European Journal of Cardiovascular Medicine

Guillain-Barre Syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy that manifests as rapidly progressive, symmetrical limb weakness and areflexia. It is one of the most common causes of acute flaccid paralysis worldwide, with an incidence of approximately 1 to 2 cases per 100,000 populations annually [1]. The pathogenesis involves immune-mediated demyelination and/or axonal damage to peripheral nerves, often triggered by antecedent infections such as Campylobacter jejuni, cytomegalovirus, or Epstein-Barr virus [2,3].

Clinically, GBS presents with varying degrees of motor, sensory, and autonomic dysfunction, and may involve cranial nerves in severe cases. Electrophysiological studies, particularly nerve conduction studies (NCS), play a pivotal role in confirming the diagnosis, classifying subtypes such as acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN), and in prognostication [4,5]. Serial electrophysiological assessments during the disease course provide insight into the dynamic evolution of nerve involvement and can help guide management decisions [6].

Despite advances in immunotherapy and supportive care, morbidity remains significant, with some patients experiencing prolonged disability. Understanding the clinical spectrum and electrophysiological evolution of GBS is essential for improving outcome prediction and tailoring therapy.

The aim of this study is to evaluate the clinical spectrum and functional outcomes of patients diagnosed with Guillain-Barré Syndrome (GBS) and to analyse the progression of electrophysiological changes through serial nerve conduction studies. The objective is to correlate these electrophysiological patterns with clinical severity and recovery, thereby improving prognostic accuracy and guiding management strategies.

This prospective observational, single center study was performed at DR. B C Roy Post Graduate Institute of Paediatric Sciences, a tertiary care hospital in Kolkata. All Guillain-Barre syndrome patients in the age group of one month to 12 years (as per the institution's norm) who were admitted to the hospital from the emergency and outpatient departments in-between February 2021 to July 2022 were included in the study group.  Any patient with pre-existing motor deficit, minor variants of GBS and lack of parental consent were  excluded from the study. The participants were followed up for the next six months. The Institutional Ethical Committee of Dr. B C Roy Post Graduate Institute of Paediatric Sciences, Kolkata, issued approval (BCH/ME/PR/: 63A).

GBS was diagnosed according to Asbury- Cornblath GBS Diagnostic criteria. Eligible children went detailed history taking, clinical assessment as per a predesigned proforma. Functional ability of the patients were tested by using Hughes GBS disability score (HGDS) and the severity of the motor weakness by MRC sum score. Those patients who were found to have impending respiratory failure and autonomic instability were transferred to HDU and PICU for further management and rest are treated in paediatric general ward.

Nerve conduction study (NCS) has been done as per standard protocol using NIHON KOHDEN machine within 72 hours of admission. For motor NCS median, ulnar, tibial and common peroneal nerve and for sensory NCS median, ulnar and sural nerves were stimulated and analysed. All the children underwent stool examination for polio virus detection & other routine blood parameters were taken alongside. CSF analysis was done in 2nd week of the disease onset. All patients were treated with administration of IVIG. Serial nerve conduction studies along with clinical evaluation were done on 1 month and at 6 months follow up.

Statistical analysis: For statistical analysis data were entered into a Microsoft excel spread sheet and then analysed by SPSS 24.0. and Graph Pad Prism version 5. Data had been summarized as mean and standard deviation for numerical variables and count and percentages for categorical variables. Two-sample t-tests for a difference in mean involved independent samples or unpaired samples. Paired t-tests were a form of blocking and had greater power than unpaired tests.

 Unpaired proportions were compared by Chi-square test or Fischer’s exact test, as appropriate. Z test (Standard Normal Deviate) was used to test the significant difference of proportions. P-value ≤ 0.05 was considered to be statistically significant.

Data from the study were analysed using SPSS software, with continuous variables (e.g., age, liver enzyme levels) expressed as mean ± SD and compared using t-tests or Mann–Whitney U tests. Categorical variables (e.g., gender, CBD stones, and complications) were presented as frequencies and percentages, and compared using Chi-square or Fisher’s exact tests. Diagnostic accuracy (sensitivity, specificity, PPV, NPV, and accuracy) was calculated for MRCP-first and EUS-first strategies, using ERCP/intraoperative findings as the reference. Kaplan-Meier analysis may be used for time-to-intervention comparisons.

Table 1: Association of Clinical and Electrophysiological Parameters and Their Outcomes in Patients (n=36)

Table 2: Association of Nerve Conduction Study (NCS) Findings at Admission and Their Association with Clinical Parameters (n=35)

Table 3: Distribution of Mode of Presentation and Sensory Symptoms (n=36)

Figure 1: Distribution of Mode of Presentation and Sensory Symptoms (n=36)

In our study of 36 patients, various clinical and electrophysiological parameters were analyzed for their association with patient outcomes categorized as good or poor. 1 patient died in acute phase and 3 patients lost follow-up after 1 month. Antecedent illness such as diarrhea (p = 0.309), respiratory tract infection,   did not show a statistically significant correlation with outcome in comparison to absent preceding illness GB cases. However, the mode of motor presentation showed a significant association (p = 0.014), where patients presenting with ascending paralysis had better outcomes compared to those with all four limbs affected simultaneously. Cranial nerve involvement was also significantly associated with poorer outcomes (p = 0.049). Autonomic dysfunction showed a significant difference between groups (p = 0.013), with patients experiencing excessive sweating and fluctuating blood pressure more frequently having poor outcomes. Peak disability duration (p = 0.259), cyto-albumin dissociation (p = 0.392), and the presence of complications such as pneumonia or septicemia (p = 0.402) did not show statistically significant differences between outcome groups. Duration of hospital stay was not significantly different (p = 0.116). Assisted ventilation was highly significantly associated with poor outcome (p < 0.001), as all patients requiring ventilation had poor outcomes. Timing of IVIG administration (p = 0.764), conduction block presence (p = 0.266), reduced CMAP (p = 0.266), distal latency prolongation (p = 0.970), and abnormal F wave (p = 0.393) did not correlate significantly with outcomes. Additionally, the Hughes Grade Disability Scale (HGDS) showed significant association with outcome (p < 0.05), with higher grades correlating to poorer prognosis.

In this cohort study among 35 patients categorization by nerve conduction study (NCS) to the following subtypes were—AMAN (14), AIDP (17), AMSAN (2), and Inexcitable (2). The mode of motor presentation was significantly associated with NCS subtype (p = 0.014), with ascending paralysis more common in AIDP and AMAN types compared to simultaneous four-limb involvement in AMSAN and inexcitable vaiant. Cranial nerve involvement was significantly different among NCS groups (p = 0.02), with higher prevalence in AMAN and AMSAN. Autonomic dysfunction also showed significant variation (p = 0.039), with symptoms like excessive sweating and sinus tachycardia distributed unevenly across groups. Peak disability duration did not differ significantly (p = 0.766). The Hughes Grade Disability Scale (HGDS) at admission was highly significant among subtypes (p = 0.0001), indicating more severe disability in AMAN and inexcitable types, while HGDS at six months also showed significant improvement differences (p = 0.01). Cyto-albumin dissociation, severity of weakness by MRC scale, and complications such as pneumonia or UTI were not significantly different (p > 0.2). Duration of hospital stay showed no statistically significant difference (p = 0.14). Assisted ventilation was significantly associated with specific subtypes (p < 0.0001), occurring mainly in AMSAN patient.

In our study, the mode of motor presentation varied among patients, with 14 patients (38.9%) presenting with weakness in all four limbs simultaneously, while the majority, 22 patients (61.1%), showed an ascending pattern of paralysis. Regarding sensory symptoms, 10 patients (43%) reported paresthesia, making it the most common sensory complaint. Other sensory symptoms included numbness of legs in 5 patients (22%), generalized muscle pain in 4 patients (17%), pain in the back and neck in 2 patients (9%), and leg pain also reported by 2 patients (9%).

In our study of 36 patients with acute motor neuropathy, clinical and electrophysiological parameters were analyzed for their association with patient outcomes and nerve conduction study (NCS) subtypes. The mode of motor presentation emerged as a significant prognostic factor, with ascending paralysis associated with better outcomes compared to simultaneous four-limb involvement. This finding aligns with previous studies by Rajabally et al. [7] and Kuwabara et al. [8], who reported that the clinical pattern of progression influences prognosis, with ascending paralysis typical of acute inflammatory demyelinating polyneuropathy (AIDP) having a relatively favorable outcome compared to rapidly progressive presentations. Similarly, cranial nerve involvement was significantly associated with poorer outcomes, consistent with results by Ropper [9] and Yuki et al. [10], who emphasized cranial nerve dysfunction as an indicator of disease severity and a predictor of prolonged recovery .Autonomic dysfunction also showed a significant correlation with poorer outcomes, particularly in patients experiencing excessive sweating and blood pressure fluctuations. This corroborates findings by Willison et al. [11], who reported that autonomic disturbances in Guillain-Barré syndrome (GBS) are linked to increased morbidity. Furthermore, assisted ventilation was strongly associated with poor prognosis, supporting evidence by Hughes et al. [12] that respiratory failure requiring mechanical ventilation is a key marker of severe disease.The Hughes Grade Disability Scale (HGDS) was a reliable predictor of outcome, with higher grades correlating with worse prognosis, as noted in studies by van Koningsveld et al. [13] and Kleyweg et al. [14], which validated HGDS as a useful clinical tool for disability assessment and monitoring response to treatment.In terms of sensory symptoms, paresthesia was the most commonly reported complaint (43%), followed by numbness and generalized muscle pain. This sensory profile is comparable to observations by Dimachkie and Barohn [15] and McGrogan et al. [16], who documented frequent paresthesia and sensory disturbances in patients with acute motor neuropathies, although sensory involvement varies by subtype and severity.The lack of significant association between antecedent illnesses and outcomes or NCS subtypes in our study contrasts with some literature suggesting that preceding infections, particularly diarrheal illness caused by Campylobacter jejuni, may influence subtype distribution and prognosis . However, this discrepancy could be due to our smaller sample size or regional variation in etiologic agents.

In this study of patients with acute motor neuropathy, the mode of motor presentation, cranial nerve involvement, autonomic dysfunction, and the need for assisted ventilation were significant predictors of patient outcomes. Ascending paralysis was associated with better prognosis, while simultaneous four-limb weakness and cranial nerve involvement indicated poorer recovery. The Hughes Grade Disability Scale effectively reflected disability severity and prognosis. Electrophysiological parameters and antecedent illnesses did not significantly correlate with outcomes. These findings underscore the importance of detailed clinical assessment in predicting prognosis and guiding management in acute motor neuropathies. Larger studies are recommended to validate these results and enhance prognostic accuracy.

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