title: Diagnosis of Guillain-Barré syndrome in children and validation of the Brighton criteria.
abstract: To describe the key diagnostic features of pediatric Guillain-Barré syndrome (GBS) and validate the Brighton criteria. Retrospective cohort study of all children (<18 years) diagnosed with GBS between 1987 and 2013 at Sophia Children's Hospital, Erasmus MC, Rotterdam. Clinical information was collected and the sensitivity of the Brighton criteria was calculated. 67 children (35 boys) were included, with a median age of 5.0 years [interquartile range (IQR) 3.0-10.0 years]. Bilateral limb weakness was present at hospital admission in 93% of children, and at nadir in all patients. Children presented with tetraparesis in 70% or with paraparesis in 23%. Reduced reflexes in paretic limbs were observed at hospital admission in 82% and during follow-up in all children. The progressive phase lasted median 6 days (IQR 3-8 days) and less than 4 weeks in all children. A monophasic disease course was seen in 97%, including 5 children with a treatment-related fluctuation. Two children had a later relapse at 9 weeks and 19 weeks after onset. 77% of the children showed an elevated protein level in CSF. Nerve conduction studies showed evidence for a poly(radiculo)neuropathy in 91% of the children. 46 children had a complete data set, the sensitivity of the Brighton criteria level 1 was 72% (95% CI 57-84) and 96% (95% CI 85-99) for level 2 and 98% (95% CI 88-100) for level 3. The majority of the pediatric GBS patients presented in this cohort fulfilled the current diagnostic criteria.
authors.affiliation: Department of Neurology, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, PO box 2040, 3000 CA, Rotterdam, The Netherlands.|Paediatric Neurology, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, Rotterdam, The Netherlands.
authors.forename: Joyce
authors.initials: J
authors.lastname: Roodbol
authors.affiliation: Paediatric Neurology, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, Rotterdam, The Netherlands.
authors.forename: Marie-Claire Y
authors.initials: MY
authors.lastname: de Wit
authors.affiliation: Department of Neurology, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, PO box 2040, 3000 CA, Rotterdam, The Netherlands.|Department of Neurology, St. Elisabeth Hospital, Tilburg, The Netherlands.
authors.forename: Bianca
authors.initials: B
authors.lastname: van den Berg
authors.affiliation: Department of Neurology, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, PO box 2040, 3000 CA, Rotterdam, The Netherlands.|Paediatric Neurology, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, Rotterdam, The Netherlands.
authors.forename: Vivienne
authors.initials: V
authors.lastname: Kahlmann
authors.affiliation: Department of Neurology, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, PO box 2040, 3000 CA, Rotterdam, The Netherlands.|Clinical Neurophysiology, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, Rotterdam, The Netherlands.
authors.forename: Judith
authors.initials: J
authors.lastname: Drenthen
authors.affiliation: Paediatric Neurology, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, Rotterdam, The Netherlands.
authors.forename: Coriene E
authors.initials: CE
authors.lastname: Catsman-Berrevoets
authors.affiliation: Department of Neurology, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, PO box 2040, 3000 CA, Rotterdam, The Netherlands. b.jacobs@erasmusmc.nl.|Immunology, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, PO box 2040, 3000 CA, Rotterdam, The Netherlands. b.jacobs@erasmusmc.nl.
authors.forename: Bart C
authors.initials: BC
authors.lastname: Jacobs
mesh_terms: D002556:Cerebrospinal Fluid Proteins / Q000134:cerebrospinal fluid; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D020275:Guillain-Barre Syndrome / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D018908:Muscle Weakness / Q000209:etiology; D009431:Neural Conduction / Q000502:physiology*; D011506:Proteins; D012018:Reflex / Q000502:physiology; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric
chemical_list: D002556:Cerebrospinal Fluid Proteins; D016756:Immunoglobulins, Intravenous; D011506:Proteins
keywords: Brighton criteria; Cerebrospinal fluid; Guillain–Barré syndrome; Nerve conduction study; Pediatrics