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Guillain–Barré syndrome in Denmark: a population-based study on epidemiology, diagnosis and clinical severity

Abstract

Objectives

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).

Methods

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.

Results

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).

Conclusion

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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Author information

Correspondence to Helle Al-Hakem.

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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.

Ethical standard

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

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Keywords

  • Guillain–Barré syndrome
  • Epidemiology
  • Brighton criteria
  • International GBS outcome study
  • IGOS