Systemic lupus erythematosus (SLE) is a heterogeneous chronic autoimmune disease characterized by immune-mediated multiple organ injuries in the setting of autoimmunity to nuclear antigens. In rare cases, it can complicated by the damage of peripheral nervous system, manifesting as Guillain-Barré syndrome (GBS). Severe GBS as the initial presentation is highly infrequent and associated with high disability and mortality rates, highlighting the importance of early detection, diagnosis, and treatment. Herein, we reported a successfully treated case of severe SLE-GBS in a 38-year-old male. Furthermore, we summarize the clinical characteristics of severe SLE-GBS reported thus far and propose the possibility of using medium-to-high dose corticosteroids in the acute progression stage of SLE-GBS. This report provides valuable insights for the analysis of disease characteristics and guidance for diagnosis and treatment of such cases.

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-Barré syndrome (GBS) is an autoimmune-mediated disorder characterized by demyelinating or axonal injury of the peripheral nerve. Our aim is to determine whether serum amyloid A (SAA) is a biomarker of demyelinating injury and disease severity in patients with GBS.

This study retrospectively enrolled 40 patients with either the demyelinating or axonal GBS and sex- and age-matched controls with other neurological diseases as well as healthy subjects. The demographic and clinical features at entry were collected. The serum levels of the SAA isoforms SAA1, SAA2, and SAA4 were determined in the patients with GBS and the controls using the enzyme-linked immunosorbent assay and analyzed for the associations between levels of different SAA isoforms and the clinical features of the patients.

The levels of SAA2 and SAA4 were significantly higher in patients with GBS than in both the other neurological disease controls and the healthy subjects (p<0.05 for all). The level of SAA1 did not differ between patients with GBS and the controls. The level of SAA2 was considerably higher in GBS patients with antecedent infection than in those without infection (p=0.020). The levels of different SAA isoforms were not associated with the disease severity or other clinical features of patients with GBS (p>0.05 for all).

Increased levels of SAA2 and SAA4 may only represent the acute inflammatory status and so cannot be utilized as biomarkers of the disease severity or demyelinating injury in patients with GBS.

Guillain-Barré syndrome (GBS) is an inflammatory disorder causing severe disability and death. The sympathetic skin response (SSR) and RR interval variability are non-invasive tests that measure autonomic dysfunction. The aim of this study was to investigate the relationship of SSR and RR interval variation with prognosis in patients with GBS in Isfahan, Iran.

This cross-sectional study analyzed 32 patients with GBS in Iran between 2023 and 2024. Patients underwent EMG-NCV during their hospitalization, measuring SSR and RR interval variation. Patients' GBS disability scores were calculated and recorded on the day of hospital discharge and one month after. Gender distribution, types of GBS, intubation status, length of ICU and ward stay, GBS disability scores, and plasmapheresis volume are also compared.

In 19 patients (59.3%), both SSR and RR interval variability were abnormal. There were no statistically significant differences between patients with and without autonomic dysfunction (AD) for the variables compared. There were no significant differences between groups with normal and abnormal SSR and between groups with normal and abnormal RR interval variation. Age and intubation status significantly affect GBS disability scores at discharge and one month after discharge, while gender does not significantly impact any outcomes. Many variables, such as age, gender, type of GBS, intubation status, and lengths of stay, do not show significant differences between the compared groups based on AD, SSR, and RR interval variability.

The study highlights the lack of correlation between AD, SSR, and RR interval variation in patients with GBS.

Miller-Fisher Syndrome (MFS), a distinct subtype of Guillain-Barré Syndrome (GBS), accounts for 5% of GBS cases and classically manifests with the triad of ophthalmoplegia, ataxia, and areflexia. Isolated bilateral mydriasis as the sole presenting feature is exceptionally rare, particularly in pediatric populations. While pupillary abnormalities have been documented in adult MFS cases, their diagnostic significance and management in children remain poorly characterized. We report a novel pediatric case of a 7-year-old girl presenting with 7 days of unexplained bilateral painless mydriasis unresponsive to light accommodation. Initial symptomatic management targeting potential toxic or neuropathic etiologies proved ineffective. Recognition of this atypical presentation prompted serological evaluation for autoimmune neuropathy markers, which demonstrated positivity for GQ1b IgM, GQ1b IgG, and GT1a IgG antibodies, confirming MFS diagnosis. Rapid clinical improvement followed intravenous immunoglobulin (IVIG) therapy. This case highlights the diagnostic challenges posed by incomplete or atypical MFS manifestations and underscores the necessity of early antibody testing in unexplained autonomic or neurological symptoms.

This report expands the phenotypic spectrum of pediatric MFS by demonstrating isolated bilateral mydriasis as a potential initial manifestation, clinicians evaluating pupillary dilation should consider MFS in differential diagnoses. Future studies should continue to explore the pathophysiological link between anti-GQ1b antibodies and isolated autonomic dysfunction in pediatric MFS.

Inflammasome plays a significant role in inflammatory responses. The role of inflammasome and its interactions with oxidative stress markers has not been examined in inflammatory neuropathies like Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). This study aims to explore the roles of inflammasome and oxidative stress pathways in inflammatory neuropathies.

This case-controlled study comprised patients with inflammatory neuropathies (n = 60, GBS = 44, CIDP = 16) and age- and gender-matched healthy controls (n = 60). The expressions of inflammasome-related genes (Nlrp3, Casp1, and Il1b) were quantified along with the plasma levels of malondialdehyde (MDA), the end product of lipid peroxidation in all study participants.

The expressions of Nlrp3 (p = 0.0083) and Casp1 (p = 0.0007) genes were significantly up-regulated in GBS patients compared to controls. The plasma MDA levels were also markedly higher in GBS patients than in controls (p = 0.029). The gene expression levels of Nlrp3, Casp1, and Il1b and plasma MDA levels were comparable between CIDP patients and healthy controls. There were no correlations between the expressions of the studied genes and MDA levels with the clinical scores of GBS.

The up-regulated expression of Nlrp3 and Casp1 genes and increased levels of MDA suggest the presence of an activated oxi-inflammatory pathway in GBS. These findings provide a new dimension to the current understanding of the immuno-pathogenesis of GBS.

We report an atypical clinical presentation of a rapidly progressive neurologic emergency that required prompt investigation and treatment of impending respiratory failure. We discuss the differential diagnosis, evaluation, emergency management, and treatment options of patients with atypical variants of this disorder.

A 56-year-old woman with a history of hypothyroidism, anxiety, and depression presented to the emergency department 3 weeks after an upper respiratory and ear infection with cough, pain with sinus palpation, tingling in her fingers bilaterally and right foot, hives, and an episode of blurry vision on awakening. She was discharged home with antibiotics. That evening, she developed rapidly progressing hoarseness and dysphagia and returned to the emergency department. An initial examination and laryngoscopy revealed complete left vocal cord paralysis, consistent with a left cranial nerve X palsy, which prompted a neurologic evaluation. Her examination progressively worsened over the next day requiring mechanical ventilation and ICU admission.

New-onset bulbar cranial neuropathies should raise concern for neurologic disorders that can be rapidly progressive and result in respiratory failure. Urgent diagnosis and treatment are warranted.

Background/Objectives: Guillain-Barré syndrome (GBS) is a neurological disease that affects the peripheral nerves. The exact cause of this condition is still uncertain, but cross-reactivity between pathogen antigens and nervous tissue may play a crucial role in disease pathogenesis. Roseolovirus humanbeta6 (HHV-6), a neurotropic virus with latency capacity, may be considered a significant candidate for triggering or worsening neurological conditions. In this study, we aimed to investigate the detection of HHV-6 in the CNS from GBS patients. Of the 23 individuals suspected of having GBS, 13 were confirmed as having the disease. We then analyzed the frequency of herpesviruses in the cerebrospinal fluid (CSF) samples from these 13 individuals with GBS who were also tested for enteroviruses and arboviruses and had negative results. Results: After extraction of viral DNA from CSF samples, real-time PCR (qPCR) methodology was used to analyze the frequency and viral load of herpesviruses. Sociodemographic and clinical data were collected for analysis and verification through statistical tests such as Fisher's exact test and the Mann-Whitney test. Thirteen individuals diagnosed with GBS were tested. Among the 13 patients analyzed, 61.5% were men, 38.4% (5/13) tested positive for HHV-6, 61.5% of the patients tested positive for a herpesvirus, 30.8% had two viral DNAs identified, and one patient presented three different strains. Patients who tested positive for HHV-6 had a significantly longer average length of stay (25.6 days versus 11 days for negative patients). HHV-6 was the most frequent subtype detected in patients positive for herpesviruses (62.5%, 5/8). Discussion/Conclusions: Our results show a possible relationship between HHV-6 and GBS cases despite the small number of patients, raising the question of whether the presence of HHV-6 influences GBS, since its investigation using qPCR is not routinely used. This may have some impact on prognosis, since antiviral therapy is not included in the standard treatment of GBS patients, and viral DNA load may interfere with the inflammatory process of GBS.

Therapeutic plasma exchange has been a well-known treatment method for many years and is widely available. It leads to the improvement of neurological symptoms in autoimmune neurological diseases by the removal of antibodies. The aim of this study was to present therapeutic plasma exchange responses and procedure-related adverse events in patients with autoimmune neurological diseases based on our 11-year experience.

A retrospective evaluation was conducted on adult patients who underwent a therapeutic plasma exchange procedure due to neurological diseases between January 2013 and January 2024. Data were gathered from electronic and written hospital and apheresis unit records.

A total of 265 patients underwent 1274 procedures with a preliminary diagnosis of autoimmune neurological disease. Five patients were excluded from the analysis due to their final diagnoses. The most common clinical indications were Guillain-Barré syndrome (45.4%), myasthenia gravis (26.1%), and multiple sclerosis (19.2%). The overall response rate was 81.3%, with 21.7% exhibiting a complete response and 59.6% demonstrating a partial response. With the exception of one patient (hypertensive crisis), no complications necessitating the termination of the procedure were observed. The most prevalent complication was an easily manageable allergic reaction.

Therapeutic plasma exchange has been demonstrated to be an efficacious and safe treatment option in autoimmune neurological diseases, with a favorable overall response rate and a manageable mild-to-moderate side effect profile.

Obesity is a worldwide health concern frequently addressed by weight reduction strategies, including bariatric surgery and restricted diets. While effective, these approaches can result in complications, including Guillain-Barré Syndrome (GBS), a rare but serious autoimmune disorder. This study aims to analyze clinical and neurophysiological features of diet-induced GBS and compare them to cases linked with bariatric surgery.

We retrospectively reviewed medical records of five patients admitted to our institution between August 2012 and August 2022, who developed GBS during active dieting resulting in significant weight loss. Clinical presentations, laboratory results, neurophysiological findings, and nutritional status during treatment were analyzed. Additionally, we performed a literature review comparing these cases with nineteen previously reported instances of bariatric surgery-associated GBS.

All five patients exhibited acute, symmetrical limb weakness primarily affecting the lower extremities, accompanied by diminished tendon reflexes. Neurophysiological assessments revealed axonal damage in all cases, and albuminocytologic dissociation was present in two patients. Three patients received intravenous immunoglobulin (IVIG) therapy, while the remaining two underwent nutritional therapy alone. All patients achieved full recovery within 6 months. Notably, the rate of weight loss observed significantly exceeded recommended safe guidelines.

Rapid and substantial weight loss may play a role in triggering GBS, possibly due to nutritional deficiencies or immune dysregulation. Clinicians should recognize the potential neurological risks associated with aggressive weight-loss strategies. Early diagnosis and appropriate intervention are crucial for favorable outcomes and preventing complications.

A unique case report on campylobacter rectus infection leading to acute motor axonal neuropathy in a pediatric patient. Campylobacter rectus is an anaerobic bacterium found in the oral cavity. While it has been linked to periodontal disease, its association with acute motor axonal neuropathy (AMAN), a variant of Guillain-Barre Syndrome, remains unverified. The majority of Guillain-Barre Syndrome (GBS) cases related to Campylobacter are attributed to the species Campylobacter jejuni. A 14-year-old male was admitted to the hospital with sepsis. About 1 week before his admission, the patient sustained an injury to his left eye from a cell phone, resulting in considerable edema. A punch biopsy of the left periorbital soft tissue yielded a culture positive for Campylobacter rectus. Following this, the patient developed weakness in all 4 limbs, with more pronounced weakness in the legs than in the arms, and he lost motor function in both lower legs, although sensory function remained intact. Analysis of cerebrospinal fluid (CSF) revealed albuminocytologic dissociation. An MRI of the spine showed anterior nerve root predominant enhancement.Electromyography was consistent with acute motor axonal neuropathy. Acute motor axonal neuropathy (AMAN), a subtype of Guillain-Barré Syndrome, arises from an atypical immune response, frequently initiated by infections caused by Campylobacter jejuni, although other infectious agents have also been identified. To the best of our knowledge, this is the first recorded instance associating Campylobacter rectus infection with AMAN.

Guillain-Barre syndrome (GBS) is a heterogeneous autoimmune disorder characterized by peripheral neuropathy, often triggered by preceding infections or vaccinations. It encompasses several clinical variants, including the rare Miller Fisher Syndrome (MFS), distinguished by ophthalmoplegia, ataxia, and areflexia. Diagnosis is challenging due to varied presentations and potential overlap with other neurological conditions. We present a case of a 42-year-old male initially suspected to have a stroke when he presented with unilateral loss of sensation and dysarthria. He was later diagnosed with MFS after his condition progressed and he developed generalized weakness, ophthalmoplegia, ataxia, and areflexia. Despite initial stability, his condition deteriorated, requiring intensive care. Early recognition and treatment, such as intravenous immunoglobulin (IVIg) and plasmapheresis, are critical for improving outcomes in GBS and its variants. This case underscores the importance of clinical suspicion and appropriate diagnostic strategies in managing these complex neurological disorders.

Neuromuscular emergencies can be anxiety provoking for neurologist and nonneurologist alike. This review will discuss common causes of neuromuscular respiratory failure and how to treat them. An algorithmic approach to neuromuscular neurology will be employed to help the nonneurologist be more comfortable with acute neuromuscular emergencies.

Electrophysiological investigations in early Guillain-Barré Syndrome (GBS) can be nondiagnostic. Improved testing for facial weakness in the early phase of GBS may improve diagnostic processes, as such weakness is found in approximately 50% of patients with GBS. This work pilots the utility of high-speed video analysis to complement blink reflex testing in early GBS.

This work prospectively evaluated consecutive patients admitted to a metropolitan teaching hospital in Melbourne, Australia, with suspected acute GBS within the first 14 days of neurological symptoms and compared them to a cohort of healthy controls. Blink reflex testing, mechanically-activated masseter reflexes, and analysis of high-speed video recordings of the evoked blinks were performed at admission (day 0), day 7, and day 21 (±2 days).

19 suspected GBS patients (12 GBS and 7 mimics) were compared to 22 healthy controls. At the first test, 83% of GBS patients and 29% of mimics demonstrated blink reflex abnormalities. 50% of GBS manifested video abnormalities (14% mimics), but abnormalities preceded electrophysiological changes in two GBS patients. The calculated reference values for peak lid velocity and lid excursion by video analysis were ~160 mm/s and 7-8 mm, respectively, with slightly different values for ipsilateral versus contralateral responses.

Combining high-speed video analysis and blink reflex testing improves the detection of facial involvement in early GBS and helps discriminate from mimic disorders. Further work in a larger cohort is required to validate the sensitivity and specificity of this technique.

Guillain-Barré syndrome (GBS) is a serious neurological condition with limited treatment options. A recent report demonstrated successful treatment with efgartigimod alone in two patients with GBS, although it did not significantly shorten the disease duration. This case series investigates the effects of sequential efgartigimod administration in patients with different GBS phenotypes and varying levels of disease severity. All three patients tested positive for immunoglobulin G (IgG) antibodies against serum gangliosides. In Case 1, the patient was treated with 0.4 g/kg of intravenous immunoglobulin (IVIg) for 5 days, showing minimal recovery. After receiving 3 weekly doses of efgartigimod (10 mg/kg), the patient achieved independent ambulation 19 days post-onset, with a reduction in serum ganglioside antibody titers and total IgG levels. Case 2 involved a middle-aged man with Miller Fisher syndrome (MFS)-GBS overlap, who experienced worsened autonomic dysfunction following IVIg treatment. After three doses of efgartigimod, the patient showed symptom improvement within 1 month, alongside a reduction in IgG antibody levels. In Case 3, a 27-year-old male with MFS-GBS overlap, initially unresponsive to IVIg, showed significant improvement in ophthalmoplegia following two doses of efgartigimod, with his serum ganglioside antibodies eventually becoming undetectable. Our findings suggest that sequential efgartigimod treatment may effectively reduce serum anti-ganglioside antibody titers and potentially shorten the disease course in severe GBS and MFS-GBS overlap syndrome. Additionally, it may offer clinical benefits for patients with GBS who have a poor or no response to IVIg, particularly in treating ophthalmoplegia.

We present a rare case of acute immune-mediated polyradiculoneuritis, a Guillain-Barré Syndrome (GBS) variant, manifesting as ophthalmoparesis-ataxia, facial diplegia, and acute bulbar palsy, accompanied by a unique autoimmune profile.

A 75-year-old female developed rapidly progressive symptoms, including bilateral non-reactive mydriasis, ptosis, complete ophthalmoplegia, bilateral facial weakness, tongue immobility, palatal paralysis, limb dysmetria, ataxia, and brisk generalized tendon reflexes, all while maintaining a preserved mental state. Symptoms emerged 10 days after a probable gastrointestinal infection. Severe bulbar dysfunction necessitated orotracheal intubation and a tracheotomy. Extensive cranial nerve involvement initially suggested a brainstem lesion, with oculomotor and acute bulbar palsy as prominent signs. However, brainstem and spinal magnetic resonance imaging along with cerebrospinal fluid analysis yielded negative results. Electromyography reveled a sensorimotor demyelinating polyradiculoneuropathy, and serum testing identified IgG antibodies targeting multiple gangliosides, including the disialosyl group and terminal NeuNAc(α2-3)Gal. Treatment with intravenous immunoglobulin (IVIG) led to gradual clinical improvement.

This case highlights a rare and severe GBS phenotype characterized by reactivity to multiple gangliosides. It highlights the role of shared ganglioside epitopes in antibody-mediated neurological damage and expands the clinical spectrum of GBS variants.

A 56-year-old woman who presented with left drop foot and low back pain a week after the onset of diarrhea. Neurological symptoms progressed for a week and gradually improved thereafter. No weakness was observed in upper limbs and clearly asymmetrical muscle weakness was observed in left lower limbs during the course of the disease. Nerve conduction study demonstrated absent motor responses in the left tibial and fibular nerves, and compound muscle action potentials in the right tibial nerve was decreased in amplitude without conduction slowing. Serum IgG anti-GalNAc-GD1a antibody and anti-ganglioside complex antibodies were positive. Based on these findings, we diagnosed her as a rare variant of Guillain-Barré syndrome (GBS) with marked asymmetrical muscle weakness. In the literature, GBS patients with asymmetrical muscle weakness often have anti-ganglioside antibodies associated with acute motor axonal neuropathy. A detailed history taking and information on the clinical course are helpful for accurate diagnosis of GBS with atypical distribution of weakness.

Neurological manifestations associated with COVID-19 remain partially described, mainly in low- and middle-income countries where diagnostic tools are limited. To address this, we assembled medical centers in Brazil with the goal of describing neurological syndromes associated with COVID-19 during the first wave of the pandemic.

From June 1st, 2020 to June 1st, 2021, non-consecutive adult patients with new onset of six neurological syndromes up to 60 days after confirmed COVID-19 were included. Data were compiled from four tertiary centers and compared with general local COVID-19 data, as well as with a previous cohort focused on vascular syndrome.

197 patients were included, presenting with vascular syndromes (81), encephalopathy (68), encephalitis (19), Guillain-Barré syndrome (13), other neuropathies (12), and myelitis (4). The incidence curve of neurocovid mirrored that of COVID-19. Neurological syndromes were present regardless of COVID-19 severity. The median time from COVID-19 to onset of neurological symptoms was 14 days, suggesting a post-infectious immune-mediated mechanism. Patients were 10 times more likely to die (χ2 (1) = 356.55, p < 0.01, OR = 10.89) and 38 times more likely to be hospitalized than other COVID-19 patients (χ2 (1) = 1167.9, p < 0.01, OR = 38.22). Those developing vascular syndromes patients were 3 times more likely to require ICU (χ2 (1) = 37.12, p < 0.01, OR = 3.78) and 4 times more likely to die (χ2 (1) = 58.808, p < 0.01, OR = 4.73) than patients with vascular syndromes due to different etiologies.

Our study corroborates the association of neurological syndromes with COVID-19. The incidence correlated with local waves of COVID-19, and patients with neurocovid exhibited a higher susceptibility to adverse outcomes compared to other COVID-19 patients. Among all neurological syndromes, vascular syndromes were the most common, and their severity surpassed that of vascular syndromes not attributed to COVID-19.

Malaria is an infectious disease caused by Plasmodium parasites, transmitted to humans by infected female Anopheles mosquitoes. Five Plasmodium species infect humans: P. vivax, P. falciparum, P. ovale, P. malariae, and P. knowlesi. Guillain-Barré Syndrome (GBS) is an inflammatory condition that can lead to paralysis, autonomic dysfunction, respiratory failure, and sensory symptoms. GBS typically follows an infection with Campylobacter bacteria, commonly found in undercooked poultry, but is rarely associated with malaria.

A 16-year-old female patient presented to our emergency department with a 1-day history of altered mentation. She had experienced a severe global headache and fever for 3 days prior to presentation. The patient tested positive for falciparum malaria and was admitted to the ward, where she received IV artesunate and other supportive management. After 3 days of admission, she noticed weakness and numbness in her lower extremities. Subsequently, the weakness progressed upward to involve her upper extremities. After extensive workup, the patient was managed with consideration of Guillain-Barré Syndrome (GBS), and she made a complete recovery after 12 weeks.

Guillain-Barré Syndrome (GBS) is an acute paralytic illness often triggered by infections, particularly viral ones. It is the leading cause of sudden muscle weakness, typically following respiratory or gastrointestinal infections, with Campylobacter jejuni being the most common cause. This patient's neurological symptoms pointed to paralysis of the lower motor neurons. Guillain-Barré Syndrome is also suggested by elevated protein levels and a lack of cells in the cerebrospinal fluid. This clinical picture emerged following a Plasmodium falciparum infection. Although the specific subtype (demyelinating or axonal) was not determined in this case due to the absence of a nerve conduction study, demyelinating subtypes have been found in GBS following Plasmodium infection.

In conclusion, while malaria is an exceptionally rare cause of Guillain-Barré Syndrome (GBS), it should be considered in patients with recent malaria infection who present with symptoms of lower motor neuron lesions.

Obesity management through laparoscopic sleeve gastrectomy can occasionally lead to rare but severe complications, including Guillain-Barré Syndrome (GBS). This report analyzes two cases of GBS following LSG, highlighting differences in symptoms, diagnosis, and management. The first patient experienced rapid onset neurological symptoms post-surgery, confirmed as GBS through clinical and electrophysiological assessments, and responded well to intravenous immunoglobulin. The second patient showed similar symptoms but received only multivitamins and physiotherapy due to financial constraints, with a slow but progressive recovery. These cases underscore the necessity for vigilant postoperative monitoring for GBS, reflecting on potential immune dysregulation and microbiota changes as contributory factors. This awareness is crucial for improving patient outcomes and understanding the neurological impacts of bariatric surgery.

The purpose was to explore the prognostic utility of neurofilament light chain (NfL) in patients with immune-mediated polyradiculoneuropathies (IMPs).

This retrospective monocentric study analysed serum and cerebrospinal fluid samples from patients diagnosed with IMP collected prior to treatment initiation. NfL concentrations were correlated with clinical outcomes, including F score and hospitalization duration.

Amongst 115 IMP patients tested, baseline cerebrospinal fluid and serum NfL (sNfL) concentrations were higher in acute inflammatory axonal polyradiculoneuropathy (AIAP) than other IMP variants. In the AIAP cohort, a positive correlation was observed between baseline sNfL concentrations, F score and hospitalization duration. Multivariate linear regression analysis further supported the predictive relationship between elevated baseline sNfL concentrations and clinical outcomes. Using receiver operating characteristic analysis, a cut-off value for sNfL of 351 pg/mL was found to predict an F score >3 in AIAP with a sensitivity of 40% and specificity of 81.8%. AIAP patients with sNfL concentrations above this threshold required longer hospitalization (extended by 15 days).

Our findings highlight the potential of baseline sNfL as an effective marker for distinguishing between IMP variants and predicting the prognosis of AIAP. Further validation may facilitate translation of sNfL into clinical practice, potentially identifying high-risk patients for tailored treatment approaches.

Guillain-Barré syndrome (GBS) is characterized classically by progressive and symmetrical motor weakness and areflexia. We describe a case of GBS with initially preserved reflexes and power, leading to delayed diagnosis, who latterly required urgent ventilator support and plasmapharesis to highlight the importance of considering atypical presentations of this common condition.

Gullain-Barré syndrome (GBS) is a rare autoimmune condition primarily presenting with symmetrical progressive limb weakness. It is frequently associated with sensory and autonomic symptoms and autonomic disturbances and often manifests seropositivity for anti-ganglioside antibodies. Infections are considered major precipitants; however, GBS post-craniotomy for severe traumatic brain injury is a rarity.

A 79-year-old female underwent craniotomy for a cerebellar contusion sustained from severe traumatic brain injury, leading to quadriplegia, autonomic dysfunction, dilated pupils, and respiratory failure. However, the patient's GBS manifested slightly differently. Her limb weakness was asymmetric and progressed from 1 upper limb to the other.

The diagnosis of GBS was confirmed based on clinical presentation, cerebrospinal fluid analysis showing albuminocytologic dissociation, and the detection of anti-sulfatide antibodies in serum.

The patient received intravenous immunoglobulin (IVIG) therapy at 2 g/kg daily, along with supportive measures including mechanical ventilation and rehabilitation.

The patient demonstrated significant improvement within 5 days of IVIG treatment, achieving near-complete functional recovery with grade 4 muscle strength at discharge 6 weeks post-intervention.

This case highlights the need to consider GBS in postoperative patients with acute limb weakness, even in atypical presentations. Early recognition and timely IVIG treatment are critical for favorable outcomes.

Guillain-Barré syndrome (GBS) is a rare immune-mediated polyradiculoneuropathy. Patients typically develop rapidly progressive weakness and sensory deficits that can result in complete paralysis requiring mechanical ventilation. GBS is usually a monophasic disease in which an aberrant immune response to an infection or other trigger damages the peripheral nerves. For example, in patients with preceding Campylobacter jejuni infection, molecular mimicry causes a cross-reactive antibody response to nerve gangliosides. Diagnosis is based on clinical features, supported by cerebrospinal fluid analysis and nerve conduction studies. Effective treatments include plasma exchange and intravenous immunoglobulins. However, ~20% of patients who received treatment are unable to walk after 6 months and ~5% die as a consequence of GBS. Important knowledge gaps in GBS include its pathogenesis, especially after viral infections. In addition, there is a lack of specific biomarkers to improve the diagnosis, monitor the disease activity, and predict the clinical course and outcome of GBS. Major challenges for the future include finding more effective and personalized treatments, which are affordable in low-income and middle-income countries, and preparation for outbreaks of infections as potential triggers for GBS.

Pain is a serious manifestation in both the acute and chronic stages of Guillain-Barre syndrome (GBS). We evaluated the frequency, characteristics and associated factors of pain and its impact on quality of life (QoL) among patients with GBS.

We enrolled 644 patients with GBS from prospective cohort studies in Bangladesh conducted between 2010 and 2024. Data were collected at enrolment and at standard follow-up time points up to 26 weeks. Pain intensity was measured by a pain numeric rating scale. Group differences were tested using the χ² or Fisher's exact test, longitudinal changes were analysed with repeated-measures analysis of variance and correlations were analysed with Spearman's rank test.

The median age of the patients was 31 years, with 70% men. During enrolment, 71% of patients reported pain, which persisted among 38% at week 13 and 26% at week 26. Pain was significantly associated with disease severity, muscle weakness and treatment with intravenous immunoglobulin in both the acute and chronic stages. Patients with acute pain had a higher proportion of axonal GBS (p=0.000) than those without pain. Chronic pain was associated with higher age (p=0.006), male sex (p=0.000), preceding diarrhoea (p=0.033) and dysautonomia (p=0.000). Higher pain intensity was reported among women (p=0.027), patients with higher age (p=0.029) and severe form of GBS (p=0.038) compared with counter groups. Acute pain was significantly associated with the 'self-care' (p=0.023), 'usual activities' (p=0.049) and 'anxiety/depression' (p=0.048) domains of QoL, whereas chronic pain was associated with the 'anxiety/depression' (p=0.005) domain.

Pain presented as a serious symptom negatively affecting the QoL in GBS. Systematic evaluation of pain is recommended to ensure a personalised treatment approach for GBS.

The COVID-19 pandemic, caused by Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2), has resulted in devastating medical and economic consequences worldwide over the past 3 years. As the pandemic enters a new stage, it is essential to consider the potential impact on rare diseases such as Guillain-Barre syndrome (GBS), which has been intimately associated with COVID-19 since the first COVID-19-related GBS case was reported in January 2020. There are notable differences between COVID-19-related GBS and GBS without COVID-19 in terms of diagnostic types and clinical manifestations. Furthermore, with the widespread administration of COVID-19 vaccines, there have been reports of GBS occurring shortly after vaccination, which requires close attention despite its rarity. This review also explores the vaccines associated with heightened GBS risks, offering insights that may guide vaccination policies and clinical practice. To provide a visual summary of these findings, we have included a graphical abstract.

Previously, we reported a cohort of Japanese encephalitis (JE) patients with Guillain-Barré syndrome. However, the evidence linking Japanese encephalitis virus (JEV) infection and peripheral nerve injury (PNI) remains limited, especially the epidemiology, clinical presentation, diagnosis, treatment, and outcome significantly differ from traditional JE. We performed a retrospective and multicenter study of 1626 patients with JE recorded in the surveillance system of the Chinese Center for Disease Control and Prevention, spanning the years 2016-2020. Cases were classified into type 1 and type 2 JE based on whether the JE was combined with PNI or not. A comparative analysis was conducted on demographic characteristics, clinical manifestations, imaging findings, electromyography data, laboratory results, and treatment outcomes. Among 1626 laboratory confirmed JE patients, 230 (14%) were type 2 mainly located along the Yellow River in northwest China. In addition to fever, headache, and disturbance of consciousness, type 2 patients experienced acute flaccid paralysis of the limbs, as well as severe respiratory muscle paralysis. These patients presented a greater mean length of stay in hospital (children, 22 years [range, 1-34]; adults, 25 years [range, 0-183]) and intensive care unit (children, 16 years [range, 1-30]; adults, 17 years [range, 0-102]). The mortality rate was higher in type 2 patients (36/230 [16%]) compared to type 1 (67/1396 [5%]). The clinical classification of the diagnosis of JE may play a crucial role in developing a rational treatment strategy, thereby mitigating the severity of the disease and potentially reducing disability and mortality rates among patients.

Major clusters of Guillain-Barré Syndrome (GBS) emerged during the Zika virus (ZIKV) outbreaks in the South Pacific and the Americas from 2014 to 2016. The factors contributing to GBS susceptibility in ZIKV infection remain unclear, although considerations of viral variation, patient susceptibility, environmental influences, and other potential factors have been hypothesized. Studying the role of viral genetic factors has been challenging due to the low viral load and rapid viral clearance from the blood after the onset of Zika symptoms. The prolonged excretion of ZIKV in urine by the time of GBS onset, when the virus is no longer present in the blood, provides an opportunity to unravel whether specific ZIKV mutations are related to the development of GBS in certain individuals. This study aimed to investigate the association between specific ZIKV genotypes and the development of GBS, taking advantage of a unique collection of ZIKV-positive urine samples obtained from GBS cases and controls during the 2016 ZIKV outbreak in Colombia. Utilizing Oxford-Nanopore technology, we conducted complete genome sequencing of ZIKV in biological samples from 15 patients with GBS associated with ZIKV and 17 with ZIKV infection without neurological complications. ZIKV genotypes in Colombia exhibited distribution across three clades (average bootstrap of 90.9±14.9%), with two clades dominating the landscape. A comparative analysis of ZIKV genomes from GBS and non-neurological complications, alongside 1368 previously reported genomes, revealed no significant distinctions between the two groups. Both genotypes were similarly distributed among observed clades in Colombia. Furthermore, no variations were identified in the amino acid composition of the viral genome between the two groups. Our findings suggest that GBS in ZIKV infection is perhaps associated with patient susceptibility and/or other para- or post-infectious immune-mediated mechanisms rather than with specific ZIKV genome variations.

We present a case of a 37-year-old gentleman diagnosed with post-infectious Guillain-Barré syndrome (GBS) secondary to a Mycoplasma pneumoniae infection. This case highlights the subclinical presentation of neurological symptoms, often overlooked as a complication of M. pneumoniae infection. The patient exhibited significant neurological deficits following initial respiratory symptoms, demonstrating the need for awareness of these potential extrapulmonary complications in clinical practice.

Guillain-Barré syndrome represents a heterogeneous group of immune-mediated peripheral neuropathies that are characterized by various clinical manifestations. Reporting this clinical case emphasizes the rarity of Guillain-Barré syndrome, the diagnostic challenges faced by healthcare providers, and the risk of delayed diagnosis for both the mother and fetus. A 34-year-old pregnant woman at 33 weeks of gestation presented to the inpatient ward complaining of paresthesia in the lower and upper limbs, muscle pain, balance disturbances, moderate headache, nausea and vertigo, general weakness, and pronounced fatigue. The patient had experienced an acute viral respiratory infection 4 weeks before presenting to the hospital. The patient was admitted to the intensive care unit with a preliminary diagnosis of acute viral respiratory infection and nasopharyngitis. The patient's condition worsened dynamically, manifesting bulbar syndrome (swallowing problems), paresthesia of the anterior abdominal wall, reduced perception of fetal movements, numbness of the tongue, and low fever (37.2°C). A diagnosis of acute inflammatory demyelinating polyradiculopathy (Guillain-Barré syndrome) was established. Despite treatment, the neurologic symptoms worsened. The paravertebral radicular type pains were difficult to manage with administered analgesic therapy, and there was a progression of the bulbar syndrome. Treatment with intravenous immunoglobulin was initiated. Consequently, it was recommended by the multidisciplinary council to perform an emergency cesarean delivery, in the interest of the mother and fetus. Guillain-Barré syndrome is a rare condition that occurs during pregnancy and requires thorough evaluation, prompt multidisciplinary assessment, and individualized management of delivery to improve maternal and fetal prognosis.

To validate the International Classification of Diseases, 10th Revision (ICD-10) codes for Guillain-Barré syndrome (GBS) in Taiwan's insurance claims database.

We identified adult patients hospitalized at any Chang Gung Memorial Foundation branch hospital between January 1st, 2017, and December 31st, 2022, with ICD-10 code G61.0 in any of the five discharge diagnosis positions, indicating possible Guillain-Barré syndrome. We then validated the possible GBS diagnosis using data from electronic medical records of the identified patients, based on the diagnostic criteria established by the National Institute of Neurological Disorders and Stroke. We determined the positive predictive values (PPV) of various operational definitions, including the position (primary or other) where the code was recorded in the discharge diagnosis, nerve conduction study (NCS) claims, and / or specific GBS treatments.

The final validation cohort of 484 patients with ICD-10 code for GBS in the discharge diagnosis was found to include 368 true GBS patients. Identifying inpatients using only the ICD-10 code for GBS in any of the five positions for discharge diagnosis yielded a PPV of 76.0%. With more restrictive definitions (primary diagnosis only, or requiring additional claims for NCS and/or treatments), the PPV tended to increase, but with fewer true GBS patients identified. Using ICD-10 GBS code in the primary diagnosis plus NCS and treatment claims yielded the highest PPV (98.3%); however, 140 (38.0%) of the true GBS patients were missed using this definition. In contrast, using the ICD-10 GBS code in any position, plus claims for NCS, achieved a relatively good PPV (85.8%) with minimal loss of true GBS patients (13, ie, 3.5%).

In Taiwan's NHI claims data, identifying true GBS patients using only the ICD-10 code yielded a PPV of 76.0%; however, adding claims for diagnostic procedure and GBS treatment increased the PPV to 98.3%.

The virus SARS-CoV-2 (Severe Acute Respiratory Syndrome Coronavirus 2) causes COVID-19, a potentially fatal disease. The COVID-19 vaccine is indicated for active immunization to prevent COVID-19 caused by SARS-CoV-2. We reported the case of a 66-year-old woman with a medical history of hypertension and anxious-depressive syndrome who developed Guillain Barré Syndrome (GBS) 4 weeks after receiving the COVID-19 vaccine. During the patient's hospital stay, they received cycles of high-dose intravenous immunoglobulin (IVIG) and plasmapheresis treatments.. Despite the treatment, a deterioration of respiratory function led the patient to premature mortality.

There is no diagnostic biomarker that can reliably detect Guillain-Barré syndrome (GBS) or chronic inflammatory demyelinating polyneuropathy (CIDP). Diagnosis relies upon integrating key clinical characteristics and relevant supportive data. Consequently, misdiagnosis and delayed diagnosis are common. Diagnostic criteria have proven valuable resources to improve diagnosis, but are underutilized during routine clinical care.

In 2021, the EAN/PNS CIDP criteria was published, and were followed by the EAN/PNS GBS criteria in 2023. Both guidelines utilized GRADE methodology to formulate evidence-based recommendations that are intended to be used by adult and paediatric clinicians across diverse care settings to optimize diagnostic accuracy and improve patient outcomes during routine clinical care.

The EAN/PNS GBS and CIDP criteria detail specific clinical, electrophysiological, and laboratory features that raise diagnostic confidence, and call attention to diagnostic mimics. The sensitivity of EAN/PNS and other modern criteria to detect GBS and CIDP is high, but utilization during clinical practice is low. Complexity is one factor limiting widespread application. Strategies are needed to optimize criteria adoption during routine clinical care such that GBS and CIDP diagnosis can be achieved with greater speed and accuracy.

Guillain-Barré syndrome (GBS) is an acute, self-limited, immune-mediated peripheral neuropathy. Current treatments for GBS include intravenous immunoglobulin (IVIg) and plasma exchange, which may not sufficiently benefit severely affected patients. This study evaluated the efficacy and safety of eculizumab add-on therapy to IVIg (standard-of-care treatment) in patients with severe GBS.

This phase 3, multicenter, double-blind, randomized, placebo-controlled clinical trial (NCT04752566), enrolled Japanese adults (age ≥ 18 years) with severe GBS (Hughes functional grade [FG] score FG3 or FG4/FG5 within 2 weeks of onset of GBS). Participants were randomized 2:1 to receive intravenous infusion of eculizumab or placebo (once weekly for 4 weeks) with IVIg treatment with 20 weeks of follow-up. Primary efficacy endpoint was the time to first reach FG score ≤1 (able to run). Key secondary endpoints were proportion of participants achieving FG ≤1 at weeks 8 and 24 and FG improvement ≥3 at week 24. Pharmacodynamic analysis of serum free C5 concentration over time was performed. Safety was evaluated.

The analysis included 57 participants (eculizumab, n = 37; placebo, n = 20). Primary endpoint was not achieved (hazard ratio, 0.9; 95% CI, 0.45-1.97; p = .89). Key secondary endpoints did not reach statistical significance. Serum C5 concentration was reduced by 99.99% at 1 h postdose and sustained to week 5 but returned to baseline at the end of follow-up period. No new safety signals for eculizumab were identified.

Although well tolerated, eculizumab treatment did not show significant effects on motor function recovery compared to placebo in patients with GBS.

Guillain-Barré Syndrome (GBS) can lead to significant functional impairments, yet little is understood about the recovery phase and long-term consequences for patients in low- and medium-income countries.

To evaluate the functional status and identify factors influencing outcomes among patients with GBS in Colombia.

Telephone interviews were conducted with GBS patients enrolled in the Neuroviruses Emerging in the Americas Study between 2016 and 2020. The investigation encompassed access to health services and functional status assessments, utilizing the modified Rankin Scale (mRS), GBS Disability Score (GDS), Barthel Index (BI), and International Classification of Functioning (ICF). Univariate analysis, principal component analysis, linear discriminant analysis, and linear regression were employed to explore factors influencing functional status.

Forty-five patients (mean age = 50[±22] years) with a median time from diagnosis of 28 months (IQR = 9-34) were included. Notably, 22% and 16% of patients did not receive rehabilitation services during the acute episode and post-discharge, respectively. Most patients demonstrated independence in basic daily activities (median BI = 100, IQR = 77.5-100), improvement in disability as the median mRS at follow-up was lower than at onset (1 [IQR = 0-3] vs. 4.5 [IQR = 4-5], p < 0.001), and most were able to walk without assistance (median GDS = 2, IQR = 0-2). A shorter period from disease onset to interview was associated with worse mRS (p = 0.015) and ICF (p = 0.019). Negative outcomes on GDS and ICF were linked to low socioeconomic status, ICF to the severity of weakness at onset, and BI to an older age.

This study underscores that the functional recovery of GBS patients in Colombia is influenced not only by the natural course of the disease but also by socioeconomic factors, emphasizing the crucial role of social determinants of health.