Date: Nov 2011, Dr Ng Chung Hoi; Supervisor: Dr So Sheung On
Objectives:
The aim of the present study was to review the clinical outcome of a case series of all GBS patients admitted into three local ICUs.
Design:
This was a retrospective case series review.
Settings:
ICUs in Kwong Wah Hospital (KWH), United Christian Hospital (UCH), and Pamela Youde Nethersole Eastern Hospital (PYNEH) in Hong Kong.
Methods:
Retrieving data by using the Clinical Information System (CIS) and Clinical Management System (CMS) databases of KWH, UCH, and PYNEH, a retrospective review of the medical records of all patients with a diagnosis of GBS admitted into ICUs of these three hospitals over a ten-year period (from 01/11/2000 to 30/10/2010) was performed. Twenty seven patients fulfilling the inclusion criteria were enrolled into the study. Demographic, clinical, laboratory, and electrodiagnostic study data, treatment modalities, and APACHE II scores after ICU admission were collected. The primary outcome measures included the complication rates, all-cause 28-day ICU mortality, ICU length of stay (LOS), hospital LOS, and neurological recovery upon discharge from hospital.
Results:
Of the 27 patients recruited, the mean age was 65.3 years +/- 16.0 and males were slightly predominant (55.6%). Nerve conduction velocity study (NCV) was done in 26 patients: eight had pure demyelinating neuropathy (30.8%), eight had mixed demyelinating and axonal neuropathy (30.8%), six had pure axonal neuropathy (23.1%), and four were normal (15.4%). For the clinical presentation: 21 patients presented with classical GBS, five were diagnosed to be the overlapping syndrome of GBS and Miller-Fisher syndrome (MFS), and one was found to be pharyngeal-cervical-brachial (PCB) variant of GBS by neurologists. The mean APACHE II score was 20.2 +/- 8.7. The median ICU LOS was eight days (1-99), and the median hospital LOS was 27 days (7-257). Concerning treatment modalities:16 patients were treated with intravenous immunoglobulin (IVIg) only (59.2%), nine patients received either IVIg and then plasma exchange (PE), or PE and then IVIg (33.3%) because of poor response to the first therapy, one patient was given PE only (3.7%), and one patient was treated with supportive therapy (3.7%) because of mild impairment. Sixteen patients needed mechanical ventilation (59.3%), and eight of them received tracheostomy (29.6%). Respiratory failure (59.3%), chest infection (55.6%), and autonomic dysfunction (40.7%) were the most common complications encountered in this cohort of patients. Overall, five patients died during ICU stay with the mortality rate of 18.5%, and in the ventilated group the mortality rate was 25%. Of the 22 patients discharged from ICU, their neurological status upon discharge from hospital: three patients walked unaided (13.6%), 16 walked with assistance +/- aid (72.7%), and three remained bedbound (13.6%). Patients with mechanical ventilation (MV) had longer ICU LOS [24.5 days (2-99) vs 5 days (1-8), p = 0.002], and hospital
LOS [36 days (8-257) vs 19 days (7-36), p = 0.005] than those without MV. Patients with MV had higher rates of chest infection (87.5% vs 9.1%, p <0.001) and autonomic dysfunction (62.5% vs 9.1%, p = 0.008) than those without MV. Mechanical ventilated patients with tracheostomy had longer ICU LOS [48.1 days (24-99) vs 8.2 days (2-25), p = 0.001], hospital LOS [112 days (38-257) vs 27.5 days (8-34), p = 0.001], and more ventilator days [47 days (22-198) vs 6 days (1-16), p = 0.001] than those without tracheostomy. A coincidence between peak influenza activity/influenza-like illness (ILI) and the development of GBS was noted in the present study, both occurring in the spring months.
Conclusion:
The clinical outcome of GBS patients admitted into three regional ICUs is presented. The present cohort of patients belongs to a more severe group with higher mechanical ventilation requirement and mortality, and most of the survivors are left with residual weakness. For the association between influenza activity/ILI and the development of GBS, and that between autonomic dysfunction and the increased risk of mechanical ventilation, no causal relationship can be shown because of the small retrospective study. A prospective multi-centre study may be needed to verify these in the future.
References
1. Lawn ND, Fletcher DD, Henderson RD, et al. Anticipating mechanical ventilation in Guillain-Barre syndrome. Arch Neurol 2001; 58:893-8.
2. Sundar U, Abraham E, Gharat A, et al. Neuromuscular respiratory failure in Guillain-Barre syndrome: evaluation of clinical and electrodiagnostic predictors. J Assoc Physicians India 2005; 53:764-8.
3. Durand MC, Porcher R, Orlikowski D, et al. Clinical and electrophysiological predictors of respiratory failure in Guillain-Barre syndrome: a prospective study. Lancet Neurol 2006; 5:1021-8.
4. Dhar R, Stitt L, Hahn AF. The morbidity and outcome of patients with Guillain-Barre syndrome admitted to the intensive care unit. J Neurol Sci 2008; 264:121-128.
5. Orlikowski D, Sharshar T, Porcher R, et al. Prognosis and risk factors of early onset pneumonia in ventilated patients with Guillain-Barre syndrome. Intensive Care Med 2006; 32:1962-1969.
6. Winer JB. Treatment of Guillain-Barre syndrome. QJM 2002; 95: 717-21.
7. Plasma Exchange/Sandoglobulin Guillain-Barre Syndrome Trial Group. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barre syndrome. Lancet. 1997; 349:225-30.
8. Wollinsky K, Hulser P, Brinkmeier H, et al. CSF filtration is an effective treatment of Guillain-Barre syndrome: a randomised clinical trial. Neurology 2001; 57:774-80.
9. Raphael JC, Chevret S, Hughes RAC, et al. Plasma exchange for Guillain-Barre syndrome. Cochrane Review. Cochrane Database Syst Rev 2008:2.
10. Pfeiffer G, Schiller B, Kruse J, et al. Indicators of dysautonomia in severe Guillain-Barre syndrome. J Neurol 1999; 246(11):1015-22.
11. Fisher CM. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N Eng J Med 1956; 255:57-65.
12. Berlit P, Rakicky J. The Miller Fisher Syndrome. Review of the literature. J Clin Neuroophthalmol 1992; 12:57-63.
13. Phillips MS, Stewart S, Anderson JR. Neuropathological findings in Miller Fisher syndrome. J Neurol Neurosurg Psychiatry 1984; 47:492-495.
14. Benito-Leon J, Bravo J, Mateos F, et al. Miller Fisher syndrome in infancy. Childs Nerv Syst 1996; 12:559-561.
15. Chiba A, Kusunoki S, Obata H, et al. Ganglioside composition of the human cranial nerves with special reference to pathophysiology of MSF. Brain Res 1997; 745:32-36.
16. O’Leary CP, William HJ. Autoimmune ataxic neuropathies (sensory ganglionopathies). Curr Opin Neurol 1997; 10:366-370.
17. Chiba A, Kusunoki S, Obata H, et al. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barre syndrome: clinical and immunohistochemical studies. Neurology 1993; 43:1911-17.
18. Ropper AH. Unusual clinical variants and signs in Guillain-Barre syndrome. Arch Neurol 1986; 43: 1150-2.
19. Vellozzi C, Burwen D, Dobardzic A, et al (2009). “Safety of trivalent inactivated influenza vaccines in adults: background for pandemic influenza vaccine safety monitoring”. Vaccine 27:2114-2120.
20. Silverstein A. Pure politics and impure science. Baltimore: The Johns Hopkins Chemistry Press 1981.
21. David Evans, Simon Cauchemez, Frederick G Hayden. “Prepandemic” immunisation for Novel Influenza Viruses, “Swine Flu” Vaccine, Guillain-Barre Syndrome, and the Detection of Rare Severe Adverse Events. The Journal of Infectious Diseases 2009; 200:321-328.
22. Paul Harasim (March 25, 2009). “Flu shots: Vaccine decisions complex – But Nevada’s health officer has no doubts what to do”. Las Vegas Review-Journal.
23. Haber P, Sejvar J, Mikaeloff Y, et al (2009). “Vaccines and Guillain-Barre Syndrome”. Drug Saf 32:309-23.
24. “Influenza/Flu Vaccine”. University of Illinois at Springfield. Morbidity and Mortality Weekly Report (MMWR) (Centres for Disease Control and Prevention).
25. “Preliminary Results: Surveillance for Guillain-Barre Syndrome after Receipt of Influenza A (H1N1) 2009 Monovalent Vaccine – United States, 2009 – 2010”. Morbidity and Mortality Weekly Report (MMWR) (Centres for Disease Control and Prevention).
26. Last Year’s [2009’s] H1N1 Flu Vaccine Was Safe, Study Finds Wednesday, February 2, 2011.
27. Landry O: Note sur la paralysicascendante aigue. GazRebd Med Chir 1859; 6:472,486.
28. Guillain G, Barre JA, Strohl A. Sur un syndrome de radiculo-nevrite avec hyperalbuminose du liquide cephalorachidien sans reaction cellulaire: 31
remarques sur les characteres clinique et graphique des reflexes tendinaux. Bull Soc Med Hop 1916; 40:1462-70.
29. Hughes RAC (1990) Guillain-Barre Syndrome. Heidelberg. Germany: Springer-Verlaq.
30. Ropper AH, Wijdicks EFM, Traux BT (Eds.) (1991). Guillain-Barre Syndrome. Philadelphia, PA, USA: FA Davis.
31. Alter M (1990). The Epidemiology of Guillain-Barre syndrome. Ann Neurol 27:S7-12.
32. Guillain-Barre syndrome Steroid Group (1993). Double blind trial of intravenous methylprednisolone in Guillain-Barre syndrome. Lancet 341:586-590.
33. McLean M, Duclos P, Jacob P, et al (1994). Incidence of Guillain-Barre syndrome in Ontario and Quebec, 1983-1989, using hospital service databases. Epidemiology 5:443-448.
34. Jiang GX, Cheng Q, Link H, et al (1997). Epidemiological features of Guillain-Barre syndrome in Sweden, 1978-93. Journal of Neurology Neurosurgery and Psychiatry 62:447-453.
35. Emilia-Romagna Study Group on Clinical and Epidemiological Problems in Neurology (1997). A prospective study on the incidence and prognosis of Guillain-Barre syndrome in Emilia-Romagna region, Italy (1992-1993). Neurology 48:214-221.
36. Rees JH, Thompson RD, Smeeton NC, et al (1998). Epidemiological study of Guillain-Barre syndrome in southeast England. Journal of Neurology Neurosurgery and Psychiatry 64:74-77.
37. Lawn ND, Wijdicks EF (1999). Fatal Guillain-Barre syndrome. Neurology 52:635-638.
38. Fletcher DD, Lawn ND, Wolter TD, et al (2000). Long-term outcome in patients with Guillain-Barre syndrome requiring mechanical ventilation. Neurology 54:2311-2315.
39. Dumitru D, Amato AA, Zwarts MJ. Electrodiagnostic Medicine, 2nd ed. Philadelphia: Hanley & Belfus Inc, 2002.p.937-56.
40. Mendel JR, Kissel JT, Cornblath DR. Diagnosis and Management of Peripheral Nerve Disorders. New York, Oxford: Oxford University Press, 2001.p.145-72.
41. Govoni V, Granieri E. Epidemiology of the Guillain-Barre syndrome. Curr Opin Neurol 2001; 14:605-13.
42. Winer JB. Guillain-Barre syndrome; BMA. Mol Pathol 2001; 54:381-5.
43. Aminoff MJ. Electromyography in Clinical Practice, 3rd ed. New York: Churchill Livingstone, 1998.
44. Tsang, RS. The relationship of Campylobacter jejuni infection and the development of Guillain-Barre syndrome. Curr Opin Infect Dis 2002; 15: 221-8.
45. Hartung HP, Kieseier BC, Kiefer R. Progress in Guillain-Barre. Curr Opin Neurol 2001; 14: 597-604.
46. Hiraga A, Mori M, Ogawara K, et al. Recovery patterns and long term prognosis for axonal Guillain-Barre syndrome. J Neurol Neurosurg Psychiatry 2005; 76: 719-22.
47. Hauck LJ, White C, Feasby TE, et al. Incidence of Guillain-Barre syndrome in Alberta, Canada: an administrative data study. J Neurol Neurosurg Psychiatry 2008: 79: 318-20.
48. Hughes RA, Choudry PP, Osbron M, et al. Immunisation and the risk of relapse of Guillain-Barre syndrome or chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 1996; 19: 1230-1.
49. Henderson RD, Lawn ND, Fletcher DD, et al. The morbidity of Guillain-Barre syndrome admitted to the intensive care unit. Neurology 2003; 60: 17-21.
50. Van Koningsveld R, Schmitz PIM, Ang CW, et al. Infections and the course of disease in mild forms of Guillain-Barre syndrome. Neurology 2002; 58: 610-4.
51. Green DM, Ropper AH. Mild Guillain-Barre syndrome. Arch Neurol 2001; 58: 1098-101.
52. De la Cour CD, Jakobsen J. Residual neuropathy in long term population-based follow-up of Guillain-Barre syndrome. Neurology 2005; 64: 246-53.
53. Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barre syndrome. Ann Neurol. 1990; 27: S21-4.