JPED

Introduction

Guillain-Barre Syndrome (GBS) is a post infectious, monophasic, immune‐mediated polyradiculoneuropathy. About a third of patients with GBS develop generalised neuropathy, and require mechanical ventilation for respiratory failure [1]. GBS affects adults and children, although it is less common in paediatric population, where incidence estimates range from 0.4 to 1.4 cases per 100,000 children [2]. This condition presents after an antecedent viral or bacterial infection, with half of paediatric cases reporting a recent respiratory or gastrointestinal illness, underscoring the autoimmune pathogenesis of GBS [3]. While some patients experience a monophasic course, others may have relapses or secondary complications due to autonomic dysfunction or respiratory muscle weakness, necessitating intensive care [3].

Since the introduction of therapies such as plasmapheresis and intravenous immunoglobulin (IVIG) in the 1980s, clinical outcomes for GBS have improved, reducing the need for ventilatory support and the rate of mortality [4]. These treatments are particularly beneficial in paediatric cases, as children tend to show a faster recovery rate and better overall prognosis than adults [5]. However, despite therapeutic advancements, the evidence base on optimal management and long-term outcomes in GBS is limited, particularly in developing countries [6].

Clinical presentations of GBS vary such as acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN) displaying distinct neurophysiology [4,7]. AIDP, the commonest subtype, involves demyelination, while AMAN primarily affects axons and is  prevalent in Asia [2,8]. Electrophysiological studies, are critical in differentiating subtypes and can guide prognosis and management [4,7]. There is a scarcity of studies focusing on paediatric GBS in India. This study aims to gain insight into the clinical characteristics, diagnostic challenges, and treatment approaches for paediatric GBS.

Material and Methods

This study was a prospective observational study. Participants were selected for the study using a non-probability convenience sampling method. Participants admitted in Paediatric intensive care unit (PICU) Kamla Raja Hospital, Gwalior with sign and symptom of GBS who met National institute of neurological and stroke Criteria (NINDS) [4]. were included in this study after taking informed consent from their parents.

Detailed demographic profile, history and clinical examination were done in all study participants. Electrophysiological studies were done by using Neuro MEP 4 Neurosoft system after stabilization of the participants in the Neurology department of Gajra Medical College and Hospital and was reported by an expert. Management strategies were documented. Disability grading was done by using Hughes disability score at the time of admission, discharge and at 8 weeks follow-up. Outcome was measured at the time of discharge and after 8 weeks.

♦          Inclusion Criteria 

i. Children admitted in PICU with 1 to 15yrs of age, satisfying NINDS criteria for GBS^[4].

♦          Exclusion Criteria 

1. Children with other causes of neuropathy, such as porphyria, botulism, post- diphtheritic neuropathy, drug or toxin induced neuropathy, or poliomyelitis, trauma.
2. Children diagnosed with neuromuscular junction disorders or myasthenia gravis.

Study Flow Chart

Statistical and graphical analyses for this study were undertaken by stata software version 17.0. Descriptive statistics were used to summarize demographic variables and clinical features, while inferential tests, such as chi-square tests and t-tests, were applied to assess relationships between independent and dependent variables. Multivariate analyses were performed to control for potential confounders, providing adjusted estimates for prognostic indicators in gbs patients.

Results

A total of 52 children from 1year up to 15 years, diagnosed with Guillain-Barré Syndrome based on clinical and electrophysiological criteria, were enrolled after fulfilling the inclusion and exclusion criteria.Relevant demographic data, clinical presentations, disability scores, electrophysiological findings, need for ventilatory support, and short-term recovery outcomes were recorded.

(Table 1) illustrates that the majority of participants were in the 6–10 years age group (42.31%), followed by 1–5 years (36.54%) and 11–15 years (21.15%). There was a slight female predominance, with 51.92% girls and 48.08% boys. Most children (61.5%) belonged to rural areas, while 38.5% were from urban settings. Seasonal distribution showed the highest incidence during the rainy season (32.69%), followed by autumn (26.92%), summer (23.07%), and winter (17.30%).

Table 1: Baseline demographic characterstics of study participants.

Antecedent illness was notable in several cases, with a history of upper respiratory tract infection reported in 34.62% and diarrhea in 28.85% of participants. Recent immunization was documented in 17.30% of cases. Ascending paralysis emerged as the most common form 44.23%, while descending paralysis was observed in 19.23%. Pain, paresthesia, sensory involvment varied in extent, with pain (26.92%) paresthesia (30.77%) sensory (36.62%) among participants had extending above the knees or elbows.

Motor symptoms were frequently reported, with difficulty in combing or reaching overhead being the most prevalent (36.54%), followed by difficulty in hand grip and turning in bed (21.15% each). Bulbar symptoms were present in 13.46%, and foot weakness, indicated by slipping off of footwear which was noted in 7.69%. Cranial nerve involvement was observed in both facial (7.69%) and bulbar nerves (11.53%). Notably, 7.69% required mechanical ventilation during their hospital stay, while the majority (92.31%) did not need any ventilatory assistance. This suggests in some cases of paediatric GBS can be severe, most children can be managed without the need for respiratory support when diagnosed and treated promptly.

(Table 2) illustrates that Acute motor axonal neuropathy (AMAN) was the most common subtype, accounting for 38.46% of cases, acute inflammatory demyelinating polyneuropathy (AIDP) at 28.85%, and acute motor-sensory axonal neuropathy (AMSAN) at 23.08%, 9.62%, fell into other less common subtypes.

Table 2: Electrophysiological Findings.

(Table 3) Highlights the disability scores at the time of admission, at discharge and after 8 weeks followup. Twenty two children (42.30%) had grading score of 1, four children (7.69%) had grading score of 5 at the time of admission. Thirty children (57.69%) had grading score of 0, four children (7.69%) children had grading score of 4 at the time of discharge. Forty-one children (78.84%) had grading score of 0, one child had grading score of 3 at the time of follow-up after 8 weeks.

Table 3: Distribution of participants according to Hughes Disability Score.

(Table 4) Depicts the recovery status of paediatric gbs patients, which was assessed at two time points: At discharge and after 8 weeks of follow-up. At the time of discharge, 57.69% of patients had achieved full recovery, while 26.92%  showed mild residual disability and 15.38% had moderate residual disability. Importantly, no patients were discharged with severe disability. At the 8-week followup, outcomes had notably improved. The proportion of patients achieving full recovery increased to 78.85%, indicating significant functional recovery post-discharge. the number of children with mild residual disability decreased to 13.46%, and those with moderate disability further declined to 7.69%. No cases of severe disability were observed at follow-up.

Table 4: Distribution of study subjects according to recovery at discharge.