Diagnosis, Treatment and Prognosis of Guillain-Barré Syndrome in Children

Research Article | DOI: https://doi.org/10.31579/2767-7370/166

Diagnosis, Treatment and Prognosis of Guillain-Barré Syndrome in Children

  • Faruk Incecik 1*
  • Murat Turhan 2
  • Özlem M. Herguner 2

1 Department of Pediatric Neurology, Bakırcay University Faculty of Medicine, İzmir, Turkey.
2 Department of Pediatric Neurology, Cukurova University Faculty of Medicine, Adana, Turkey.

*Corresponding Author: Faruk Incecik. Department of Pediatric Neurology, Bakırcay University Faculty of Medicine, İzmir, Turkey.

Citation: Faruk Incecik , Murat Turhan , Özlem M. Herguner, (2025), Diagnosis, Treatment and Prognosis of Guillain-Barré Syndrome in Children, J New Medical Innovations and Research, 6(4); DOI:10.31579/2767-7370/166

Copyright: © 2025, Faruk Incecik. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Received: 16 April 2025 | Accepted: 21 April 2025 | Published: 25 April 2025

Keywords: guillain-barre syndrome; prognosis; children

Abstract

Aim: Guillain-Barre syndrome (GBS) is one of the reasons of acute polyneuropathy causing severe morbidity and mortality. In this study, it was aimed to identify the clinical findings, treatment modalities and factors affecting the prognosis of patients who were followed up and treated with GBS.

Material and Method: A total of 47 patients diagnosed and treated with GBS between 2006 and 2016 were evaluated in Child Health and Diseases Services. Parameters such as age, gender, presenting complaints, previous infection history, seasonal distribution, cranial nerve involvement, presence of autonomic symptoms, muscle weakness, need for respiratory support, electroneuromyography (ENMG) findings, length of stay in the intensive care unit and general wards, and treatment were investigated in the patients.

Results: Twenty nine (61,7%) of the patients were male, 18 (38,3%) were female and the mean age was 7,94±4,49 years. The complaints of the cases observed in the application; 44 patients (93,6%) had weakness and 3 (6,4%) patient had numbness in the feet. Twenty seven (57,4%) of the 47 patients had prior history of infection. Upper respiratory tract infections and gastrointestinal system infections were found in 14 (51,9%) and 12 (44,5%) cases respectively. Gender, presence of infection, type of infection, cranial nerve involvement, presence of autonomic symptoms, and subtype of GBS were not found to be prognostic. The duration of stay in intensive care unit was 9,43±7,81 days and the total length of stay in hospital was 19,32±14,62 days (p= 0,009). The need for respiratory support and intravenous immunoglobulin (IVIG) treatment were found to be effective factors on prognosis. 

Conclusion: While the need for respiratory support in patients with GBS was a poor prognostic factor, it was observed that IVIG treatment alone could be associated with good prognosis. There is a need for extensive research to determine prognostic factors.

Introduction

Guillain-Barré Syndrome (GBS) is an acute inflammatory polyneuropathy characterized by rapidly progressive, ascending, symmetrical weakness and areflexia. Its global incidence ranges from 0.6 to 2.4 per 100,000 individuals annually [1]. GBS can affect individuals of all ages and both sexes. Symptoms typically begin in the lower extremities and progress to the trunk and upper limbs within a few days. While progression is usually symmetrical, in rare cases, an asymmetrical onset may occur. Sensory loss, autonomic dysfunction, cranial nerve involvement, and neuropathic pain frequently accompany the clinical presentation.

GBS symptoms commonly appear following a viral or bacterial infection; more rarely, the condition may develop after vaccination [1]. There are both demyelinating and axonal variants of GBS, with differing prevalence across geographical regions. Acute inflammatory demyelinating polyneuropathy (AIDP) is the most common subtype in North America and Europe, accounting for approximately 90% of all cases [2]. In contrast, acute motor axonal neuropathy (AMAN) and acute motor and sensory axonal neuropathy (AMSAN) are more prevalent in Central and South America as well as in Asia [3].

The prognosis is generally more favorable in children than in adults. Several studies have investigated the factors influencing the prognosis of pediatric GBS [4-6]; however, data on childhood GBS cases remain limited. Identifying prognostic factors is crucial for optimizing treatment and follow-up strategies. The objective of this study was to evaluate the demographic and clinical characteristics, treatment approaches, and prognostic outcomes in pediatric GBS cases.

Materials and Methods

This retrospective study included 47 patients who were diagnosed with Guillain-Barré syndrome and received treatment and follow-up care between 2006 and 2016. Patient records were reviewed, and a standardized data collection form was completed for each individual. Ethical approval for the study was obtained from the institutional Ethics Committee.

Data collected included age, gender, presenting complaints, clinical findings at admission, history of preceding infection (including the type of infection), seasonal distribution, cranial nerve involvement, presence of autonomic symptoms, severity of muscle weakness, need for respiratory support, electroneuromyography (ENMG) findings, duration of stay in the intensive care unit and general wards, treatment protocols, and clinical status and examination findings at both discharge and two months post-discharge.

The examination findings of the patients at the time of admission and at the two-month follow-up after discharge were made according to the scale of Hughes et al [7]

0 - No findings

1 - Minor findings

2 - Walking without support

3 - Walking with support

4 - Bedridden

5 - Requiring respiratory support

6 - Death 

Electroneuromyography was performed between 7 and 20 days after the onset of symptoms. Based on clinical and ENMG findings, patients were classified into three subtypes of GBS: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor and sensory axonal neuropathy (AMSAN). Autonomic symptoms were defined as the presence of high or low blood pressure, urinary retention, and cardiac dysrhythmias.

To evaluate prognostic factors, patients were divided into two groups (G1 and G2) based on their clinical findings at the follow-up visit conducted two months after discharge. 

G1: 0 - No findings, 1 - Minor findings

G2: 2 - Walking without support, 3 - Walking with support, 4 – Bedridden, 5 - Requiring respiratory support, 6 – Death.                

Treatment modalities were categorized as either intravenous immunoglobulin (IVIG) alone or a combination of IVIG and plasmapheresis. The length of stay in both the intensive care unit and general wards was also recorded.

Demographic and clinical data of all 47 patients were retrospectively analyzed using the statistical software package "SPSS for Windows, version 21.0". Descriptive statistics, t-tests, and chi-square tests were employed for data analysis. A p-value of <0>

Results

This study included 47 patients diagnosed with Guillain-Barré syndrome. Of these, 29 were male (61.7%) and 18 were female (38.3%), with a mean age of 7.94 ± 4.49 years. Thirty-two patients (68.1%) were under 10 years of age, and 15 (31.9%) were over 10 years of age. Among those under ten years, 20 patients (62.5%) were classified in Group 1 (G1) and 12 (37.5%) in Group 2 (G2). Of the patients over 10 years of age, 10 (66.7%) were in G1 and 5 (33.3%) in G2.

A history of preceding infection was reported in 57.4% of the patients, the majority of which were upper respiratory tract infections (URTIs). The disease most frequently occurred in the spring.

Initial clinical evaluations showed that 44 patients (93.6%) presented with limb weakness, while 3 (6.4%) reported numbness. Muscle weakness was symmetrical in all patients. In 16 patients (34.0%), only the lower extremities were affected, while 31 patients (66.0%) exhibited involvement of both lower and upper extremities. Cranial nerve involvement was observed in 10 patients (21.3%), and autonomic symptoms were noted in 8 patients (17.0%). Among those with autonomic involvement, 6 patients (75%) exhibited blood pressure fluctuations. One patient had both blood pressure changes and urinary retention, while 3 patients (37.5%) had only urinary retention. Respiratory support was required in 13 patients (27.7%).

Among the 47 patients, 18 (38.3%) were diagnosed with AIDP, 18 (38.3%) with AMAN, and 11 (23.4%) with AMSAN with electrophysiological findings.

Regarding treatment, 25 patients (53.2%) received intravenous immunoglobulin (IVIG) alone, while 22 patients (46.8%) received a combination of IVIG and plasmapheresis. General characteristics of the patients are presented in Table 1.

The mean length of stay in the intensive care unit was 9.43 ± 7.81 days, and the mean total hospital stay was 19.32 ± 14.62 days. 

For prognostic evaluation, patients were divided into two groups based on clinical findings at the two-month follow-up visit after discharge. Thirty patients (63.8%) were classified as G1 and 17 patients (36.2%) as G2. When evaluating prognostic factors, no statistically significant relationship was found between prognosis and variables such as gender, age, history of prior infection, type of infection, seasonal distribution, cranial nerve involvement, or the presence of autonomic symptoms. However, the need for respiratory support and the type of treatment administered were found to have a statistically significant impact on prognosis. Prognostic factors are summarized in Table 2.

Discussion

Guillain-Barré Syndrome is a common, immune-mediated, acute inflammatory demyelinating polyradiculopathy affecting the peripheral nerves. In the pediatric population, GBS is more prevalent in boys than in girls, with the incidence approximately 1.5 times higher in boys (8). In a study by Korinthenberg et al. (9), the female-to-male ratio was 1:1.27, and the age range was between 11 months and 17.7 years. In our study, 29 patients (61.7%) were male and 18 (38.3%) were female, with a female-to-male ratio of 1:1.6 and a mean age of 7.94 ± 4.49 years, which aligns with the existing literature.

Approximately two-thirds of GBS patients report a preceding infection. Upper respiratory tract infections (URTIs) are the most common, followed by gastroenteritis caused by Campylobacter jejuni (10). In a study by Varkal et al. (11), 32 out of 40 patients (80%) had a history of preceding infection, with 21 cases (65.6%) being URTIs and 11 (34.4%) gastroenteritis. In our study, 57.4% of patients had a preceding infection: 14 patients (51.9%) had URTIs, 12 (44.5%) had gastrointestinal infections, and 1 patient (3.6%) had a history of vaccination  Additionally, most patients presented in the spring.

In typical GBS, rapidly progressive bilateral weakness is the hallmark symptom. The weakness classically begins in the distal lower extremities and ascends proximally. However, it may sometimes begin proximally or in the upper extremities. Akbayram et al. (13) reported that muscle weakness was the initial symptom in 34 of 36 patients (94.4%), with muscle pain reported in 8, dysarthria in 3, and numbness in 2. Similarly, in our study, 44 patients (93.6%) presented with limb weakness and 3 (6.4%) with numbness in the feet.

GBS is classified into axonal and demyelinating subtypes based on clinical and electrophysiological findings. AIDP is the most common clinical form. Previous studies have suggested differences in clinical features and prognosis among subtypes [14]. Akbayram et al. [13] reported that 25 of 36 patients (69.4%) had AIDP, 10 (27.8%) had AMAN, and 1 (2.8%) had AMSAN, with no significant difference in prognosis between subtypes. Nagasawa et al [15] found that the AIDP subtype was more commonly associated with respiratory tract infections and showed greater cranial and sensory nerve involvement, whereas the AMAN subtype was more commonly linked to gastroenteritis and had purely motor involvement. Although both subtypes were associated with good outcomes, recovery was slower in AMAN patients. In our study, 18 patients (38.3%) had AIDP, 18 (38.3%) had AMAN, and 11 (23.4%) had AMSAN. No significant association between subtype and prognosis was observed. Despite reports suggesting a poorer prognosis in axonal forms, we did not find a difference in our cohort, possibly due to the limited sample size.

Cranial nerve involvement is also observed in GBS patients. While 9th and 10th cranial nerve involvement is more common in younger children, facial nerve involvement is frequently reported in older children [16]. Although the association between cranial nerve involvement and prognosis remains unclear, some studies have suggested that these patients may be at higher risk of respiratory muscle involvement, requiring intubation, and may have worse outcomes [16]. Autonomic symptoms such as tachycardia, hypotension, and sinus arrhythmia are frequently observed, particularly in the AIDP and AMSAN subtypes and in patients with quadriplegia and respiratory failure [17]. Several studies have demonstrated an association between autonomic symptoms and increased need for respiratory support [18,19]. DiMario et al [20] reported autonomic dysfunction in 24 of 26 patients (92.3%) and established a correlation with disease severity. In our study, 10 patients (21.3%) had cranial nerve involvement, and 8 (17.0%) exhibited autonomic symptoms. However, neither cranial nerve involvement nor autonomic dysfunction was found to be significantly associated with prognosis.

Respiratory failure occurs in 20–30% of GBS cases and is associated with the rapidity of disease progression. The prognosis is generally worse in children requiring respiratory support. Mortality in ventilated patients has been reported to be between 15% and 30% [21]. In a study by Kalita et al [22] involving 328 GBS patients, including 85 under 15 years of age, 43 required ventilatory support. Of these, 26 had good outcomes, and 17 had poor outcomes, with no significant difference in prognosis between the groups. In contrast, Fletcher et al [23]., in a study of 114 cases, found that 60 patients required respiratory support and that 81% of patients with poor prognosis had received mechanical ventilation. They concluded that the need for respiratory support was associated with worse outcomes. In our study, 13 patients (27.7%) required respiratory support, and these patients had a slower recovery. We also found that respiratory support was significantly associated with poor prognosis.

Both intravenous immunoglobulin (IVIG) and plasmapheresis have been proven effective in GBS treatment [3,12]. IVIG is generally preferred in children due to its ease of administration and lower risk of complications. Van der Meché et al  [24], in a randomized trial involving 147 patients (74 treated with IVIG and 73 with plasmapheresis), found no significant difference in prognosis between the two treatments. In our study, 25 patients (53.2%) were treated with IVIG alone, while 22 (46.8%) received both IVIG and plasmapheresis. At the two-month follow-up, 22 of the 25 patients treated with IVIG alone were in Group 1 (good prognosis), whereas only 8 of the 22 patients who received combination therapy were in Group 1. Most of the patients in the combination group were classified as Group 2. These findings indicate that patients treated with IVIG alone had a significantly better prognosis than those treated with IVIG plus plasmapheresis. 

In pediatric GBS, the prognosis is generally more favorable than in adults. However, the prognostic factors remain incompletely understood. Various studies have explored clinical and electrophysiological predictors. In our study, factors such as age, gender, antecedent infection, cranial nerve involvement, and autonomic symptoms were not significantly associated with prognosis. However, the need for respiratory support and the type of treatment administered were significant prognostic indicators. We observed that the prognosis was worse in patients who required respiratory support and in those treated with a combination of IVIG and plasmapheresis.

In conclusion, our study suggests that in pediatric GBS, gender, age, preceding infection, seasonality, cranial nerve involvement, and autonomic symptoms do not significantly affect prognosis. In contrast, the need for respiratory support and the treatment method were significant predictors of outcome.

Conflict of interest

Authors declare that there is no conflict of interest between the authors of the article.  

Financial conflict of interest

Authors declare that they did not receive any financial support in this study.

Data availability: Data are available on reasonable request.

References

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Luiz Sellmann

I would like to offer my testimony in the support. I have received through the peer review process and support the editorial office where they are to support young authors like me, encourage them to publish their work in your esteemed journals, and globalize and share knowledge globally. I really appreciate your journal, peer review, and editorial office.

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Zhao Jia

Dear Agrippa Hilda- Editorial Coordinator of Journal of Neuroscience and Neurological Surgery, "The peer review process was very quick and of high quality, which can also be seen in the articles in the journal. The collaboration with the editorial office was very good."

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Thomas Urban

I would like to express my sincere gratitude for the support and efficiency provided by the editorial office throughout the publication process of my article, “Delayed Vulvar Metastases from Rectal Carcinoma: A Case Report.” I greatly appreciate the assistance and guidance I received from your team, which made the entire process smooth and efficient. The peer review process was thorough and constructive, contributing to the overall quality of the final article. I am very grateful for the high level of professionalism and commitment shown by the editorial staff, and I look forward to maintaining a long-term collaboration with the International Journal of Clinical Case Reports and Reviews.

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Cristina Berriozabal

To Dear Erin Aust, I would like to express my heartfelt appreciation for the opportunity to have my work published in this esteemed journal. The entire publication process was smooth and well-organized, and I am extremely satisfied with the final result. The Editorial Team demonstrated the utmost professionalism, providing prompt and insightful feedback throughout the review process. Their clear communication and constructive suggestions were invaluable in enhancing my manuscript, and their meticulous attention to detail and dedication to quality are truly commendable. Additionally, the support from the Editorial Office was exceptional. From the initial submission to the final publication, I was guided through every step of the process with great care and professionalism. The team's responsiveness and assistance made the entire experience both easy and stress-free. I am also deeply impressed by the quality and reputation of the journal. It is an honor to have my research featured in such a respected publication, and I am confident that it will make a meaningful contribution to the field.

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Dr Tewodros Kassahun Tarekegn

"I am grateful for the opportunity of contributing to [International Journal of Clinical Case Reports and Reviews] and for the rigorous review process that enhances the quality of research published in your esteemed journal. I sincerely appreciate the time and effort of your team who have dedicatedly helped me in improvising changes and modifying my manuscript. The insightful comments and constructive feedback provided have been invaluable in refining and strengthening my work".

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Dr Shweta Tiwari

I thank the ‘Journal of Clinical Research and Reports’ for accepting this article for publication. This is a rigorously peer reviewed journal which is on all major global scientific data bases. I note the review process was prompt, thorough and professionally critical. It gave us an insight into a number of important scientific/statistical issues. The review prompted us to review the relevant literature again and look at the limitations of the study. The peer reviewers were open, clear in the instructions and the editorial team was very prompt in their communication. This journal certainly publishes quality research articles. I would recommend the journal for any future publications.

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Dr Farooq Wandroo

Dear Jessica Magne, with gratitude for the joint work. Fast process of receiving and processing the submitted scientific materials in “Clinical Cardiology and Cardiovascular Interventions”. High level of competence of the editors with clear and correct recommendations and ideas for enriching the article.

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Dr Anyuta Ivanova

We found the peer review process quick and positive in its input. The support from the editorial officer has been very agile, always with the intention of improving the article and taking into account our subsequent corrections.

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Dr David Vinyes

My article, titled 'No Way Out of the Smartphone Epidemic Without Considering the Insights of Brain Research,' has been republished in the International Journal of Clinical Case Reports and Reviews. The review process was seamless and professional, with the editors being both friendly and supportive. I am deeply grateful for their efforts.

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Gertraud Teuchert-Noodt

To Dear Erin Aust – Editorial Coordinator of Journal of General Medicine and Clinical Practice! I declare that I am absolutely satisfied with your work carried out with great competence in following the manuscript during the various stages from its receipt, during the revision process to the final acceptance for publication. Thank Prof. Elvira Farina

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Dr Elvira Farina

Dear Jessica, and the super professional team of the ‘Clinical Cardiology and Cardiovascular Interventions’ I am sincerely grateful to the coordinated work of the journal team for the no problem with the submission of my manuscript: “Cardiometabolic Disorders in A Pregnant Woman with Severe Preeclampsia on the Background of Morbid Obesity (Case Report).” The review process by 5 experts was fast, and the comments were professional, which made it more specific and academic, and the process of publication and presentation of the article was excellent. I recommend that my colleagues publish articles in this journal, and I am interested in further scientific cooperation. Sincerely and best wishes, Dr. Oleg Golyanovskiy.

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Dr Oleg Golyanovski

Dear Ashley Rosa, Editorial Coordinator of the journal - Psychology and Mental Health Care. " The process of obtaining publication of my article in the Psychology and Mental Health Journal was positive in all areas. The peer review process resulted in a number of valuable comments, the editorial process was collaborative and timely, and the quality of this journal has been quickly noticed, resulting in alternative journals contacting me to publish with them." Warm regards, Susan Anne Smith, PhD. Australian Breastfeeding Association.

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Dr Susan Anne Smith

Dear Jessica Magne, Editorial Coordinator, Clinical Cardiology and Cardiovascular Interventions, Auctores Publishing LLC. I appreciate the journal (JCCI) editorial office support, the entire team leads were always ready to help, not only on technical front but also on thorough process. Also, I should thank dear reviewers’ attention to detail and creative approach to teach me and bring new insights by their comments. Surely, more discussions and introduction of other hemodynamic devices would provide better prevention and management of shock states. Your efforts and dedication in presenting educational materials in this journal are commendable. Best wishes from, Farahnaz Fallahian.

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Dr Farahnaz Fallahian

Dear Maria Emerson, Editorial Coordinator, International Journal of Clinical Case Reports and Reviews, Auctores Publishing LLC. I am delighted to have published our manuscript, "Acute Colonic Pseudo-Obstruction (ACPO): A rare but serious complication following caesarean section." I want to thank the editorial team, especially Maria Emerson, for their prompt review of the manuscript, quick responses to queries, and overall support. Yours sincerely Dr. Victor Olagundoye.

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Dr Victor Olagundoye

Dear Ashley Rosa, Editorial Coordinator, International Journal of Clinical Case Reports and Reviews. Many thanks for publishing this manuscript after I lost confidence the editors were most helpful, more than other journals Best wishes from, Susan Anne Smith, PhD. Australian Breastfeeding Association.

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Dr Susan Anne Smith

Dear Agrippa Hilda, Editorial Coordinator, Journal of Neuroscience and Neurological Surgery. The entire process including article submission, review, revision, and publication was extremely easy. The journal editor was prompt and helpful, and the reviewers contributed to the quality of the paper. Thank you so much! Eric Nussbaum, MD

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Dr Eric S Nussbaum

Dr Hala Al Shaikh This is to acknowledge that the peer review process for the article ’ A Novel Gnrh1 Gene Mutation in Four Omani Male Siblings, Presentation and Management ’ sent to the International Journal of Clinical Case Reports and Reviews was quick and smooth. The editorial office was prompt with easy communication.

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Hala Al Shaikh

Dear Erin Aust, Editorial Coordinator, Journal of General Medicine and Clinical Practice. We are pleased to share our experience with the “Journal of General Medicine and Clinical Practice”, following the successful publication of our article. The peer review process was thorough and constructive, helping to improve the clarity and quality of the manuscript. We are especially thankful to Ms. Erin Aust, the Editorial Coordinator, for her prompt communication and continuous support throughout the process. Her professionalism ensured a smooth and efficient publication experience. The journal upholds high editorial standards, and we highly recommend it to fellow researchers seeking a credible platform for their work. Best wishes By, Dr. Rakhi Mishra.

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Dr Rakhi Mishra