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      Distinct Clinical Characteristics of Pediatric Guillain-Barré Syndrome: A Comparative Study between Children and Adults in Northeast China

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          Abstract

          Objective

          Clinical characteristics of pediatric Guillain-Barré syndrome (GBS) have been extensively studied whereas scarcely been compared with those of adult GBS. Herein we compared the clinical features of GBS between pediatric and adult patients.

          Methods

          We retrospectively collected the clinical data of 750 patients with GBS (541 adults and 209 children), and compared the clinical characteristics between children and adults.

          Results

          Pain was a more frequent complaint in children (17.2% vs 9.6%, p < 0.01), who were also found with shorter interval from disease onset to nadir (6.3d vs 7.3d, p < 0.01) and higher incidence of bulbar dysfunction (22.0% vs 14.8%, p < 0.05). The disease severity in children was comparable with adults. In addition, a higher incidence of pediatric GBS was found in summer, especially in July and August (both p < 0.01). However, the incidence of antecedent infections of different seasons in adult and pediatric patients was comparable ( p > 0.05). The clinical features of acute motor axonal neuropathy (AMAN) and acute inflammatory demyelinating polyneuropathy (AIDP) in children were overall comparable with adult ones ( p > 0.05). Similar to adults, bulbar dysfunction (odds ratio [OR]: 4.621, 95% confidence interval [CI]: 1.240–17.218, p < 0.05) and lower nadir Medical Research Council (MRC) sum score (OR: 0.897, 95% CI: 0.855–0.941, p < 0.01) were also risk factors for mechanical ventilation in children. However, distinct from adult ones, autonomic dysfunction was significantly higher in mechanically ventilated childhood GBS (39.1% vs 8.8%, p < 0.01), which also served as a predictor for mechanical ventilation in pediatric GBS (OR: 70.415, 95% CI: 9.265–535.158, p < 0.01). As to the efficacy of intravenous immunoglobulin, insignificant difference was identified between children and adults.

          Conclusion

          The clinical features of pediatric GBS differ from those of adults. Autonomic dysfunction is an independent risk factor for mechanical ventilation in pediatric patients.

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          Patterns of Guillain-Barre syndrome in children: results from a Mexican population.

          Patricia M T Garcia, Luis Garcia, Daniel E C Perez … (2007)
          Guillain-Barré syndrome (GBS) is an acute, immune-mediated flaccid paralysis frequently associated with Campylobacter infection. Of two predominant GBS subtypes, a demyelinating subtype (acute inflammatory demyelinative polyneuropathy [AIDP]) predominates in the United States and Europe, and axonal subtype (acute motor axonal neuropathy [AMAN]) is the predominant form in China. Previous clinical studies suggested that AMAN also occurs in Mexican children. The purpose of this study was to describe the subtypes of GBS in children from Mexico City. We prospectively studied 121 children admitted to two pediatric hospitals in Mexico City from 1996 to 2002. Clinical histories were obtained, electrophysiologic studies were performed to determine GBS subtype, and microbiologic studies were performed. Of the 121 children, 46 had AMAN and 32 had AIDP. The male to female ratio was 1.3 for AMAN cases (mean age = 6.3) and 3.0 for AIDP cases (mean age = 7.0). There was a strong seasonal distribution of AMAN cases in July to September. Children with AMAN, but not AIDP, had worsening of illness during hospitalization as judged by peak severity scores. Vomiting was more likely in AIDP (28.1%) vs AMAN (6.5%) (p = 0.012) and diarrhea was more common in AMAN (32.6%) than AIDP (12.5%) (p = 0.06). IgG anti-GM1 antibody titers were higher in patients with AMAN vs AIDP (p = 0.067). Anti-GD1a antibodies were equally present in both groups. Anti GQ1b titers were higher in AMAN vs AIDP (p = 0.009). Campylobacter antibody responses were positive in 44.1% of patients with AMAN and 37.0% of patients with AIDP. Twenty patients (14 = AMAN, 6 = AIDP) had positive stool cultures for C jejuni. Two serotypes, HS:19 and HS:41, accounted for 6 of 10 Campylobacter isolates available for serotyping from these cases. This study confirms that acute motor axonal neuropathy is an important Guillain-Barré syndrome subtype in Mexican children, is associated with diarrhea, and occurs seasonally.
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            Predictors for mechanical ventilation and short-term prognosis in patients with Guillain-Barré syndrome

            Xiujuan Wu, Chunrong Li, Bing Zhang … (2015)
            Introduction Guillain-Barré syndrome (GBS) is an immune-mediated disorder of the peripheral nervous system. Respiratory failure requiring mechanical ventilation (MV) is a serious complication of GBS. Identification of modifiable risk factors for MV and poor short-term prognosis in mechanically ventilated patients with GBS may contribute to the individualized management and may help improve the outcome of the patients. Methods We retrospectively analyzed the clinical data of 541 patients who were diagnosed with GBS from 2003 to 2014. Independent predictors for MV and short-term prognosis in mechanically ventilated patients were identified via multivariate logistic regression analysis. Results The mean age was 41.6 years with a male predilection (61.2 %). Eighty patients (14.8 %) required MV. Multivariate analysis revealed that shorter interval from onset to admission (p < 0.05), facial nerve palsy (p < 0.01), glossopharyngeal and vagal nerve deficits (p < 0.01) and lower Medical Research Council (MRC) sum score at nadir (p < 0.01) were risk factors for MV; disease occurrence in summer (p < 0.01) was a protective factor. As to prognostic factors, absence of antecedent infections (p < 0.01) and lower MRC sum score at nadir (p < 0.01) were predictors of poor short-term prognosis in mechanically ventilated patients regardless of treatment modality. We further investigated the predictors of poor short-term prognosis in patients requiring MV with different nadir MRC sum scores. Combined use of intravenous corticosteroids with intravenous immunoglobulin (odds ratio 10.200, 95 % confidence interval 1.068–97.407, p < 0.05) was an independent predictor of poor short-term prognosis in mechanically ventilated patients with a nadir MRC sum score from 0 to 12 points, regardless of existence of antecedent infection. Conclusions Clinical predictors of MV and poor short-term prognosis in mechanically ventilated GBS patients were distinct. Add-on use of intravenous corticosteroids was a risk factor for poor short-term prognosis in mechanically ventilated patients with a nadir MRC sum score from 0 to 12 points.
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              Epidemiological, clinical, and electrodiagnostic findings in childhood Guillain-Barré syndrome: a reappraisal.

              G Paradiso, N Fejerman, J Tripoli … (1999)
              We evaluated 61 children with Guillain-Barré syndrome, 14 months to 14 years of age, admitted to the Hospital Nacional de Pediatria in Buenos Aires. According to the electrodiagnostic findings, they fit into two groups, those with acute motor axonal neuropathy (AMAN) (18 patients) and those with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (43 patients). Ninety percent of the children with AMAN resided in suburban or rural areas without running water, whereas half of the AIDP patients lived in a metropolitan district. Summer and winter months showed a higher incidence of both variants. Children with AMAN were younger, evolved more acutely, reached a higher maximum disability score, required assisted ventilation more often, had lower mean level of cerebrospinal fluid protein, improved more slowly, and had a poorer outcome 6 months and 12 months after onset. Electrophysiological findings in those with AIDP revealed a pattern of severe diffuse slowing in children 5 years old or younger and a multifocal pattern in children 6 years old or older. This difference was not reflected in the clinical picture. In contrast, AMAN showed a uniform pattern with normal sensory conduction, severely reduced compound muscle action potential amplitude, near normal conduction velocity, and early denervation. Epidemiological, clinical, electrodiagnostic, cerebrospinal fluid, and prognostic data indicate that these variants of Guillain-Barré syndrome should be regarded as different entities.
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                Author and article information

                Contributors
                Martin Stangel: Role: Editor
                Journal
                Journal ID (nlm-ta): PLoS One
                Journal ID (iso-abbrev): PLoS ONE
                Journal ID (publisher-id): plos
                Journal ID (pmc): plosone
                Title: PLoS ONE
                Publisher: Public Library of Science (San Francisco, CA USA )
                ISSN (Electronic): 1932-6203
                Publication date (Electronic): 14 March 2016
                Publication date Collection: 2016
                Volume: 11
                Issue: 3
                Electronic Location Identifier: e0151611
                Affiliations
                [1 ]Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China
                [2 ]Department of Pediatric Surgery, the First Hospital of Jilin University, Jilin University, Changchun, China
                Hannover Medical School, GERMANY
                Author notes

                Competing Interests: The authors have declared that no competing interests exist.

                Conceived and designed the experiments: H-LZ KL XW. Performed the experiments: XW DS TL. Analyzed the data: XW BZ CL MM. Contributed reagents/materials/analysis tools: JZ. Wrote the paper: XW KL H-LZ.

                Article
                Publisher ID: PONE-D-15-52362
                DOI: 10.1371/journal.pone.0151611
                PMC ID: 4790924
                PubMed ID: 26974666
                SO-VID: 896bffab-e599-4350-bdd8-5e6bcb4186ca
                Copyright © © 2016 Wu et al
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date received : 2 December 2015
                Date accepted : 1 March 2016
                Page count
                Figures: 2, Tables: 6, Pages: 12
                Funding
                The work was supported by grants from the National Natural Science Foundation of China (No. 81271294 and 81241147), the Young Scholars Program of Norman Bethune Health Science Center of Jilin University (No. 2013205035), the Young Scholars Program of the First Hospital of Jilin University (No. JDYY42013003), and the Scientific Research Foundation for the Returned Overseas Chinese Scholars, State Education Ministry (3C113BK73428).
                Categories
                Subject: Research Article
                Subject: People and Places
                Subject: Population Groupings
                Subject: Age Groups
                Subject: Adults
                Subject: Medicine and Health Sciences
                Subject: Pediatrics
                Subject: People and Places
                Subject: Population Groupings
                Subject: Age Groups
                Subject: Children
                Subject: People and Places
                Subject: Population Groupings
                Subject: Families
                Subject: Children
                Subject: Medicine and Health Sciences
                Subject: Pediatrics
                Subject: Pediatric Infections
                Subject: Medicine and Health Sciences
                Subject: Pulmonology
                Subject: Respiratory Infections
                Subject: Medicine and Health Sciences
                Subject: Biology and Life Sciences
                Subject: Anatomy
                Subject: Nervous System
                Subject: Nerves
                Subject: Cranial Nerves
                Subject: Medicine and Health Sciences
                Subject: Anatomy
                Subject: Nervous System
                Subject: Nerves
                Subject: Cranial Nerves
                Subject: Biology and Life Sciences
                Subject: Physiology
                Subject: Sensory Physiology
                Subject: Somatosensory System
                Subject: Pain Sensation
                Subject: Medicine and Health Sciences
                Subject: Physiology
                Subject: Sensory Physiology
                Subject: Somatosensory System
                Subject: Pain Sensation
                Subject: Biology and Life Sciences
                Subject: Neuroscience
                Subject: Sensory Systems
                Subject: Somatosensory System
                Subject: Pain Sensation
                Custom metadata
                Data Availability All relevant data are within the paper.

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                ScienceOpen disciplines: Uncategorized

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