To describe the epidemiology and clinical heterogeneity of Guillain–Barré syndrome (GBS) in Denmark and to compare a population-based cohort to prospectively included patients in the International GBS Outcome Study (IGOS).
The incidence rate (IR) of GBS in Denmark from September 2012 to December 2015, applying the National Institute of Neurological Disorders and Stroke (NINDS) diagnostic criteria, was estimated and the level of diagnostic certainty was described with the Brighton criteria. All cases registered with a diagnosis of GBS or other inflammatory neuropathies in the Danish National Hospital Registry were reviewed for diagnostic criteria and for information on treatment and clinical course.
A total of 299 GBS cases were confirmed, corresponding to a crude IR of 1.59 (95% CI 1.42–1.78) per 100,000 per year. The Brighton criteria level 1–3 of diagnostic certainty was met in 279 (93%) of the patients. Thirty-five percent of the patients were mildly affected (GBS disability score < 3) and a correlation between high age and high disability score at nadir was found (Spearman’s rank correlation coefficient 0.42, p < 0.0001). The group of 89 (30%) patients who were enrolled in IGOS had higher GBS disability score at nadir, were admitted 5 days earlier, reached nadir 4 days faster, and a larger proportion received treatment with IVIg (all p < 0.05).
The epidemiology and full clinical spectrum of GBS are described in a population-based study. This includes a larger proportion of milder cases that are underrepresented in prospective cohorts such as IGOS.
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van den Berg B, Walgaard C, Drenthen J et al (2014) Guillain–Barre syndrome: pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol 10:469–482. https://doi.org/10.1038/nrneurol.2014.121
Dornonville de la Cour C, Jakobsen J (2005) Residual neuropathy in long-term population-based follow-up of Guillain–Barré syndrome. Neurology 64:246–253. https://doi.org/10.1212/01.WNL.0000149521.65474.83
Hughes RA, Swan AV, van Doorn PA (2014) Intravenous immunoglobulin for Guillain–Barré syndrome. In: Hughes RA (ed) Cochrane database of systematic reviews. Wiley, Chichester
Raphaël JC, Chevret S, Hughes RA, Annane D (2002) Plasma exchange for Guillain–Barré syndrome. In: Annane D (ed) Cochrane database of systematic reviews. Wiley, Chichester
Asbury AK, Cornblath DR (1990) Assessment of current diagnostic criteria for Guillain–Barré syndrome. Ann Neurol 27:S21–S24. https://doi.org/10.1002/ana.410270707
Fokke C, van den Berg B, Drenthen J et al (2014) Diagnosis of Guillain–Barré syndrome and validation of Brighton criteria. Brain 137:33–43. https://doi.org/10.1093/brain/awt285
Sejvar JJ, Kohl KS, Gidudu J et al (2011) Guillain–Barré syndrome and Fisher syndrome: case definitions and guidelines for collection, analysis, and presentation of immunization safety data. Vaccine 29:599–612. https://doi.org/10.1016/j.vaccine.2010.06.003
Islam MB, Islam Z, Farzana KS et al (2016) Guillain–Barré syndrome in Bangladesh: validation of Brighton criteria. J Peripher Nerv Syst 21:345–351. https://doi.org/10.1111/jns.12189
Sejvar JJ, Baughman AL, Wise M, Morgan OW (2011) Population incidence of Guillain–Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology 36:123–133. https://doi.org/10.1159/000324710
Jacobs BC, van den Berg B, Verboon C et al (2017) International Guillain–Barre Syndrome Outcome Study (IGOS): protocol of a prospective observational cohort study on clinical and biological predictors of disease course and outcome in Guillain–Barre syndrome. J Peripher Nerv Syst. https://doi.org/10.1111/jns.12209
Doets AY, Verboon C, van den Berg B et al (2018) Regional variation of Guillain–Barré syndrome. Brain 141:2866–2877. https://doi.org/10.1093/brain/awy232
Wakerley BR, Uncini A, Yuki N (2014) Guillain–Barre and Miller Fisher syndromes–new diagnostic classification. Nat Rev Neurol 10:537–544. https://doi.org/10.1038/nrneurol.2014.138
Wakerley BR, Yuki N (2014) Pharyngeal-cervical-brachial variant of Guillain–Barre syndrome. J Neurol Neurosurg Psychiatry 85:339–344. https://doi.org/10.1136/jnnp-2013-305397
Hughes RA, Newsom-Davis JM, Perkin GD, Pierce JM (1978) Controlled trial prednisolone in acute polyneuropathy. Lancet 2:750–753. https://doi.org/10.1016/S0140-6736(78)92644-2
Hadden RDM, Cornblath DR, Hughes RAC et al (1998) Electrophysiological classification of Guillain–Barré syndrome: clinical associations and outcome. Ann Neurol 44:780–788. https://doi.org/10.1002/ana.410440512
Statistics Denmark (2017) Statbank Denmark. http://www.statistikbanken.dk/statbank5a/default.asp?w=1280. Accessed 16 April 2017
Leo CA, Sindrup SH, Gaist D (2010) Unchanged incidence of Guillain–Barre syndrome in the Funen county during a decade. Ugeskr Laeger 172:3093–3097
Sejvar JJ, Baughman AL, Wise M, Morgan OW (2011) Population incidence of Guillain–Barre syndrome: a systematic review and meta-analysis. Neuroepidemiology 36:123–133. https://doi.org/10.1159/000324710
McGrogan A, Madle GC, Seaman HE, de Vries CS (2009) The epidemiology of Guillain–Barré syndrome worldwide. A systematic literature review. Neuroepidemiology 32:150–163. https://doi.org/10.1159/000184748
Hense S, Schink T, Kreisel SH et al (2014) Estimation of background incidence rates of Guillain-Barré syndrome in Germany—a retrospective cohort study with electronic healthcare data. Neuroepidemiology 43:244–252. https://doi.org/10.1159/000369344
Govoni V, Granieri E (2001) Epidemiology of the Guillain–Barré syndrome. Curr Opin Neurol 14:605–613
Benedetti MD, Pugliatti M, D’Alessandro R et al (2015) A multicentric prospective incidence study of Guillain–Barré syndrome in Italy. The ITANG Study. Neuroepidemiology 45:90–99. https://doi.org/10.1159/000438752
Delannoy A, Rudant J, Chaignot C et al (2017) Guillain–Barré syndrome in France: a nationwide epidemiological analysis based on hospital discharge data (2008–2013). J Peripher Nerv Syst 22:51–58. https://doi.org/10.1111/jns.12202
Sipilä JOT, Soilu-Hänninen M, Ruuskanen JO et al (2017) Epidemiology of Guillain–Barré syndrome in Finland 2004–2014. J Peripher Nerv Syst 22:440–445. https://doi.org/10.1111/jns.12239
Peric S, Milosevic V, Berisavac I et al (2014) Clinical and epidemiological features of Guillain–Barre syndrome in the Western Balkans. J Peripher Nerv Syst 19:317–321. https://doi.org/10.1111/jns.12096
Kaplan JE, Schonberger LB, Hurwitz ES, Katona P (1983) Guillain–Barre syndrome in the United States, 1978–1981: additional observations from the national surveillance system. Neurology 33:633–637
Rabie M, Nevo Y (2009) Childhood acute and chronic immune-mediated polyradiculoneuropathies. Eur J Paediatr Neurol 13:209–218. https://doi.org/10.1016/j.ejpn.2008.04.009
Asbury AK, Asbury A, Carrell-Krusen Symposium invited lecture new concepts of Guillain–Barré syndrome
Witsch J, Galldiks N, Bender A et al (2013) Long-term outcome in patients with Guillain–Barré syndrome requiring mechanical ventilation. J Neurol 260:1367–1374. https://doi.org/10.1007/s00415-012-6806-x
Rajabally YA, Uncini A (2012) Outcome and its predictors in Guillain–Barre syndrome. J Neurol Neurosurg Psychiatry 83:711–718. https://doi.org/10.1136/jnnp-2011-301882
van Koningsveld R, Steyerberg EW, Hughes RAC et al (2007) A clinical prognostic scoring system for Guillain–Barré syndrome. Lancet Neurol 6:589–594. https://doi.org/10.1016/S1474-4422(07)70130-8
Walgaard C, Lingsma HF, Ruts L et al (2011) Early recognition of poor prognosis in Guillain–Barre syndrome. Neurology 76:968–975. https://doi.org/10.1212/WNL.0b013e3182104407
Peric S, Berisavac I, Stojiljkovic Tamas O et al (2016) Guillain–Barré syndrome in the elderly. J Peripher Nerv Syst 21:105–110. https://doi.org/10.1111/jns.12163
Flachenecker P (2007) Autonomic dysfunction in Guillain–Barré syndrome and multiple sclerosis. J Neurol 254:II96–II101. https://doi.org/10.1007/s00415-007-2024-3
Anandan C, Khuder SA, Koffman BM (2017) Prevalence of autonomic dysfunction in hospitalized patients with Guillain–Barré syndrome. Muscle Nerve 56:331–333. https://doi.org/10.1002/mus.25551
Hadden RD, Karch H, Hartung HP et al (2001) Preceding infections, immune factors, and outcome in Guillain–Barré syndrome. Neurology 56:758–765. https://doi.org/10.1212/WNL.56.6.758
Zaeem Z, Siddiqi ZA, Zochodne DW (2018) Autonomic involvement in Guillain-Barré syndrome: an update. Clin Auton Res. https://doi.org/10.1007/s10286-018-0542-y
Conflicts of interest
The authors received no specific funding for this work. Thomas Harbo has received speakers’ honoraria from CSL Behring. Bart Jacobs reports grants from Baxalta, grants from Grifols, grants from CSL-Behring, grants from Annexon, grants from Prinses-Beatrix Spierfonds, grants from GBS-CIDP Foundation International, outside the submitted work. The remaining authors report no competing interests.
The manuscript does not contain patient data. Permission to obtain and process data was granted by the Danish Data Protection Agency [reference number 1-16-02-3-16] and the Danish Patient Safety Authority [reference number 3-3013-1449/1/].
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Al-Hakem, H., Sindrup, S.H., Andersen, H. et al. Guillain–Barré syndrome in Denmark: a population-based study on epidemiology, diagnosis and clinical severity. J Neurol 266, 440–449 (2019). https://doi.org/10.1007/s00415-018-9151-x
- Guillain–Barré syndrome
- Brighton criteria
- International GBS outcome study