-73. [Medline].

  17. Rantala H, Cherry JD, Shields WD, Uhari M. Epidemiology of Guillain-Barré syndrome in children: relationship of oral polio vaccine administration to occurrence. J Pediatr. Feb 1994;124(2):220-3. [Medline].

  18. Friedrich F. Rare adverse events associated with oral poliovirus vaccine in Brazil. Braz J Med Biol Res. Jun 1997;30(6):695-703. [Medline].

  19. Tuttle J, Chen RT, Rantala H, Cherry JD, Rhodes PH, Hadler S. The risk of Guillain-Barré syndrome after tetanus-toxoid-containing vaccines in adults and children in the United States. Am J Public Health. Dec 1997;87(12):2045-8. [Medline].

  20. Shaw FE Jr, Graham DJ, Guess HA, Milstien JB, Johnson JM, Schatz GC. Postmarketing surveillance for neurologic adverse events reported after hepatitis B vaccination. Experience of the first three years. Am J Epidemiol. Feb 1988;127(2):337-52. [Medline].

  21. da Silveira CM, Salisbury DM, de Quadros CA. Measles vaccination and Guillain-Barré syndrome. Lancet. Jan 4 1997;349(9044):14-6. [Medline].

  22. Centers for Disease Control and Prevention (CDC). Guillain-Barre Syndrome among recipients of Menactra meningococcal conjugate vaccine--United States. Morb Mortal Wkly Rep. Oct 14 2005;54(40):1023-5. [Medline].

  23. Stowe J, Andrews N, Wise L, Miller E. Investigation of the temporal association of Guillain-Barre syndrome with influenza vaccine and influenzalike illness using the United Kingdom General Practice Research Database. Am J Epidemiol. Feb 2009;169(3):382-8. [Medline].

  24. Awong IE, Dandurand KR, Keeys CA, Maung-Gyi FA. Drug-associated Guillain-Barre syndrome: a literature review. Ann Pharmacother. Feb 1996;30(2):173-80. [Medline].

  25. Shin IS, Baer AN, Kwon HJ, Papadopoulos EJ, Siegel JN. Guillain-Barré and Miller Fisher syndromes occurring with tumor necrosis factor alpha antagonist therapy. Arthritis Rheum. May 2006;54(5):1429-34. [Medline].

  26. Silburn S, McIvor E, McEntegart A and Wilson, H. -Barre syndrome i na patient receiving anti-tumour necrosis factor alpha for rheumatoid arthritis: a case report and discussion of literature. Annals of the rheumatic Diseases. April 2008;67(4):575-6. [Medline].

  27. Kurmann PT, Van Linthoudt D, So AK. Miller-Fisher syndrome in a patient with rheumatoid arthritis treated with adalimumab. Clinical Rheumatology. Jan 2009;28(1):23-4. [Medline].

  28. Machado FC, Valério BC, Morgulis RN, Nunes KF, Mazzali-Verst S. Acute axonal polyneuropathy with predominant proximal involvement: an uncommon neurological complication of bariatric surgery. Arq Neuropsiquiatr. Sep 2006;64(3A):609-12. [Medline].

  29. Teitelbaum JS, Borel CO. Respiratory dysfunction in Guillain-Barre syndrome. Clin Chest Med. Dec 1994;15(4):705-14. [Medline].

  30. Wijdicks EF, Henderson RD, McClelland RL. Emergency intubation for respiratory failure in Guillain-Barre syndrome. Arch Neurol. Jul 2003;60(7):947-8. [Medline].

  31. Koski CL, Patterson JV. Intravenous immunoglobulin use for neurologic diseases. J Infus Nurs. May-Jun 2006;29(3 Suppl):S21-8. [Medline].

  32. [Best Evidence] Hughes RA, Raphael JC, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2006;CD002063. [Medline][Full Text].

  33. [Best Evidence] Hughes RA, Swan AV, van Koningsveld R. Corticosteroids for Guillain-Barre syndrome. Cochrane Database Syst Rev. Apr 19 2006;2:CD001446. [Medline][Full Text].

  34. Dalakas MC. Intravenous immunoglobulin in autoimmune neuromuscular diseases. JAMA. May 19 2004;291(19):2367-75. [Medline].

  35. Fergusson D, Hutton B, Sharma M, et al. Use of intravenous immunoglobulin for treatment of neurologic conditions: a systematic review. Transfusion. Oct 2005;45(10):1640-57. [Medline].

  36. Hughes RA, Wijdicks EF, Barohn R, Benson E, Cornblath DR, Hahn AF. Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. Sep 23 2003;61(6):736-40. [Medline].

  37. Yata J, Nihei K, Ohya T, Hirano Y, Momoi M, Maekawa K. High-dose immunoglobulin therapy for Guillain-Barré syndrome in Japanese children. Pediatr Int. Oct 2003;45(5):543-9. [Medline].

  38. Lindenbaum Y, Kissel JT, Mendell JR. Treatment approaches for Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy. Neurol Clin. Feb 2001;19(1):187-204. [Medline].

  39. Odaka M, Tatsumoto M, Hoshiyama E, et al. Side effects of combined therapy of methylprednisolone and intravenous immunoglobulin in Guillain-Barre syndrome. Eur Neurol. 2005;53(4):194-6. [Medline].

  40. Seta T, Nagayama H, Katsura K. Factors influencing outcome in Guillain-Barre Syndrome: comparison of plasma adsorption against other treatments. Clin Neurol Neurosurg. Oct 2005;107(6):491-6. [Medline].

  41. Pritchard J, Gray IA, Idrissova ZR, Lecky BR, Sutton IJ, Swan AV, et al. A randomized controlled trial of recombinant interferon-beta 1a in Guillain-Barré syndrome. Neurology. Nov 2003;61(9):1282-1284.

  42. Hughes RA, Wijdicks EF, Benson E, Cornblath DR, Hahn AF, Meythaler JM. Supportive care for patients with guillain-barre syndrome. Arch Neurol. Aug 2005;62(8):1194-8. [Medline].

  43. Pandey CK, Raza M, Tripathi M, et al. The comparative evaluation of gabapentin and carbamazepine for pain management in Guillain-Barre syndrome patients in the intensive care unit. Anesth Analg. Jul 2005;101(1):220-5, table of contents. [Medline].

  44. Ilyas M, Tolaymat A. Minimal change nephrotic syndrome with Guillain-Barré syndrome. Pediatr Nephrol. Jan 2004;19(1):105-6. [Medline].

  45. Dornonville de la Cour C, Jakobsen J. Residual neuropathy in long-term population-based follow-up of Guillain-Barré syndrome. Neurology. Jan 2005;64(2):246-53. [Medline].

  46. Rudolph T, Larsen JP, Farbu E. The long-term functional status in patients with Guillain-Barre syndrome. European Journal of Neurology. Dec 2008;15(12):1332-7. [Medline].

  47. Zochodne DW. Autonomic involvement in Guillain-Barre syndrome: a review. Muscle Nerve. Oct 1994;17(10):1145-55. [Medline].

  48. Maher J, Rutledge F, Remtulla H, et al. Neuromuscular disorders associated with failure to wean from the ventilator. Intensive Care Med. Sep 1995;21(9):737-43. [Medline].

  49. Hund EF, Borel CO, Cornblath DR, et al. Intensive management and treatment of severe Guillain-Barre syndrome. Crit Care Med. Mar 1993;21(3):433-46. [Medline].

  50. Roper TA, Alani SM. Recurrent Guillain-Barre syndrome: lightning does strike twice. Br J Hosp Med. Apr 19-May 2 1995;53(8):403-7. [Medline].

  51. Fulgham JR, Wijdicks EF. Guillain-Barre syndrome. Crit Care Clin. Jan 1997;13(1):1-15. [Medline].

  52. Ho TW, Li CY, Cornblath DR, et al. Patterns of recovery in the Guillain-Barre syndromes. Neurology. Mar 1997;48(3):695-700. [Medline].

  53. Koski CL, Patterson JV. Intravenous immunoglobulin use for neurologic diseases. J Infus Nurs. May-Jun 2006;29(3 Suppl):S21-8. [Medline].

  54. McGillicuddy DC, Walker O, Shapiro NI, Edlow JA. Guillain-Barre syndrome in the emergency department. Ann Emerg Med. Apr 2006;47(4):390-3. [Medline].

  55. McLeod JG. Autonomic dysfunction in peripheral nerve disease. J Clin Neurophysiol. Jan 1993;10(1):51-60. [Medline].

  56. Melendez-Vasquez C, Redford J, Choudhary PP, et al. Immunological investigation of chronic inflammatory demyelinating polyradiculoneuropathy. J Neuroimmunol. Mar 1997;73(1-2):124-34. [Medline].

  57. Oomes PG, van der Meche FG, Kleyweg RP. Liver function disturbances in Guillain-Barre syndrome: a prospective longitudinal study in 100 patients. Dutch Guillain-Barre Study Group. Neurology. Jan 1996;46(1):96-100. [Medline].

  58. Pentland B, Donald SM. Pain in the Guillain-Barre syndrome: a clinical review. Pain. Nov 1994;59(2):159-64. [Medline].

  59. Postmarketing surveillance for neurologic adverse events reported after hepatitis B vaccination. Experience of the first three years. Postmarketing surveillance for neurologic adverse events reported after hepatitis B vaccination. Experience of the first three years. Am J Epidemiol. Feb 1988;127(2):337-352.

  60. Rees J. Guillain-Barre syndrome. Clinical manifestations and directions for treatment. Drugs. Jun 1995;49(6):912-20. [Medline].

  61. Rockel A, Wissel J, Rolfs A. Guillain-Barre syndrome in pregnancy--an indication for caesarian section?. J Perinat Med. 1994;22(5):393-8. [Medline].

  62. Rostami AM. Pathogenesis of immune-mediated neuropathies. Pediatr Res. Jan 1993;33(1 Suppl):S90-4. [Medline].

  63. Shahar E. Current therapeutic options in severe Guillain-Barre syndrome. Neuropharmacol. Jan-Feb 2006;29(1):45-51. [Medline].

  64. Vandenbulcke M, Janssens J. Acute axonal polyneuropathy in chronic alcoholism and malnutrition. Acta Neurol Belg. Sep 1999;99(3):198-201. [Medline].

  65. Walk D. Guillain-Barre syndrome. Surg Neurol. Mar 1997;47(3):305-7. [Medline].

Further Reading

Keywords

Guillain-Barre syndrome, Guillain-Barré syndrome, GBS, nervous system, myelin sheath, neuropathy, nerves, causes, symptoms, treatment, viral infection, weakness, autoimmune disease, acute inflammatory demyelinating polyneuropathy, AIDP, acute motor axonal neuropathy, AMAN, acute motor-sensory axonal neuropathy, AMSAN, Miller-Fisher syndrome, MFS, acute panautonomic neuropathy, pharyngeal-brachial-cervical variant, pure sensory variant, Campylobacter jejuni, IVIG, plasmapheresis, acute flaccid paralysis

Contributor Information and Disclosures

Author

Andrew C Miller, MD, Chief Resident and Clinical Assistant Instructor, Departments of Emergency Medicine and Internal Medicine, State University of New York Downstate Medical Center, Kings County Hospital Center
Andrew C Miller, MD is a member of the following medical societies: American College of Emergency Physicians, American College of Physicians, American Medical Association, Emergency Medicine Residents Association, Islamic Medical Association of North America, Medical Society of the State of New York, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Razi M Rashid, MD, MPH, Intern, Department of Internal Medicine, St John's Hospital and Medical Center, Detroit, Michigan
Disclosure: Nothing to disclose.

Richard H Sinert, DO, Associate Professor of Emergency Medicine, Clinical Assistant Professor of Medicine, Research Director, State University of New York College of Medicine; Consulting Staff, Department of Emergency Medicine, Kings County Hospital Center
Richard H Sinert, DO is a member of the following medical societies: American College of Physicians and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Medical Editor

Edward A Michelson, MD, Program Director, Associate Professor, Department of Emergency Medicine, University Hospital Health Systems in Cleveland
Edward A Michelson, MD is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

J Stephen Huff, MD, Associate Professor, Emergency Medicine and Neurology, Department of Emergency Medicine, University of Virginia Health Sciences Center
J Stephen Huff, MD is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Neurology, American College of Emergency Physicians, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Steven C Dronen, MD, FAAEM, Director of Emergency Services, Director of Chest Pain Center, Department of Emergency Medicine, Ft Sanders Sevier Medical Center
Steven C Dronen, MD, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

 
 
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