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Guillain-Barrés syndrom

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Definition:
Akut polyneuropati av autoimmun orsak. Oftast efter infektionssjukdom.
Förekomst:
0,8–1,9 fall per 100 000 personer årligen
Symtom:
Akut insättande svaghet, domningar, parestesier och/eller smärtor i extremiteter, symmetriskt lokaliserat.
Kliniska fynd:
Pareser och försvagade reflexer, nedsatt perifer sensibilitet. Ofta även autonom påverkan.
Diagnostik:
Klinisk bild, analys av cerebrospinalvätska och neurofysiologisk undersökning. I vissa fall även MR av ryggen och nervrötter.
Behandling:
Sjukhusinläggning (oftast) med övervakning av vitala funktioner. Intravenöst immunglobulin (IVIg) eller plasmaferes.
  1. Walling AD, Dickson G. Guillain-Barré syndrome. Am Fam Physician 2013; 87: 191-7. American Family Physician  
  2. Leonard SE, Mandarakas MR, Gondim FAA, et al. Diagnosis and management of Guillain–Barré syndrome in ten steps. Nature Reviews Neurology 2019; 15: 671–683 .
  3. Winer JB. Guillain-Barre syndrom. Clinical review. BMJ 2008; 337: a671. PubMed  
  4. Lindenbaum Y, Kissel JT, Mendell JR. Treatment approaches for Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy. Neuro Clin 2001; 19: 187-204. PubMed  
  5. Newswanger DL, Warren CR. Guillain-Barré syndrome. Am Fam Physician 2004; 69: 2405-10. PubMed  
  6. Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet. 2016 Aug 13;388(10045):717-27. PMID: 26948435 PubMed  
  7. Sejvar JJ, Baughman AL, Wise M, Morgan OW. Incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology 2011; 36: 123-33.
  8. Leonhard SE, van der Eijk AA, Andersen H, et al. An International Perspective on Preceding Infections in Guillain-Barré Syndrome The IGOS-1000 Cohort. Neurology 2022. pmid:35981895 PubMed  
  9. Leonhard SE, Mandarakas MR, Gondim FAA, Bateman K, Ferreira MLB, Cornblath DR, van Doorn PA, Dourado ME, Hughes RAC, Islam B, Kusunoki S, Pardo CA, Reisin R, Sejvar JJ, Shahrizaila N, Soares C, Umapathi T, Wang Y, Yiu EM, Willison HJ, Jacobs BC. Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 2019 Nov;15(11):671-683. doi: 10.1038/s41582-019-0250-9. Epub 2019 Sep 20. PMID: 31541214 PubMed  
  10. Shahrizaila N, Lehmann HC, Kuwabara S. Guillain-Barré syndrome. Lancet. 2021 Mar 27;397(10280):1214-1228. doi: 10.1016/S0140-6736(21)00517-1. Epub 2021 Feb 26. PMID: 33647239 PubMed  
  11. Rudolph T, Larsen JP, Farbu E. The long-term functional status in patients with Guillain-Barré syndrome. Eur J Neurol. 2008 Dec. 15(12):1332-7. www.ncbi.nlm.nih.gov  
  12. Zaeem Z, Siddiqi ZA, Zochodne DW. Autonomic involvement in Guillain-Barré syndrome: an update. Clin Auton Res. 2019 Jun;29(3):289-299. PMID: 30019292 PubMed  
  13. Behandling av Guillain-Barrés syndrom. Janusinfo. Region Stockholm. Hämtad 2020-12-03 www.janusinfo.se  
  14. Nobuhiro Y, Hartung H-P. Guillain-Barré syndrome. N Engl J Med 2012; 366: 2294-304. New England Journal of Medicine  
  15. Dash S, Pai AR, Kamath U, et al. . Pathophysiology and diagnosis of Guillain-Barré syndrome - challenges and needs. Int J Neurosci. 2015. pmid:24731000 PubMed  
  16. Hughes RA, Raphaël JC, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2014 Sep 19;(9):CD002063. PMID: 25238327 PubMed  
  17. Walgaard C, Jacobs BC, Lingsma HF et al. Second intravenous immunoglobulin dose in patients with Guillain-Barré syndrome with poor prognosis (SID-GBS): a double-blind, randomised, placebo-controlled trial. Lancet Neurol. 2021 Apr;20(4):275-283. doi: 10.1016/S1474-4422(20)30494-4. Epub 2021 Mar 17. PMID: 33743237 PubMed  
  18. Raphaël JC, Chevret S, Hughes RAC, Annane D. Plasma exchange for Guillain-Barré syndrome. Cochrane Database of Systematic Reviews 2012, Issue 7. Art. No.: CD001798. DOI: 10.1002/14651858.CD001798.pub2. DOI  
  19. Liu J, Wang LN, McNicol ED. Pharmacological treatment for pain in Guillain-Barré syndrome. Cochrane Database Syst Rev. 2015 Apr 9;2015(4):CD009950. PMID: 25855461 PubMed  
  20. Hughes RA, Brassington R, Gunn AA, et al. Corticosteroids for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2016 Oct 24;10:CD001446. Epub ahead of print. pmid: 27775812 PubMed  
  21. van Koningsveld R, Schmitz PIM, van der Meché FGA, et al. Effect of methylprednisolone when added to standard treatement with intravenous immunoglobulin for Guillain-Barré syndrome: randomised trial. Lancet 2004; 363: 192-6. PubMed  
  22. Vriesendorp FJ. Guillain-Barré syndrome in adults: Clinical features and diagnosis. UpToDate, last updated Dec 04, 2018. UpToDate  
  23. Wijdicks EF, Henderson RD, McClelland RL. Emergency intubation for respiratory failure in Guillain-Barre syndrome. Arch Neurol 2003; 60: 947-8. PubMed  
  24. The prognosis and main prognostic indicators of Guillain-Barré syndrome: a multicentre prospective study of 297 patients. The Italian Guillain-Barré Study Group. Brain 1996; 119(pt 6): 2053-61.
  25. Korinthenberg R, Monting JS. Natural history and treatment effects in Guillain-Barré syndrome: a multicentre study. Arch Dis Child 1996; 74: 281. PubMed  
  26. Merkies IS, Schmitz PI, Samijn JP, van der Meche FG, van Doorn PA. Fatigue in immune-mediated polyneuropathies. Neurology 1999; 53: 1648-54. Neurology  
  27. Fletcher DD, Lawn ND, Wolter TD, Wijdicks EF. Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation. Neurology 2000; 54: 2311-5. PubMed  
  28. Lawn ND, Wijdicks EF. Fatal Guillain-Barré syndrome. Neurology 1999; 52: 635-8. Neurology  
  29. Khan F, Pallant JF, Ng L, Bhasker A. Factors associated with long-term functional outcomes and psychological sequelae in Guillain-Barre syndrome. J Neurol. 2010 Dec. 257(12):2024-31. www.ncbi.nlm.nih.gov  
  30. Das A, Kalita J, Misra UK. Recurrent Guillain Barre' syndrome. Electromyogr Clin Neurophysiol. 2004 Mar. 44(2):95-102. www.ncbi.nlm.nih.gov  
  31. Koga M, Takahashi M, Masuda M, Hirata K, Yuki N. Campylobacter gene polymorphism as a determinant of clinical features of Guillain-Barré syndrome. Neurology. 2005 Nov 8. 65(9):1376-81. www.ncbi.nlm.nih.gov  
  32. Azim D, Nasim S, Kumar S, Hussain A, Patel S. Neurological Consequences of 2019-nCoV Infection: A Comprehensive Literature Review. Cureus. 2020 Jun 24;12(6):e8790. PMID: 32601577 PubMed  
  33. Gordon PH, Wilbourn AJ. Early electrodiagnostic findings in Guillain-Barré syndrome. Arch Neurol 2001; 58: 913-7. PubMed  
  34. Ropper AH, Wijdicks EF, Shahani BT. Electrodiagnostic abnormalities in 113 consecutive patients with Guillain-Barré syndrome. Atch Neurol 1990; 47: 881-7. PubMed  
  35. Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2017 Feb 27;2:CD001798. PMID: 28241090 PubMed  
  • Ivan Kmezic, specialist i neurologi, Karolinska Universitetssjukhuset
  • Anna Nager, docent och specialist i allmänmedicin, Karolinska institutet

Tidigare sakkunniga

  • Ingard Løge, spesialist allmennmedisin, Trondheim