Pediatric Guillain-Barré Syndrome in a 30-Year Nationwide Cohort

Abstract

Background: Guillain-Barré syndrome is the most common cause of acute flaccid paresis in childhood. Few validated large-scale population-based data are available concerning pediatric Guillain-Barré syndrome, including incidence, risk factors, and initial clinical characteristics.

Methods: In the Danish National Patient Registry, we identified all children aged below 16 years (N = 212) diagnosed with Guillain-Barré syndrome and admitted to any Danish department of pediatrics between 1987 and 2016. A total of 145 (68%) medical files could be retrieved and reviewed, enabling classification of patients with true Guillain-Barré syndrome. The nationwide Guillain-Barré syndrome incidence rate was calculated and stratified by age, gender, time periods, and season. Risk factors and initial Guillain-Barré syndrome characteristics were assessed by medical record review.

Results: The positive predictive value of Guillain-Barré syndrome diagnosis codes was 86%. The crude Guillain-Barré syndrome incidence rate was 0.69 per 100,000 person years and peaked at two years of age. The incidence rate was higher among men (0.80) than women (0.58) and was relatively stable over the 30-year period. No seasonal difference of the incidence rate was found. Of the 125 Guillain-Barré syndrome cases, 63% were preceded by infection, whereas none were preceded by surgery or malignant disease. Medically treated pain was documented in 70%, mainly confined to the lower extremities.

Conclusions: Pediatric Guillain-Barré syndrome diagnoses in the Danish National Patient Registry have high validity, the incidence peaks at age two years, and is preceded by infection in two-thirds of children. Lower extremity pain is a common clinical presentation in the acute setting.

Keywords: GBS characteristics; GBS epidemiology; GBS incidence; GBS risk factors; GBS validation; Pediatric GBS.