Guillain-Barré Syndrome Subtypes Research Articles

Guillain–Barré syndrome in children: clinical and diagnostic features

Introduction. In 2025, we will commemorate the 100th anniversary of the first pediatric case of Guillain–Barré Syndrome (GBS), initially documented by M. Monnier-Vinard. This significant milestone invites a reevaluation of our contemporary understanding of GBS in children. While the original description of GBS was thorough, the categorization has expanded over time to include a wider array of conditions, resulting in the term applied to various acute demyelinating polyneuropathies. The atypical forms of GBS present notable diagnostic challenges for healthcare providers. Objective. To analyze the demographic and clinical characteristics, treatment methods, and prognosis of GBS in children in Russia. Materials and methods. The study included fifty 6 months – 15 years patients with typical and atypical forms of Guillain-Barré syndrome (GBS). Neurological status was assessed using the Medical Research Council sum score and the Hughes disability scale. Additionally, data from cerebrospinal fluid analysis, nerve conduction study, magnetic resonance imaging of the spine, and peripheral nerves ultrasound was evaluated. Results. The classic ascending subtype of GBS was diagnosed in 30 (60%) patients, while atypical manifestations were observed in 17 (34%). Three (6%) patients had an overlap syndrome: GBS with acute myelitis. The median age of the patients was 7 [5.0; 11.0] years. Two peaks of GBS incidence associated with respiratory and gastrointestinal infections were identified. The most common initial symptoms were pain (80%), weakness in the legs (64%), and difficulty walking (54%). All 26 (100%) GBS patients were followed for 12 months from the onset of the disease regained the ability to walk. Conclusion. Both typical and atypical forms of GBS can occur in children. Overlap syndrome (GBS with acute myelitis) is a rare complication following COVID-19 and may go undiagnosed. With appropriate therapy the prognosis for both typical and atypical forms of GBS is generally favourable.

Resolution of acute motor axonal neuropathy in a patient after treatment with efgartigimod: A case report.

Guillain-Barré syndrome (GBS) is an acute autoimmune neuropathy characterized by progressive muscle weakness, often caused by immunoglobulin G (IgG) autoantibodies. There are several subtypes of GBS, of which acute motor axonal neuropathy (AMAN) is one of the most severe subtypes associated with axonal damage. It is well known that the current clinical standard of treatment is intravenous immunoglobulin (IVIg) and plasma exchange (PLEX), but some patients often show limited response or experience persistent disability. Efgartigimod, an Fc fragment of human IgG antibody, provides a way to target and reduce pathogenic IgG antibodies as a natural ligand Fc receptor (FcRn). The purpose of this study was to observe the therapeutic effect of efgartigimod on axonal GBS, which is expected to be a potential therapeutic method for GBS and AMAN. We present a case of a 58-year-old man diagnosed with AMAN, presenting with ascending symmetrical limb weakness, flaccid paralysis, and multiple cranial nerve palsies. Electromyography confirmed the axonal subtype of GBS. Despite receiving IVIg and PLEX, the patient showed suboptimal recovery. Subsequently, he was treated with efgartigimod at a dose of 10 mg/kg weekly for 4 weeks, demonstrating significant improvement in both clinical symptoms and electromyographic findings, with good tolerability. This case highlights the potential efficacy and safety of a 4-dose efgart-igimod regimen in AMAN, particularly for patients with inadequate response to conventional therapies. By targeting FcRn and promoting IgG degradation, efgartigimod offers a novel mechanism to modulate the aberrant immune response underlying AMAN. Efgartigimod at a dose of 10 mg/kg weekly for 4 weeks demonstrated promising results in this case of AMAN. While further research is warranted, our findings suggest that efgartigimod may represent a valuable addition to the therapeutic armamentarium for AMAN and potentially other autoimmune neurological conditions. Well-designed clinical trials are crucial to confirm these findings and establish optimal treatment protocols for efgartigimod in AMAN.

OUTCOME OF PLEX IN GUILLAIN–BARRÉ SYNDROME PATIENTS TREATED BEFORE AND AFTER 7 DAYS OF SYMPTOMS ONSET

Background: Guillain-Barré Syndrome (GBS) is an acute, immune-mediated polyneuropathy that often follows infections, characterized by varying degrees of motor weakness, sensory impairment, and autonomic dysfunction. The disease has multiple subtypes, including Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) and Acute Motor Axonal Neuropathy (AMAN), with diverse functional outcomes. Plasma exchange (PLEX) has emerged as a cost-effective and efficient therapeutic option in resource-limited settings, but its impact on various subtypes and functional outcomes requires further exploration. Objective: To evaluate the effectiveness of plasma exchange (PLEX) as a treatment for GBS and to prospectively analyze the clinical and functional outcomes of patients aged 16 and above across different subtypes of GBS. Methods: This prospective observational study was conducted over six months at a tertiary care hospital in Rawalpindi. Participants aged 16 and older, meeting inclusion criteria, underwent detailed history taking, clinical examination, and electrophysiological studies, including Forced Vital Capacity (FVC) and EMG, to categorize GBS subtypes. Functional disability was assessed using the Hughes GBS Disability Scale. All patients received five sessions of PLEX initiated on the day of admission. Outcomes were evaluated on the seventh day. Data analysis was performed using SPSS, with quantitative variables presented as means and qualitative variables as percentages. Results: A total of 40 participants were included, with a mean age of 38.97 ± 5.4 years. Males constituted 63% (n = 25) and females 37% (n = 15). Participants were categorized by Hughes GBS Disability Score into Group 1 (n = 5, Score 2), Group 2 (n = 12, Score 3), Group 3 (n = 20, Score 4), and Group 4 (n = 3, Score 5). Based on EMG findings, 57.5% (n = 23) had AIDP, 30% (n = 12) had AMAN, and 12.5% (n = 5) had AMSAN. After seven days of treatment, 80% of patients in Groups 1 and 2 showed significant improvement, while 75% in Group 3 and 33% in Group 4 demonstrated partial recovery. AIDP had the best response to PLEX, with 24 patients showing improvement, compared to 1 patient with AMAN and 2 with AMSAN. Conclusion: Patients with GBS exhibit varying functional disabilities and outcomes across different subtypes. PLEX demonstrated significant improvement, particularly in patients with lower initial disability scores and the AIDP subtype. Early intervention and subtype-specific management are critical for optimizing outcomes.

Potential advantage of Therapeutic Plasma Exchange over Intravenous Immunoglobulin in children with axonal variant of Guillain-Barré syndrome: A report of six paediatric cases

Guillain-Barre syndrome (GBS) is a disease entity described in literature since 1859. It is associated with various etiological, clinical and immunological factors with prognostic predictive value. Both Intravenous immunoglobulin (IVIG) and Therapeutic Plasma Exchange (TPE) have been regarded as the first-line treatment for GBS. Certain diagnostic tools help us in early identification of GBS subtypes that may aid clinical management. Here, we have discussed six paediatric cases of GBS of Acute Motor Axonal Neuropathy (AMAN) subtype that were considered for TPE. 5 out of 6 patients were eventually weaned from mechanical ventilation and discharged from the hospital. This study emphasizes the role of TPE in management of severe IVIG refractory GBS with axonal involvement that can be beneficial to the patient. TPE may be considered early in GBS cases with axonal involvement.

Microscopical anatomy of the peripheral nervous system: An essential notion for understanding the pathophysiology of very early classic Guillain-Barré syndrome.

The aim of this paper is to analyze the pathophysiological mechanisms acting in very early classic Guillain-Barré syndrome (GBS) (≤4 days of symptomatic onset). In this inaugural period, both in GBS and its animal model, experimental autoimmune neuritis, the outstanding pathological feature is inflammatory edema predominating in proximal nerve trunks, particularly spinal nerves, and possibly in preterminal nerve segments. Nerve trunks external to the subarachnoid angle possess epi- perineurium that is relatively inelastic and of low compliance. Here such edema can increase endoneurial fluid pressure that, when sufficiently critical, may stretch the perineurium and constrict transperineurial microcirculation, compromising blood flow and producing the potential for ischemic nerve injury, whose consequence is rapid partial or complete loss of nerve excitability. These histopathological features correlate well with electrophysiological and imaging findings reported in early GBS stages. Spinal nerve edema and ischemia help to understand the pattern of Wallerian-like degeneration observed in the axonal form of GBS, predominating in motor spinal roots at their exit from the dura matter (spinal nerves) with centrifugal distribution in more distant motor nerve trunks, and centripetal extension to the distal portion of intrathecal roots. The similarity of initial pathogenic mechanisms between demyelinating and axonal forms of GBS explains why an early increase of serum biomarkers of axonal damage is detected in both forms. In conclusion, knowledge of the microscopic anatomy of the peripheral nervous system is an essential step for a reliable understanding of pathophysiological mechanisms operating in the early phase of any classic GBS subtype.

Early Electrophysiology in Suspected Acute Guillain-Barré Syndrome: A Prospective Study of Comprehensive Testing.

Electrophysiologic changes in early Guillain-Barré Syndrome (GBS) can be nondiagnostic. Improved testing paradigms may improve hyperacute treatment. This work prospectively evaluated consecutive patients admitted to a metropolitan teaching hospital in Melbourne, Australia, with suspected acute GBS. We performed extensive neurophysiology at three different time points. Novel tests, including cutaneous silent periods, long latency reflexes, and contraction-induced H reflexes, were assessed. Twenty-three participants were studied, including 13 cases of acute GBS. In total, 69% of acute cases of GBS were accurately diagnosed on the first nerve conduction study using published neurophysiologic criteria, with serial studies rarely altering the GBS subtype classification. Antidromic and orthodromic upper limb sensory studies were diagnostically equivalent. A sural sparing pattern was seen in 77% of cases of GBS at the first test. Long latency reflexes and contraction-induced H reflexes testing were abnormal in most participants but were limited by muscle weakness in some. Cutaneous silent periods testing was unobtainable in approximately 50% of cases because of weakness and did not discriminate from mimic disorders. Abnormalities of long latency reflexes and contraction-induced H reflexes may be helpful where initial electrophysiology is nondiagnostic but are nonspecific. Cutaneous silent periods testing seems of limited value. Comprehensive testing provides diagnostic certainty in most cases of GBS from the very first study.

Guillain-Barrè Syndrome-Retrospective Analysis of Data from a Cohort of Patients Referred to a Tertiary Care Pediatric Neuromuscular Center from 2000 to 2017: Electrophysiological Findings, Outcomes, and a Brief Literature Review.

Background and Objectives: Guillain-Barré syndrome (GBS) is the most frequent cause of acute flaccid paresis in children. The aim of this study was to describe the clinical and electrophysiological findings and outcomes of children with GBS diagnosed in our unit. Moreover, the literature on pediatric GBS cases from the past 5 years was reviewed. In this retrospective study, we reported data on 12 patients (9 male and 3 female patients; mean age: 5 y, 4 mo; range: 9 mo-11 y) clinically diagnosed at the Child Neurology Unit of the AUSL-IRCCS of Reggio Emilia, Italy, between 2000 and 2017 and a brief analysis/comparison with data from the literature. Materials and Methods: Data were collected from medical charts. Results: In our cohort, male patients were more frequent than female ones (9 vs. 3), and upper respiratory tract infection (n = 8, 66.7%) was the most frequent triggering factor. The main clinical symptoms on admission were distal lower limbs' weakness with gait difficulties (83.3%), pain (50%), upper limbs' weakness (50%), and dysphagia for liquids (25%). Peripheral neurophysiological studies revealed acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 66.6% of the children, acute motor and sensory axonal neuropathy (AMSAN) in 25%, and acute motor axonal neuropathy (AMAN) in 8.3%. Ten individuals (83.3%) received timely treatment with intravenous immunoglobulins (IVIG), and, out of these ten patients, 58% received concomitant treatment with IV methylprednisolone because of a progressive disease course. Complete remission was observed in the majority of individuals (91.6%) within 6 months of symptom onset. Conclusions: Different subtypes of GBS can affect children; however, the outcome is usually positive. Early treatment appears to be important for a favorable outcome.

Expanding our understanding of Guillain-Barré syndrome: Recent advances and clinical implications.

Guillain-Barré syndrome (GBS) is a rare yet potentially life-threatening disorder of the peripheral nervous system (PNS), characterized by substantial clinical heterogeneity. Although classified as an autoimmune disease, the immune mechanisms underpinning distinct GBS subtypes remain largely elusive. Traditionally considered primarily antibody-mediated, the pathophysiology of GBS lacks clarity, posing challenges in the development of targeted and effective treatments. Nevertheless, recent investigations have substantially expanded our understanding of the disease, revealing an involvement of autoreactive T cell immunity in a major subtype of GBS patients and opening new biomedical perspectives. This review highlights these discoveries and offers a comprehensive overview of current knowledge about GBS, including ongoing challenges in disease management.

Effectiveness of limited plasma exchange in Guillain-Barré syndrome

Objective: The aim of this study is to compare the efficacy of limited plasma exchange against supportive and standard treatments in treatment of Guillain-Barré syndrome (GBS). Method: A total of 90 GBS patients admitted to Yangon General Hospital, Yangon, Myanmar were recruited over 1.5-year-period from January 2017 to June 2018 and they were divided into 3 groups according to the treatment they received (convenience sampling method): supportive (n=36), limited plasma exchange (LPE) (n=35) and standard (therapeutic plasma exchange - TPE or IVIg) (n=19)). Their clinical features, electrophysiological subtypes and severity were compared at baseline, and outcome (change in GBS disability score (GDS) at 30 days after entry) was assessed and compared. Results: Baseline characteristics such as gender, GBS subtypes and respiratory involvement were comparable, but standard treatment group had older patients, and LPE group had shorter latency to nadir and more cases with antecedent diarrhea, which are poor outcome predictors. At 30 days from entry, mean GDS improvement was 0.9 + 0.6 in supportive, 1.4 + 0.6 in LPE and 1.2 + 0.8 in standard treatment groups respectively. The difference between LPE and supportive treatment was statistically significant (p 0.002) but there was no difference between LPE and standard treatment (p = 0.512). Regarding untoward effects, apart from one transient hypotension and 2 anemic cases, no other serious adverse effects were noted from LPE therapy. Conclusion: LPE may be superior to supportive treatment and may not be inferior to standard treatment. Therefore, LPE may be an alternative effective treatment for GBS patients who are not accessible to standard treatments especially in low income countries with limited resources.

Electrophysiological variations of Guillain-Barre syndrome in Bangladesh- Hospital based study

Introduction: Guillain-Barre syndrome (GBS) is an immune mediated disorder of peripheral nerves which usually presents by rapidly evolving ascending weakness & mild sensory loss and hypo- or areflexia. Electrodiagnostic study (EDS) is the basis for classification of different subtypes of the disease. EDS also has a crucial role in diagnosis, ruling out of some differential diagnosis like myopathic and motor neuron disorders and confirming the neuropathic nature of GBS. The benefit of immunotherapy is greatest when introduced early. In addition, electrophysiological characteristic can predict the prognosis of patients with GBS. This study was conducted to determine the predominant subtype and electrophysiological pattern of GBS in the context of Bangladesh. Objectives: Objective of this study was to compare the electrophysiological variations among different electrophysiological subtypes of GBS. Methods: It is an observational cross sectional study conducted in Department of Medicine and Neurology, Sir Salimullah Medical College & Mitford Hospital and National Institute of Neuroscience (NINS), Dhaka, over a period of one year & four months. Total 30 patients were selected by purposive sampling technique. Demographic data were collected from the patients and recorded in structured case report form. Clinical examination and relevant investigations were done meticulously. Collected questionnaire were checked to identify any error in data. Data was analyzed with SPSS version 21 software. Result: In this study, maximum numbers of patients 53% were between 21-30 years of age group with mean value 27.47±8.1 years. Male to female ratio was 1.7:1. Frequency of Guillain-Barre syndrome is predominance at middle age group. Commonest presentation was limb weakness (parapledgia or quadripledgia) in 60% patients, paresthesias & numbness (40%), pain (100%) and deviation of mouth (63%) of GBS patients. Cerebrospinal fluid shows a mild pleocytosis (5 to 50 cells/µl) in majority of cases was found in 76% of patients. Whereas elevated CSF Protein (>45 mg/dl) was seen in all GBS patient. Increased distal motor latency (DML) was found in 93% patients, whereas 7% patients had normal DML. In the case of the lower limbs, increased distal motor latency was predominant. Decreased amplitude of sensory nerve action potential (SNAP) was seen in 83% patients while 16% patients had normal. Slowing of motor conduction velocities, decreased amplitude as well as increase in distal motor latencies were observed, being more pronounced in the lower limbs. F-wave was completely absent in 20.0% patients while 20% patients showed decreased conduction velocity with prolonged latency. Sensory nerve action potential revealed that decreased sensory conduction velocity (SCV) was seen in 26%, absent SCV in 10% and normal SCV in 63% patients. Present study demonstrated that Acute inflammatory demyelinating polyneuropathy (AIDP) was the commonest type of Guillain-Barré syndrome, present in 56% of patient. Around 30% of the patients belonged to acute motor axonal polyneuropathy (AMAN) and 13% were acute motor sensory axonal polyneuropathy (AMSAN). Conclusion: In this study AIDP was the most frequent subtype. The characteristic findings supportive of AIDP include prolonged distal motor latencies, reduced conduction velocities, conduction blocks at non-entrapment sites, temporal dispersion and prolonged F wave latencies. J MEDICINE 2024; 25: 11-16

Assessment of Serum Inflammatory Markers and their Correlation with Clinical Severity and Electrophysiological Subtypes of Guillain Barre Syndrome, and Investigating their Use as Prognostic Markers of Guillain Barre Syndrome.

Assessment of Serum Inflammatory Markers and their Correlation with Clinical Severity and Electrophysiological Subtypes of Guillain Barre Syndrome, and Investigating their Use as Prognostic Markers of Guillain Barre Syndrome.

Reversible Conduction Failure in Guillain-Barré Syndrome

Gangliosides are the primary antigenic target in acute motor axonal neuropathy. When anti-ganglioside antibodies bind to gangliosides near the motor axon node of Ranvier, complement system activation and Wallerian degeneration occur. Nevertheless, debate persists regarding the impact of anti-ganglioside antibodies on Guillain-Barré syndrome (GBS). Certain patients with these antibodies experience a swift recovery or exhibit conduction abnormalities indicative of demyelination in nerve conduction studies. The concept of reversible conduction failure was introduced by Kuwabara et al. in 1998. They proposed that this could result from compromised physiological conduction at the node of Ranvier. Auto-antibodies that bind to GM1 or GD1a gangliosides at this node can activate the complement system and disrupt sodium channel and axo-glial junctions, causing conduction failure. In 2003, Cappaso et al. described two cases of rapidly improving flaccid paralysis following Campylobacter jejuni infection. Initial nerve conduction studies indicated motor conduction block, which resolved quickly within 2 to 5 weeks. The authors termed this phenomenon acute motor conduction block neuropathy and considered it a form of arrested or partial acute motor axonal neuropathy. Since acute motor conduction block neuropathy could be misclassified as acute inflammatory demyelinating polyneuropathy based on existing electrophysiological criteria, several suggestions were made to refine the classification of GBS subtypes.

The clinical significance of systemic immune-inflammation index and platelet/neutrophil to lymphocyte ratio in Guillain-Barré syndrome

ObjectiveSystemic immune-inflammation index (SII) and platelet/neutrophil to lymphocyte ratio (P/NLR) are two novel hematological inflammatory indices, this study invested the role of SII and P/NLR in Guillain-Barré syndrome (GBS). MethodsA total of 115 GBS patients and 120 healthy controls were enrolled in this retrospective study, SII and P/NLR were calculated from the value of complete blood counts. The Hughes Functional Grading Scale (HFGS) score on admission and at discharge was used to evaluate the severity and short-term outcome of GBS. The level of SII and P/NLR was compared between GBS patients and healthy controls, and the correlation between SII, P/NLR, and GBS’s severity as well as poor short-term outcome was analyzed. ResultsIncreased SII (p < 0.001) and decreased P/NLR (p < 0.001) were observed in patients with GBS and the level of SII (p = 0.689) and P/NLR (p = 0.879) was not different between GBS subtypes. Patients with severe GBS and poor short-term outcomes had higher levels of SII and lower levels of P/NLR (p < 0.05), SII was positively correlated and P/NLR was negatively correlated with the HFGS score on admission and at discharge (p < 0.05). To predict severe GBS, the optimal cutoff value of SII was 620.87 (AUC0.633, sensitivity 60.8 % and specificity 60.9 %, p = 0.014), the optimal cutoff value of P/NLR was 53.11 (AUC0.635, sensitivity 45.1 %, specificity 82.8 %, p = 0.013). The SII level of 620.87 was found to be optimal predictive cutoff value for the poor short-term outcome of GBS (AUC 0.728, sensitivity of 82.6 %, specificity of 59.8 %, p = 0.001), the P/NLR level of 62.80 was found to be optimal predictive cutoff value for the poor short-term outcome of GBS (AUC 0.669, sensitivity 60.9 %, specificity 71.7 %, p = 0.012). Both SII>620.87 (p = 0.005) and P/NLR<53.11 (p = 0.002) were independent risk factors for severe GBS, and SII>620.87 (p = 0.035) was independently associated with the poor short-term outcome of GBS. ConclusionSII and P/NLR may be useful biomarkers to reflect GBS patients’ severity and short-term outcome.

Correlation of Total Protein in Cerebrospinal Fluid with the Type of Guillain-Barre Syndrome based on the Electrodiagnostic Examination at Prof. Dr. IGNG Ngoerah General Hospital Denpasar

Introduction: Guillain-Barre syndrome (GBS) is an autoimmune disease of the peripheral nervous system with an incidence of around 0.6 to 2.4 cases per 100,000 population. GBS pathogenesis is related to inflammatory factors, cytokines, chemokines, and complement. Protein levels in the cerebrospinal fluid of GBS patients will increase due to damage or increased permeability of the blood-nerve barrier and blood-cerebrospinal fluid (CSF) barrier. However, there are still pros and cons to the relationship between the amount of CSF protein and the subtype of GBS. Objective: To determine the most common GBS subtype and the characteristics of electrodiagnostic examination at Prof Dr IGNG Ngoerah General Hospital and the relationship between the GBS subtype characteristics on electrodiagnostic examination and CSF protein levels. Methods: This study used an analytic observational study with a cross-sectional study design with consecutive sampling. The sample of this study is 42 GBS patients (21 patients with normal total protein and 21 patients with increased total protein) at Prof Dr IGNG Ngoerah General Hospital, Denpasar-Bali. Data is collected through medical records and analyzed by Chi-Square test. Results: There is no correlation between the increase in total CSF protein in GBS patients and the GBS subtype on electrodiagnostic examination (p=0.215). Discussion: The most common GBS subtype is the AIDP type. Although the average amount of CSF protein was higher in the AIDP, AMAN and AMSAN subtypes respectively, the increase in total CSF protein did not have a significant relationship to the GBS subtype in Prof Dr IGNG Ngoerah General Hospital, Denpasar-Bali. Keywords: Guillain-Barre syndrome, autoimmune, total protein, cerebrospinal fluid, AIDP, AMAN, AMSAN

Clinical and demographic characteristics of guillain-barré syndrome (GBS) variants in a tertiary care hospital.

Objective: To evaluate clinical and demographic characteristics of guillian-barre syndrome. Material &amp; Methods: Study Design: Descriptive, Cross-sectional study. Setting: Department of Neurology, Lady Reading Hospital, Peshawar. Period: December 2020 to March 2022. Results: The study identified 117 patients with GBS, and the mean age was 43.59 ± 17.39 years. There were 78 male (67%) and 39 female (33%) patients. The most common GBS subtype was AMAN (46.2%), followed by AMSAN (34.2%), AIDP (16.2%), and atypical (2.6%). The study found a significant difference in gender distribution between the two subtypes, with male patients being more likely to have axonal GBS. Regarding antecedent events, the study found that 61% of patients had a preceding illness, with gastrointestinal infection being the most common (50%). The clinical features of GBS varied by subtype, with patients with AMAN being more likely to have walking difficulty, symmetric ascending weakness, and numbness/tingling, while patients with AIDP were more likely to have a fever and respiratory distress. Conclusion: The study's findings are broadly consistent with previous research, highlighting the heterogeneity of GBS in terms of its presentation and antecedent events. However, other differentiating points that need to be taken into consideration have also been mentioned. Furthermore, work needs to be done to raise awareness among medical professionals in Nothern Pakistan regarding the salient features of the problem.

Study of clinical profile and outcome in children aged 1-12 years presenting with Guillain Barre syndrome

Background: Guillain-Barré syndrome (GBS) is a group of clinical syndromes involving acute polyneu­ropathy secondary to an immune-mediated process. The aim of the research is to study clinical profile and outcome in children aged 1-12 years presenting with GBS. Methods: It is a prospective plus retrospective observational study (5-year retrospective + 18 months prospective). All cases of GBS admitted from January 2020-july 2021 were enrolled for the study and retrospective cases were taken from case record from January 2015 onwards in a tertiary care centre. Results: Total 45 children were enrolled. The male: female ratio was 1.64:1. Mean age at presentation was 6.3 years, in a range of 1.5 -11.5 years of age. History of antecedent illness prior to the occurrence of GBS was present in 93.3% patients with upper respiratory tract infection being most common seen in 53.3% patients. Following variants of GBS were seen on NCV study performed in 34 patients, the most common was AIDP in 46.7% patients, followed by AMAN in 22.2% subjects and AMSAN in 6.7% patients. All children presented with lower limb weakness which progressed to involve upper limbs. Other associated features like bulbar cranial nerve involvement, respiratory muscle weakness, sensory symptoms and autonomic disturbance (arrythmia, hypotension, tachycardia, bradycardia) was observed in 30,12,7,16 patients respectively. Out of the total 45 patients, 12 patients required mechanical ventilation. Conclusions: Male predominance seen in GBS. AIDP the most common subtype of GBS. Respiratory distress and autonomic instability were associated with greater severity of Hughes disability score. Factors associated with poor outcome were sensory symptoms, autonomic instability, respiratory distress and bulbar cranial nerve involvement.

Detection of anti-ganglioside antibodies in Guillain-Barré syndrome.

Gangliosides are a class of glycosphingolipid molecules that are highly enriched in cellular membranes of the nervous system. The gangliosides associated with autoimmune diseases of the nervous system are mainly GM1, GD1a, GalNAc-GD1a, GM1b, GD3, CD1b, GT1a, and GQ1b. Multiple antibodies recognizing gangliosides are associated with some acute or chronic peripheral neuropathies, especially Guillain-Barré syndrome (GBS) and its clinical variants. Antibodies binding to gangliosides can activate complement system and recruit macrophages on the axolemma at the nodes of Ranvier of motor fibers, which are found in the course of GBS, causing axonal degeneration and reversible conduction block or conduction failure. Testing of anti-gangliosides autoantibodies is helpful for diagnosis of autoimmune peripheral neuropathies or support the diagnosis of the subtypes. These anti-gangliosides antibodies are usually detected by several qualitative or quantitative methods, particularly enzyme-linked immunosorbent assay (ELISA) and immunodot assays, which have been commercialized or established in-house worldwide. Herein, we introduce the methods and clinical applications of these assays in the diagnosis of autoimmune peripheral neuropathies. Anti-gangliosides antibodies are diagnostic markers of GBS subtypes. We use GBS as an example to explain the role of anti-gangliosides antibodies in the pathogenesis and diagnostic classification of neuropathies.

Frequency, Age, Gender Distribution, and Seasonal Variation of Guillain-Barré Syndrome in a Province of Pakistan: A Retrospective Study

Characterized by the sudden onset of muscle weakness, Guillain-Barré Syndrome (GBS) is a rare autoimmune disorder which can progress to paralysis. GBS has different subtypes based on the clinical and electrophysiological characteristics, including acute inflammatory demyelinating polyradiculoneuropathy (AIDP), axonal GBS (AMAN and AMSAN), and Miller Fisher Syndrome (MFS). Objective: To study frequency of the disease in Khyber Pakhtunkhwa. Methods: In this retrospective study, medical records of 39 patients diagnosed with GBS at Lady Reading Hospital Peshawar, Pakistan, were analyzed to determine the prevalence of GBS subtypes in the country. Results: The results showed that the most prevalent subtype of GBS was AMAN, accounting for 59% of cases, followed by AMSAN at 25.6%, and AIDP at 15.3%. The axonal variety made up 84.6% of the total GBS cases in this study. On average, the patients with AMSAN were 39.2 years old, while patients with AMAN and AIDP were relatively younger, with mean ages of 30 and 28 years, respectively. There was a male predominance in all subtypes except for AIDP, which showed equal distribution. Conclusions: These findings provide valuable information on the distribution of GBS subtypes in Peshawar, Pakistan, which may have implications for the diagnosis and management of GBS in the country. Additionally, the study's results can contribute to the global understanding of GBS epidemiology and may help improve the diagnosis and treatment of GBS patients worldwide.

When Guillain-Barre syndrome took a wrong path

The pharyngeal cervical brachial (PCB) variant of Guillain-Barre-syndrome (GBS) is defined by rapidly progressive oropharyngeal and cervicobrachial weakness associated with areflexia in upper limbs. On nerve conduction study PCB represents a localized subtype of GBS characterized by axonal rather than demyelinating neuropathy. PCB is often misdiagnosed as brainstem stroke, myasthenia gravis or botulism. Herein we describe a case of symmetric descending type of acute flaccid paralysis with predominant upper limb involvement and also lower limb involvement diagnosed as a PCB- GBS overlap mainly based on nerve conduction study.

GUİLLAİN-BARRÉ SENDROMUNDA AĞIR ÖZÜRLÜLÜĞÜN ERKEN BELİRTEÇLERİ

Objective&#x0D; Guillain-Barré syndrome (GBS) is an autoimmune&#x0D; neurological disorder with non-negligible morbidity&#x0D; and mortality. This study aimed to evaluate different&#x0D; patient characteristics and laboratory findings as early&#x0D; predictors of severe disability in GBS patients.&#x0D; Material and Method&#x0D; We retrospectively reviewed the medical records of&#x0D; 121 patients diagnosed with GBS between January&#x0D; 1, 2018, and December 31, 2021. Data regarding&#x0D; demographic characteristics, presenting complaints,&#x0D; co-morbidities, previous infection history, neurological&#x0D; examination findings, GBS Disability Scores (GDS)&#x0D; on the 1st day and by the end of the first month,&#x0D; laboratory parameters of serological and cerebrospinal&#x0D; fluid (CSF) examination, electromyoneurography&#x0D; results, GBS subtypes, treatments, treatment-related&#x0D; complications, and prognoses were recorded.&#x0D; Results&#x0D; The median age of the 121 patients was 58 (20–87)&#x0D; years (n = 73 males, 60%). The average GDS was&#x0D; 3 on admission and 2 at the end of the first month.&#x0D; The serum C-reactive protein (CRP) and CSF protein&#x0D; levels were raised, while vitamin D levels were&#x0D; reduced. Advanced age, cranial nerve involvement,&#x0D; history of infection, admission to the intensive care&#x0D; unit (ICU), need for mechanical ventilation (MV),&#x0D; presence of complications, high plasma CRP levels,&#x0D; neutrophil-lymphocyte ratio (NLR), and plateletlymphocyte&#x0D; ratio (PLO) were significantly associated&#x0D; with severe disability in GBS patients at day 1 and at&#x0D; the end of the first month.&#x0D; Conclusion&#x0D; We identified multiple characteristics that can predict&#x0D; severe disability in GBS patients.