Variant Of Guillain-Barre Syndrome Research Articles

Acute encephalitis with flaccid paralysis following an adenoviral infection presenting as a rare variant of Guillain Barre syndrome in a school-age child

Acute encephalitis with flaccid paralysis following an adenoviral infection presenting as a rare variant of Guillain Barre syndrome in a school-age child

Unmasking Ataxic Guillain–Barre Syndrome: A Rare Presentation

Abstract A 68-year-old right-handed male with a history of hypertension presented with unsteadiness while walking for 5 days preceded by a vague pain in the left shoulder and both thighs and paresthesias in all extremities. It rapidly progressed to severe gait ataxia, bilateral facial palsy, and speech difficulties. On examination, he had bilateral lower motor neuron type of facial palsy, a wide-based stance, gait ataxia, left-sided hypotonia, cerebellar signs, and generalized areflexia. He had left-sided hypotonia and normal power of all four limbs. Sensory system examination was normal, and Rhomberg’s sign was negative. He had no ophthalmoplegia. While initial considerations pointed toward a cerebrovascular accident, normal magnetic resonance imaging results prompted us to do a lumbar puncture and cerebrospinal fluid analysis, which revealed albuminocytological dissociation indicative of Guillain–Barré Syndrome (GBS). Nerve conduction studies then confirmed our diagnosis. Given these results and a preceding respiratory infection, the patient was diagnosed with a rare ataxic variant of GBS. The patient was treated with plasmapheresis following which he showed dramatic clinical improvement. He was subsequently able to walk without support and perform the majority of his day-to-day activities on his own. He was discharged in a hemodynamically stable condition. This case report, thus, highlights a rare ataxic variant of GBS presenting with acute onset ataxia, areflexia, and facial diplegia. Thus, understanding the GBS and its variants is crucial to managing rare presentations swiftly and avoiding severe complications. It has tremendous scope for further research to understand the pathophysiological dynamics completely and find more targeted therapeutic approaches. We, thus, present a rare case of an ataxic variant of GBS with acute onset ataxia, areflexia, and facial diplegia.

Journey of Guillain Barre syndrome from the pre-pandemic era to the pandemic era: A 4-year retrospective study.

To study demographic and clinical profiles of Guillain Barre syndrome (GBS) in the pre-pandemic and coronavirus disease 2019 (COVID-19) pandemic era and to compare the GBS incidence, severity, and its outcome in the pre-pandemic and pandemic eras. This is a 4-year retrospective study done in a tertiary care hospital in Delhi, India, between March 2018 and March 2022. Patients were divided into the pre-pandemic era and pandemic era (2 years before and 2 years after March 2020). The number of patients (N) was 25 in the pandemic/vaccine era, while N = 49 in the pre-pandemic era. The mean duration of hospitalization was significantly higher (P = 0.03) during the pandemic era (10.68 ± 6.67 days) compared to the pre-pandemic era (7.59 ± 3.55 days). There was no statistical difference in age (P = 0.56), gender (P = 0.70), GBS variants (P = 0.40), clinical spectrum, antecedent infection (P = 0.91), Hughes Disability Score on admission and discharge (P = 0.93 and P = 0.52, respectively), respiratory involvement requiring a ventilator (P = 0.19), and mortality (P = 0.26) in both the eras. Our study showed no association of the incidence of GBS with the ongoing COVID-19 pandemic. The mean hospitalization days were significantly increased during COVID-19 in view of associated respiratory involvement. The commonly held hypothesis of the increase in GBS cases during the pandemic/vaccine era has not been observed in our study.

When Guillain–Barré Syndrome Defies Expectations: A Case of Atypical Features

Abstract Guillain–Barré syndrome (GBS) is one of the most common causes of acute flaccid paralysis. In typical GBS, there are no central nervous system (CNS) features. GBS with prominent CNS involvement can be seen in uncommon variants of GBS (such as Miller Fisher Syndrome and Bickerstaff brainstem encephalitis [BBE]) or rarely, when it coexists with other conditions such as acute disseminated encephalomyelitis (ADEM). We report a case of a 35-year-old male who presented with fever followed by ascending lower motor neuron type paraparesis suggestive of GBS which progressed rapidly to involve the respiratory muscles necessitating invasive mechanical ventilation. The patient had many atypical features, namely, persistent fever, early bladder and bowel involvement, facial twitching, ophthalmoplegia with ptosis, and a comatose state. Based on these clinical features and the investigations done, a diagnosis of GBS with BBE with a possibility of ADEM was made. The patient was managed intensively, but there was no improvement in the neurological manifestations and the patient succumbed to the illness due to ventilator-associated complications.

A case of variant of GBS with positive serum ganglioside GD3 IgG antibody

BackgroundAcute bulbar palsy-plus (ABPp) syndrome is an unusual variant of Guillain-Barré syndrome (GBS). Anti-GT1a and anti-GQ1b antibodies have been reported in patients with ABPp, but without reports related to GD3 antibodies.MethodsClinical data of a patient diagnosed as ABPp syndrome were reviewed clinically. And we summarized the GBS patients with ABP and facial paralysis reported in the literature.ResultsWe reported a 13-year-old girl presented with asymmetric bifacial weakness, bulbar palsy and transient limb numbness, and had positive serum IgG anti-GD3 antibody. Through reviewing the GBS patients with ABP and facial paralysis reported previously, we found that facial palsy could be unilateral or bilateral. The bilateral facial palsy could present successively or simultaneously, and could be symmetrical or asymmetrical. Other common symptoms included ophthalmoplegia, sensory abnormality and ataxia. IgG anti-GT1a and IgG anti-GQ1b antibodies were the most frequent. Most of the patients had full recovery within two weeks to one year of follow-up.ConclusionsWe reported a patient with asymmetric bifacial palsy and bulbar palsy, which seemed to fit the diagnosis of ABPp syndrome. This was the first report of ABPp variant of GBS with positive serum ganglioside GD3 IgG antibody.

A rare case of a pharyngeal-cervical- brachial variant of Guillain–Barré syndrome

Abstract: Guillain–Barre syndrome (GBS) is an acute, usually symmetrical, and typically de myelinating polyneuropathy, characterized by progressive, ascending paralysis and areflexia, with or without abnormal sensory function. Therapeutic plasma exchange(TPE) was the first therapeutic modality to impact the disease favorably, and several major randomized controlled trials (RCTs) have confirmed its efficacy. Our case is unique, as this critically ill patient admitted in the intensive care unit (ICU), developed a rare form of GBS, the PCB variant of the disease, and was successfully treated with TPE. The diagnosis of PCB can be challenging due to atypical clinical features but its unique presentation does provide sufficient clues to narrow the differential diagnosis, which is helpful in starting empirical therapy at the earliest. Immunomodulation with plasma exchange and IVIG have been found to improve treatment outcomes; 70%–80% of patients usually have a complete recovery. Early diagnosis is very important in the case of the PCB variant of GBS to enable timely intervention. Diagnosis can be made on history and neurological findings alone.

Comparison of Clinical Features, Short-Term Outcome of Guillain-Barré Syndrome Between Adults and Children: A Retrospective Study in Vietnam

BackgroundDespite extensive research on Guillain-Barré syndrome (GBS) in adults and children, there is a lack of comparison regarding short-term outcomes in various age groups. Our study aims to elucidate the differences in clinical features and short-term outcomes in Vietnam. MethodsAfter retrospective data collection, we compared clinical features in patients with GBS aged ≤16 years at Children's Hospital 2 and aged >16 years at University Medical Center Ho Chi Minh City from 2017 to 2021. A positive short-term outcome was recorded if patients had a GBS Disability Score of 0 to 2 at hospital discharge. ResultsWe analyzed 109 adults (58.7% males; mean age 50.6 ± 17.7) and 111 children (58.6% males; mean age 7.2 ± 4.9). Comparable antecedent infection and immunization incidence in both groups were observed (35.8% vs 45.9%, P > 0.05). Pain and sensory disturbance were the most common onset symptom in adults (57.8%), whereas lower limb weakness predominated in children (61.3%). Ophthalmoplegia (18.3% vs 5.4%), pain, sensory disturbance (85.3% vs 67.6%), ataxia (33.0% vs 15.3%) were more prevalent in adults (P < 0.05). The axonal subtype was prominent in both adults (51.4%) and children (53.2%). Patients were classified into: classic GBS (49.5% and 68.5%), GBS variants (11.0% and 15.3%), classic Miller Fisher syndrome (MFS) (1.8% and 1.8%), MFS variants (2.8% and 0%), and GBS/MFS overlap (34.9% and 14.4%). Short-term outcomes did not significantly differ based on age. ConclusionsAge-related variations in clinical features were observed, but adults and children exhibited similar short-term functional outcomes.

The sural-sparing pattern in clinical variants and electrophysiological subtypes of Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide and can be classified into electrophysiological subtypes and clinical variants. This study aimed to compare the frequency of the sural-sparing pattern (SSP) in subtypes and variants of GBS. This retrospective cohort study analyzed clinical and electrophysiological data of 171 patients with GBS hospitalized in public and private hospitals of Natal, Rio Grande do Norte, Brazil, between 1994 and 2018; all cases were followed up by the same neurologist in a reference neurology center. Patients were classified according to electrophysiological subtypes and clinical variants, and the SSP frequency was compared in both categories. The exact Fisher test and Bonferroni correction were used for statistical analysis. The SSP was present in 53% (57 of 107) of the patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 8% (4 of 48) of the patients with axonal subtypes, and 31% (5 of 16) of the equivocal cases. The SSP frequency in the AIDP was significantly higher than in the axonal subtypes (p < 0.0001); the value was kept high after serial electrophysiological examinations. Only the paraparetic subtype did not present SSP. The SSP may be present in AIDP and axonal subtypes, including acute motor axonal neuropathy, but it is significantly more present in AIDP. Moreover, the clinical variants reflect a specific pathological process and are correlated to its typical electrophysiological subtype, affecting the SSP frequency.

MILLER FISHER SYNDROME: A RARE BENIGN VARIANT OF GUILLAIN- BARRE SYNDROME

Abstract&#x0D; Introduction : Guillain-Barré Syndrome (GBS) is a broad category of syndromes that includes several types of acute immune-mediated polyneuropathy. Miller Fisher Syndrome (MFS) is a scarce variant of GBS (1-2:1,000,000) that presents at least 2 of ataxia, areflexia, and ophthalmoplegia.&#x0D; Case Illustration : A 32 years-old-man came to JEC-Candi with ophthalmic discomfort and exhibited a week onset of total ophthalmoplegia, ptosis, diplopia, and lagophthalmos. Two weeks backward, the patient had a respiratory infection then emerged symmetrical sensory abnormalities (glove-and-stocking-type pinprick sensations in distal extremities). Neurological examinations were performed, and the patient got multiple cranial nerve weaknesses (oculomotor, trochlear, abducens, trigeminal, and facial) both in motoric and sensory, ataxia (positive Romberg sign, stepping test, gait, shallow-knee- bend), and negative pathologic reflexes. Hematology showed leukocytosis, MRI and CT-Scan were normal. Pulse therapy was given (Methylprednisolone 1g/day) for three days, neuroprotector and artificial tears added. Ataxia and diplopia improved, then the patient was referred to Kariadi Hospital for five-times plasmapheresis that involved neurologist and internist. Ophthalmoplegia improved slowly, and lagophthalmos disappeared one month after hospital admission.&#x0D; Discussion : GBS subvariant could overlap; this case presents bifacial weakness with paresthesia and MFS. Viral infection precedes neurological symptoms in most cases, with an average of 10 days of the incubation period. Ataxia, diplopia, and ophthalmoplegia may confuse the physician and presume an upper motor neuron sign or central cause. Steroid and plasmapheresis evince meaningful upturn.&#x0D; Conclusion : This rare disease requires a multidisciplinary approach to cure and diagnose. Nonetheless, MFS has a good prognosis. Appropriate treatment selection will enhance patients' quality of life.

Correlation of Cerebrospinal Fluid Total Protein and Serum Neutrophil-to-Lymphocyte Ratio with Clinical Outcomes of Guillain-Barre Syndrome Variants.

To evaluate the correlation of cerebrospinal fluid total protein and serum neutrophil-to-lymphocyte ratio with the clinical outcomes and the various clinical and electrophysiological variants of Guillain-Barre syndrome. Cross-sectional study. Place and Duration of the Study: Department of Neurology, Mayo Hospital and King Edward Medical University, Lahore, Pakistan, from November 2022 to April 2023. Fourty-six Guillain-Barre syndrome patients, aged 12-70 years, were included in the study diagnosed by using the Brighton's criteria. Functional disability and respiratory insufficiency were assessed by using the modified Hughes disability score and the Erasmus Guillain-Barre syndrome respiratory insufficiency score, respectively.Serum neutrophil-to-lymphocyte ratio and cerebrospinal fluid total protein were calculated for each patient at the time of admission. Axonal variants had a higher mean neutrophil-to-lymphocyte ratio (5.29 ± 4.38) than demyelinating variants (4.71 ± 3.4) and Miller-Fischer syndrome (3 ± 2.828). This ratio was positively correlated with the modified Hughes's disability score (r = 0.790, p = 0.001) and the Erasmus Guillain-Barre syndrome respiratory insufficiency score (r = 0.936, p = 0.002). Mean cerebrospinal fluid total protein was higher for demyelinating (218 ± 136 mg/dl) than axonal variants (86 ± 56 mg/dl) and Miller-Fischer syndrome (34 ± 21 mg/dl). However, higher modified Hughes disability score (4-6) (r = 0.020, p = 0.117) and a high Erasmus Guillain-Barre syndrome respiratory insufficiency score (5-7) (r = 0.115, p = 0.302) did not significantly affect mean cerebrospinal fluid total proteins. Serum neutrophil-to-lymphocyte ratio can be regarded as a reliable biomarker to assess disease severity and clinical outcome in Guillain-Barre syndrome. Cerebrospinal fluid total protein is a poor predictor of the prognosis and severity of Guillain-Barre syndrome. Guillain-Barre syndrome (GBS), Clinical outcome, Cerebrospinal fluid total protein (CSF-TP), Neutrophil-to-lymphocytic ratio (NLR), Prognostic biomarker.

Evaluating yield and utilization of ganglioside antibody testing in clinical practice

Background and objectivesGanglioside antibodies can help diagnose distinct acute and chronic inflammatory neuropathies including axonal variants of Guillain-Barre syndrome, Miller-Fisher syndrome (MFS), multifocal motor neuropathy, and chronic sensory ataxic neuropathies. Because ganglioside antibody testing may be routinely ordered in patients with suspected inflammatory neuropathy, we sought to evaluate its yield and utilization in clinical practice. MethodsWe performed a retrospective chart review of all patients at London Health Sciences Centre who underwent ganglioside antibody testing between April 2019 and August 2023. The disease phenotype was determined for each patient, and the proportion of all tests that yielded a true-positive result was calculated. Ganglioside antibody positivity was classified as a true-positive result if the disease phenotype was robustly associated with the detected ganglioside antibody and there was no other more likely diagnosis. ResultsWe identified 92 patients who underwent ganglioside antibody testing. One patient (1%) was classified as having a true-positive result; this patient had GQ1b-IgG positivity with MFS. Among 92 patients tested, 20 patients (22%) had a disease phenotype that was considered to be robustly associated with ganglioside antibody positivity. ConclusionsThe yield of ganglioside antibody testing in clinical practice is low. We found that this testing is frequently ordered in patients with disease phenotypes that are not robustly associated with ganglioside antibody positivity, indicating that suboptimal test utilization is a primary contributor to its low yield. Restricting ganglioside antibody testing to patients with characteristic disease phenotypes would be valuable to improving yield and utilization of this testing.

An Observational Study on Factors Causing Variable Response to Therapeutic Doses of Intravenous Immunoglobulin in Acute Immune-Mediated Polyneuropathy: A Tertiary Care Center Experience

Context: Immune-mediated neuropathies, notably Guillain–Barré syndrome (GBS) and its subtypes, exhibit diverse clinical presentations. Intravenous immunoglobulin (IVIG) is a standard treatment for these conditions, but the variable clinical course complicates outcome prediction. Despite standard IVIG dosing, some patients continue to deteriorate. Aim: The aim of this study was to assess the factors responsible for variable response to therapeutic doses of immunoglobulins in acute immune-mediated polyneuropathy. Settings and Design: This was a prospective, observational study. Materials and Methods: Acute immune-mediated polyneuropathy cases within 14 days of symptom onset and receiving IVIG were recruited. Hughes disability score (HDS), modified Erasmus GBS outcome score (mEGOS), and modified Rankin score (mRS) were assessed before IVIG, immediately after IVIG, and 4 weeks thereafter. Statistical Analysis Used: Categorical variables are expressed as percentages, and continuous variables are presented as mean ± standard deviation. Relationships between parameters were assessed using analysis of variance and multivariate analysis of covariance. Correlations were measured using Pearson’s correlation, and proportions were compared using the Chi-square test or Fisher’s exact test. Results: Among 60 GBS patients, the median age was 38 years, with upper respiratory tract infection as the most common antecedent infection (60%). The most common variant was pure motor type, while demyelinating (88.33%) was the most common electrophysiological subtype. Outcome correlated with post-IVIG scores of HDS and mRS. Linear regression analysis showed a positive correlation between onset-to-treatment duration and HDS and mEGOS scores after 4 weeks of IVIG, while mRS showed minimal correlation. No significant correlations were found between antecedent infection, gender, nerve conduction study pattern, GBS variant, and scores of HDS, mEGOS, and mRS. Conclusions: The study highlights the significance of onset-to-treatment duration. Factors with unfavorable course were age, delay in receiving IVIG, respiratory involvement, dysautonomia, cranial nerve involvement, and those with higher mEGOS scores at the 7th day of admission. Seasonal trends should not be overlooked. Serial evaluation of disability scores can predict the varying response to IVIG.

Sural nerve involvement in patients with acute inflammatory demyelinating polyneuropathy variant of Guillain-Barre syndrome with sural sparing at initial presentation

Introduction: Neurophysiological testing is a valuable tool in the diagnosis of Guillain-Barre syndrome (GBS). Sural sparing is a usual feature of acute inflammatory demyelinating polyneuropathy (AIDP) type GBS. However, sural involvement has been reported in later stages of GBS. It is important to identify patterns of sural nerve involvement to differentiate GBS from its mimickers and to stage the disease. This research aimed to detect the pattern of sural nerve involvement in AIDP-GBS cases with normal electrophysiological responses in the sural nerve at the beginning.Objectives: To determine the location and timing of sural nerve involvement in AIDP-GBS.Methods: This prospective follow up study included diagnosed cases of AIDP-GBS with preserved bilateral sural responses. Nerve conduction and somatosensory evoked potentials (SSEP) were done on admission and weekly thereafter for four consecutive weeks. The last evaluation was done four weeks after the fourth study.Results: All patients (100%) showed normal distal sural responses over the initial four weeks of follow up. They continued to remain normal up to eight weeks in eight patients (53.3%). Two patients had gradual prolongation of their sural SSEP on consecutive studies. One of them had gradual reduction of sural sensory nerve action potential and nerve conduction velocity along with the prolongation of sural SSEP latencies. The difference of SSEP latency increments in the left sural nerve of these two patients was statistically significant (p&lt;0.05). The right sural SSEP latency difference was not significant.Conclusion: Sparing of the distal sural sensory response was demonstrated in 100% of AIDP-GBS cases during the first four weeks of follow up. More than 50% of the cohort demonstrated preserved sural sensory responses for eight weeks from the initial presentation. Two out of fifteen patients showed statistically significant proximal sural sensory pathway involvement with increasing SSEP latencies. This finding suggests that in some patients, the sural sensory pathway may get affected at its proximal segments or at the central nervous system before the distal nerve is affected.

A review of the clinical and epidemiological aspects of Guillain-Barré syndrome in patients infected with SARS-CoV-2: An Extended Comprehensive Review

Since 2019, cases of patients with COVID-19 who developed Guillain-Barré Syndrome (GBS) have been reported. This review explores mechanisms that explain pathophysiology, clinical features, laboratory findings, and imaging characteristics in these patients.Methodology: A bibliographic search was made of studies on the topic published in NCBI and Scielo, between December 2019 and April 2022.Results: Ninety articles were found, 53 of which were included in this article. No studies were found that explain an association between GBS and COVID19. Specific clinical manifestations found were areflexia (56.95%), hyporeflexia (19.44%), muscle weakness (65.28%), gait disturbance (12.5%), hypoesthesia (26.39%), paresthesia (30.55%), and micturition disturbance (6.94%). The CSF findings included albumin-cytological dissociation (66.67%), and an average protein level of 140.23 mg/dL (SD: 106.71). Some cases reported enhancement of the cervical leptomeningeal, brainstem and cranial nerves on magnetic resonance imaging tests. The predominant variant of GBS was acute inflammatory demyelinating polyneuropathy (56.94%). The findings in the nerve conduction studies were the absence of F waves (61.54%), increased distal motor latency (80%), decreased motor amplitude (93.1%), and decreased motor conduction velocity (75%). In addition, the nerves mainly involved were the tibial (20.21%), peroneal (24.47%), median (20.21%), and ulnar (18.09%). The most frequent alteration of cranial nerves was bilateral (25%) and unilateral (13.89%) facial palsy.Conclusion: The primary GBS variant was Acute Inflammatory Demyelinating Polyneuropathy. Cerebrospinal fluid analysis revealed albumin-cytological dissociation as the most common finding, and MRI tests showed cranial nerves enhancements. An additional differential feature was the lower commitment of the autonomous system.

Injudicious use of cranio-spinal imaging in Guillain-Barré syndrome in a low resource country.

Background: Many patients being investigated for Guillain-Barré syndrome (GBS) undergo unnecessary neuroimaging. The objective of this study was to determine the proportion of patients with GBS undergoing neuroimaging investigation, and to investigate any association with different GBS variants using the Brighton criteria. Methods: This cross-sectional observational study was conducted in the leading tertiary care hospital in Pakistan; 148 patients being investigated for, and subsequently diagnosed with GBS between January 2017 and March 2020 were enrolled. Participants were asked if they had undergone neuroimaging of the craniospinal axis before or during hospital admission, and the purpose of any computed tomography (CT) scan was investigated. We enquired whether fundoscopy had been performed before lumbar puncture (LP) and determined the level of certainty based on the Brighton criteria. Results: The majority of participants were men (n = 107, 73%), with a mean age of 42.85 ± 18.40 years. The mean waiting time to their first interaction with a neurologist was 5.20 ± 4.01 days, and the demyelinating variant of GBS was more common than the axonal variant (1.6:1). Most patients were diagnosed with level I certainty using the Brighton criteria (n = 113, 76%). Brain and spine magnetic resonance imaging (MRI) were performed ahead of admission in 48 (32%) and 59 (39%) patients, respectively. Brain CT scan was performed in 121 (82%) patients before LP, while 27 (18%) only underwent fundoscopic examination before LP. Conclusion: Clinical examination is fundamental in the diagnosis of GBS. Neuroimaging may be inappropriate and unnecessary, and may detract attention from crucial peripheral neuropathy measures while misusing limited resources.

Ataxic variant of Guillain Barre syndrome: a case report

A 17-year-old girl was admitted after acute onset of unsteady gait succeeding acute gastroenteritis. Neurological examination reported normal power in all four limbs, impaired finger-nose, heel-shin tests, areflexia and ataxic gait. We eliminated other diseases with cerebellar symptoms; for example, Wernicke encephalopathy, multiple sclerosis, cerebellar vascular disease , encephalitis in the brain stem and cerebellum. Blood serum collected from the patient during the acute phase showed no anti-ganglioside antibodies. As the patient presented with evident cerebellar ataxia without muscle weakness, ophthalmoplegia or proprioceptive sensory disruption a diagnosis of ataxic form of Guillain-Barré syndrome (GBS) after nerve conduction studies. Though ataxic GBS is not a settled impression, we should have to give heed to the potential existence of such a scarce GBS variant.

Interleukin-10 promoter polymorphisms and haplotypes in patients with Guillain-Barré syndrome.

Interleukin-10 (IL-10) is a multifunctional cytokine that exerts both pro- and anti-inflammatory effects on the immune system as well as in the pathogenesis of Guillain-Barré syndrome (GBS). We investigated whether the three common polymorphisms -1082 G/A(rs1800896), -819 C/T(rs1800871), and -592 C/A(rs1800872) in the promoter region of IL-10 have any influence on the susceptibility, severity, and clinical outcome of GBS. IL-10 promoter polymorphisms were investigated in 152 patients with GBS and 152 healthy controls from Bangladesh using polymerase chain reaction and restriction fragment length polymorphism (PCR-RFLP), and allele-specific oligonucleotide-PCR (ASO-PCR). Haplotype patterns and frequencies were analyzed using Heatmaply R-package, chi-square, and Fisher's exact test. The serum level of IL-10 was measured through enzyme-linked immunosorbent assays. p-values < 0.05 were considered statistically significant. IL-10 promoter polymorphisms -1082 G/A, -819 C/T, and -592 C/A were not associated with GBS susceptibility. The homozygous -819 TT genotype showed a tendency with susceptibility (p = 0.029; pc = 0.08) and was prevalent in axonal variants of GBS compared to demyelinating subtypes and controls (p = 0.042, OR = 8.67, 95% CI = 1.03-72.97; pc = 0.123 and p = 0.005, OR = 4.2, 95% CI = 1.55-11.40; pc = 0.015, respectively). Haplotype analysis revealed 19 patterns of genotypes and high IL-10 expression haplotype combinations (GCC/GTA, GCC/ATA, and GCC/GCA) may have influence on disease severity (p = 0.026; pc = 0.078). Serum expression of IL-10 was elevated in GBS patients ([GBS, 12.16 ± 45.71] vs. [HC, 0.65 ± 5.17] pg/mL; p = 0.0027) and varied with disease severity ([severe-GBS, 15.25 ± 51.72] vs. [mild-GBS, 3.59 ± 19.79] pg/mL, p = 0.046). The -819 TT genotypes influence axonal GBS, and high frequency of IL-10 expression haplotype combination with elevated serum IL-10 may play an important role in disease severity.

Systemic Lupus Erythematosus Initially Presenting as Acute Motor andSensory Axonal Neuropathy Variant ofGuillain-Barre Syndrome ina Healthy Active Duty Female.

Guillain-Barre syndrome (GBS) is an acute monophasic immune-mediated polyradiculoneuropathy characterized by rapidly evolving ascending weakness, mild sensory loss, and hypo- or areflexia, typically progressing to peak symptoms over the course of 4 weeks. The precise mechanism is unclear but is proposed to be an immune-mediated reaction with the generation of antibodies against peripheral nerves triggered by a preceding viral infection. Acute motor and sensory axonal neuropathy (AMSAN) is a rare and severe variant of Guillain-Barre syndrome with limited published literature. Discussion of risk factors for this subtype has not been done in a systematic way. This case report involves a 34-year-old, active duty, West African female, who immigrated to the United States in 2019. She presented with worsening diplopia, bilateral distal upper and lower extremity paresthesias as well as progressively worsening bilateral upper extremity weakness. Her clinical picture was complicated by constitutional symptoms, diffuse lymphadenopathy, no preceding viral illness, and marked clinical deterioration. Ultimately, she was diagnosed with acute motor and sensory axonal neuropathy in the setting of a new diagnosis of systemic lupus erythematosus, a rarely described association emphasizing the importance of a thorough evaluation for underlying causes of acute neurologic pathologies.

Clinical Profile and Predictors of Outcome of Guillain Barre Syndrome Variants among Children Admitted in the Pediatric Department of a Tertiary Care Hospital, Karachi

Background: Guillain Barre Syndrome (GBS) is one of the leading cause of Acute Flaccid Paralysis presenting in pediatric emergency, marking incidents in Asian countries as 3-4 per 100, 000 population. To improve outcome, an accurate and early recognition of predictors of bad prognosis is required. Objective: To identify requirement of intensive monitoring and aggressive treatment to help the health system in decreasing morbidity and mortality associated with Guillain Barre Syndrome. Materials and Methods: This is a cross-sectional study, conducted at Dr. RKMP Civil Hospital Karachi. Medical records of patients &gt;3 months to 12 years of age, admitted with diagnosis of Guillian Barre syndrome from Jan 2017 to June 2022 were reviewed. Data was analyzed in SPSS version 23. For independent variables, frequency and percentage was used. Results: A total of 46 patients were enrolled in the study, mean age of study participants was 80.26 ± 31.7 months. The most common variant of GBS identified was AIDP in 19 (41.3%) followed by ASMAN 14 (30.4%). A positive association between winter season with 2.7 (95% CI 1.4-5.8) and progression within 7 days with 3.2 (95% CI 1.9-6.7) was found with mortality. Conclusion: Acute inflammatory demyelinating polyneuropathy was the most common variant of Gullian-Barre syndrome with male predominance. Presentation in winter seasons and progression of disease in less than 7 days are risk factors for mortality. Bulbar palsy is an independent risk factor for mechanical ventilation, irrespective of GBS variant. Keywords: Acute inflammatory demyelinating polyneuropathy (AIDP), Guillain Barre Syndrome (GBS), Pediatric population, Bulbar palsay, Areflexia, Acute motor axonal neuropathy (AMAN).

Guillain-Barré Syndrome with multiple cranial neuropathies

Background. Guillain-Barré Syndrome (GBS) is an autoimmune reaction against peripheral nerves that manifests clinically as acute polyradiculoneuropathy. Classic sensory-motor, pure motor, paraparesis, pharyngeal-cervical-brachial, Bickerstaff brainstem encephalitis, pure sensory, bilateral facial palsy with paresthesia, and Miller-Fisher syndrome are all known variants of GBS. This case report seeks to describe a case of GBS with involvement of the cranial nerves. Case report. A 39-year-old right-handed Balinese woman presented with LMN (Lower Motor Neuron) type paraparesis with ascending paralysis and involvement of cranial nerves III through XII. The results of the LCS (Liquor Cerebro-Spinal) demonstrate albuminocytological dissociation. According to the patient’s EMNG (electromyoneurography) findings, Guillain-Barré Syndrome was of the AMAN (acute motor axonal neuropathy) variety. The patient was diagnosed with multiple cranial neuropathies and GBS of the AMAN type. EGRIS (Erasmus GBS Respiratory Insufficiency Score) was 3, and EGOS (Erasmus GBS Obstructive Sleep Apnea Score) was also 3. Following IVIG (Intravenous Immunoglobulin) treatment, the patient exhibited clinical improvement (Hughes score decreased from 4 to 2). Discussion. The patient exhibits clinical manifestations of flaccid tetraparesis and multiple cranial nerve paresis. The clinical condition of the patient did not meet the criteria for GBS clinical variation. Symptoms of flaccid tetraparesis that do not involve the senses are classified as the motor-only variant. This patient’s differential diagnosis includes the possibility of MFS and ophthalmoplegia. This patient, however, had flaccid tetraparesis and no ataxia. Electromyoneurography (EMNG) examination produces AMAN-type GBS. The patient was diagnosed with one of the clinical variants of GBS, AMAN-type GBS with multiple cranial neuropathies. Conclusion. A case of GBS of the AMAN type accompanied by multiple cranial neuropathies has been reported.