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Background and Aims: 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., Methods: 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., Results: 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., Interpretation: Although well tolerated, eculizumab treatment did not show significant effects on motor function recovery compared to placebo in patients with GBS., (© 2024 The Author(s). Journal of the Peripheral Nervous System published by Wiley Periodicals LLC on behalf of Peripheral Nerve Society.)

Introduction: Small cross-sectional studies and case reports observed improvement after administration of second IVIG dose (SID) amongst Guillain-Barré Syndrome (GBS) patients not responsive to initial IVIG cycle. Nevertheless, recent clinical trial and larger observational studies did not find any positive effects of SID. Instead, an increased risk of thromboembolism and mortality was noted. The conclusions of these studies however were not robust as confounding and selection bias were present., Methodology: Two neurologists conducted the search process (KBA and MBP) using the following terms in Medline: [(" Guillain-Barré Syndrome"[MeSH Terms] or GBS or Acute Motor Axonal Neuropathy or Acute Motor Axonal Neuropathy or Acute Inflammatory Demyelinating Polyneuropathy) AND (Poorly Responsive or Poor Prognosis or Progressive)] AND [("Intravenous Immunoglobulin"[MeSH Terms] or IVIG or IGIV) AND (second dose or retreatment or SID)]., Results: Only 7 articles were included in this review. In terms of primary outcomes, although the cross-sectional study found improvement in GBS DS score at 4 weeks (Median GBS DS: 3 vs 5, p = 0.033) and the 2 case series observed improvement after SID, no significant differences between the control and intervention groups were found in the cohort [Early SIV OR: 0.7 (95% CI 0.16-3.04), Late SIV OR: 0.66 (CI: 0.18-2.5)] and clinical trial studies (Adjusted OR: 1.4 (95% CI:0.6-3.3, p = 0.45). Moreover, 4 patients who died in the clinical trial were from the intervention group., Conclusion: Based on studies with research designs of higher quality, SID is not effective in the management of GBS patients who poorly responded to initial IVIG. Nevertheless, an adequately powered, randomized, double-blinded, placebo-controlled clinical trial, using GBS-DS of 3 and above after first IVIG dose should be done to effectively establish the efficacy and safety of SID as intervention for this cohort of patients., (© 2024. The Author(s) under exclusive licence to Belgian Neurological Society.)

Background: Guillain‒Barre syndrome (GBS) is an acute inflammatory peripheral neuropathy caused by autoimmunity. Gangliosides and sulfatides are important components of peripheral nerves. Anti-sulfatide antibody-mediated complement is associated with acute sensorimotor peripheral neuropathy in GBS, which is characterized by pain and paresthesias., Case Presentation: The child was a 7-year-old girl with headache and abdominal pain, followed by limb numbness and pain. Cranial imaging showed ventricular dilatation, peripheral nerve function conduction examination showed polyradiculopathy, and cerebrospinal fluid tests showed normal cell counts but elevated protein levels, all of which led to the diagnosis of GBS. After treatment with intravenous immunoglobulin (400 mg/kg × 5 days), the symptoms did not improve, and muscle strength progressively worsened, accompanied by paroxysmal complexion flushing, heart rate fluctuation, hyperhidrosis, and a progressive increase in cerebrospinal fluid protein (up to 3780.1 mg/L). On the basis of these findings combined with serum anti-sulfatide IgM positivity, anti-sulfatide antibody-related GBS was considered, and treatment with low-dose prednisolone (1 mg/kg/d) led to symptom improvement., Conclusions: Anti-sulfatide antibody-associated GBS is associated with small fiber peripheral neuropathy. The main manifestations are pain, paresthesias and autonomic dysfunction. In addition to the dysfunction of spinal nerve root absorption caused by increased cerebrospinal fluid protein, autonomic dysfunction may be involved in pain. When the therapeutic effect of immunoglobulin is not satisfactory, a low dose and short course of corticosteroids can be considered, and the prognosis is good., (© 2024. The Author(s).)

Background: Guillain-Barré Syndrome (GBS) is caused by immunoglobulin G (IgG) autoantibodies. Efgartigimod, a human IgG antibody Fc fragment that acts as a natural ligand for the FcRn, can increase IgG degradation, which thus may be a promising therapeutic drug for GBS., Case Presentation: The two patients presented with postinfectious and acute flaccid paralysis. On admission, they were bedridden. Nerve conduction studies indicated peripheral neuropathy. GBS was suspected and they are treated with two doses of efgartigimod (10 mg/kg) within 5 days. Their muscle strength improved gradually and 4 weeks after the initial dose, they could walk independently. Following the first dose, Patient 1 complaint of muscle soreness, which subsided the next morning. Patient 2 was intubated due to respiratory failure the day after the initial dose, and did not report other adverse effects., Discussion: In GBS patients, two doses of efgartigimod (10 mg/kg) were effective in rapidly improving muscle strength, with a satisfactory safety profile. The findings suggest a potential role for efgartigimod in modifying the disease process in GBS patients., Conclusion: Efgartigimod seems effective and safe in the treatment of GBS. This study indicates the potential role of efgartigimod as a novel treatment option for GBS. Well-designed clinical trials should be conducted., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Background: Both humoral and cell-mediated immunity of the patient affected by multiple myeloma (MM) are impaired; thus, infection is the main cause of the onset of symptoms and death caused by MM. Bortezomib is a first-line drug approved for patients with multiple myeloma (MM) and has significantly increased their overall survival. However, bortezomib-induced peripheral neuropathy (PN) remains a significant side effect that has led to its discontinuation in some patients. Guillain-Barre syndrome (GBS) is thought to be related to immune damage, and most patients have cytomegalovirus (CMV), Epstein-Barr virus (EBV), or mycoplasma infection before onset. Cases of GBS secondary to MM are rare., Methods: We provide a case of GBS caused by cytomegalovirus infection after MM treatment, and briefly review the existing literature., Results: Secondary GBS after MM. This patient received active treatment. The clinical symptoms are gradually improving., Conclusions: The use of bortezomib has the risk of reactivating the virus. It is more about the reactivation of hep-atitis B virus. Nonetheless, cytomegalovirus and Epstein-Barr virus shall have our attention. Patients with MM need to monitor CMV, regularly, especially during the treatment of bortezomib. At the same time, they also need to closely monitor the symptoms and signs of the nervous system to guard against the occurrence of GBS.

A woman in her 40s presented with thoracic banding dysaesthesia and lower motor neuron weakness. Spinal imaging revealed a short segment of transverse myelitis and neurophysiology was suggestive of concurrent acute inflammatory demyelinating polyneuropathy. The patient improved with consecutive intravenous immunoglobulin and methylprednisolone treatment. Acute inflammatory demyelinating polyneuropathy is a progressive immune-mediated peripheral neuropathy which responds to intravenous immunoglobulin or plasmapheresis, whereas transverse myelitis is a central inflammatory syndrome usually treated with corticosteroid. We highlight differentiating features of the clinical presentation and the utility of investigations such as neurophysiology and MRI along with a review of treatment and the role for corticosteroid therapy., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Scrub typhus is an acute infectious disease caused by Orientia tsutsugamushi. Guillain-Barre syndrome (GBS) is an autoimmune-mediated peripheral neuropathy with a frequent history of prodromal infections, but GBS associated with scrub typhus is very rare., Case Presentation: We report a 51-year-old male patient who developed dysarthria and peripheral facial paralysis following the cure of scfrub typhus. CSF examination and electrophysiological findings suggested a diagnosis of GBS. After treatment with intravenous immunoglobulin, the patient's neurological condition improved rapidly., Conclusions: Scrub typhus infection is likely to be a potential predisposing factor in GBS, while scrub typhus-associated GBS has a favorable prognosis., (© 2024. The Author(s).)

Background: Neurofilament light chain (NfL) is a biomarker for axonal damage in several neurological disorders. We studied the longitudinal changes in serum NfL in patients with Guillain-Barré syndrome (GBS) in relation to disease severity, electrophysiological subtype, treatment response, and prognosis., Methods: We included patients with GBS who participated in a double-blind, randomised, placebo-controlled trial that evaluated the effects of a second course of intravenous immunoglobulin (IVIg) on clinical outcomes. Serum NfL levels were measured before initiation of treatment and at one, two, four, and twelve weeks using a Simoa HD-X Analyzer. Serum NfL dynamics were analysed using linear mixed-effects models. Logistic regression was employed to determine the associations of serum NfL with clinical outcome and the prognostic value of serum NfL after correcting for known prognostic markers included in the modified Erasmus GBS Outcome Score (mEGOS)., Findings: NfL levels were tested in serum from 281 patients. Serum NfL dynamics were associated with disease severity and electrophysiological subtype. Strong associations were found between high levels of serum NfL at two weeks and inability to walk unaided at four weeks (OR = 1.74, 95% CI = 1.27-2.45), and high serum NfL levels at four weeks and inability to walk unaided at 26 weeks (OR = 2.79, 95% CI = 1.72-4.90). Baseline serum NfL had the most significant prognostic value for ability to walk, independent of predictors included in the mEGOS. The time to regain ability to walk unaided was significantly longer for patients with highest serum NfL levels at baseline (p = 0.0048) and week 2 (p < 0.0001). No differences in serum NfL were observed between patients that received a second IVIg course vs. IVIg and placebo., Interpretation: Serum NfL levels are associated with disease severity, axonal involvement, and poor outcome in GBS. Serum NfL potentially represents a biomarker to monitor neuronal damage in GBS and an intermediate endpoint to evaluate the effects of treatment., Funding: Prinses Beatrix Spierfonds W.OR19-24., Competing Interests: Declaration of interests Research of CT is supported by the European Commission (Marie Curie International Training Network, grant agreement No 860197 (MIRIADE), Innovative Medicines Initiatives 3TR (Horizon 2020, grant no 831434) EPND (IMI 2 Joint Undertaking (JU), grant No. 101034344) and JPND (bPRIDE), National MS Society (Progressive MS alliance), Alzheimer Drug Discovery Foundation, Alzheimer Association, Health Holland, the Dutch Research Council (ZonMW), The Selfridges Group Foundation, Alzheimer Netherlands. CT is recipient of ABOARD, which is a public-private partnership receiving funding from ZonMW2 (#73305095007) and Health ∼ Holland, Topsector Life Sciences & Health (PPP-allowance; #LSHM20106). CT is recipient of TAP-dementia, a ZonMw funded project (#10510032120003) in the context of the Dutch National Dementia Strategy. CT performed contract research for ADx Neurosciences, AC-Immune, Aribio, Axon Neurosciences, Beckman–Coulter, BioConnect, Bioorchestra, Brainstorm Therapeutics, Celgene, Cognition Therapeutics, EIP Pharma, Eisai, Eli Lilly Fujirebio, Grifols, Instant Nano Biosensors, Merck, Novo Nordisk, Olink, PeopleBio, Quanterix, Roche, Siemens, Toyama, and Vivoryon. CT received payment or honoraria for lectures, presentations or educational events from Roche, Novo Nordisk and Grifols. All payments were made to her institution. CT serves on editorial boards of Medidact Neurologie/Springer; and in Neurology: Neuroimmunology & Neuroinflammation. She is editor of Alzheimer Research and Therapy. RH reports institutional funding from the GBS-CIDP Foundation International, Stichting GBS, T2B collaboration project funded by PPP Allowance made available by Top Sector Life Sciences & Health to Samenwerkende Gezondheidsfondsen (SGF) under project number LSHM18055-SGF to stimulate public-private partnerships and co-financing by health foundations that are part of the SGF, NIH, and PANDIA collaboration project co-funded by BÜHLMANN Laboratories AG and the PPP Allowance made available by Health ∼ Holland, Top Sector Life Sciences & Health, to Prinses Beatrix Spierfonds to stimulate public-private partnerships under project number LSHM23017-SGF. She serves as editorial board member of the Journal of the Peripheral Nervous System and was board member of the Inflammatory Neuropathy Consortium. PD reports grants from the Prinses Beatrix Spierfonds and Sanquin to conduct the SID-GBS trial. PD reports consulting fees from Annexon and Roche, all paid to the institution. PD participates on a Data Safety Monitoring Board or Advisory Board for Argenx, Hansa, Octapharma and Sanofi. All fees go to the institution. He reports an unpaid leadership role in the Peripheral Nerve Society (PNS) and serves on the Medical advisory board for the GBS/CIDP Foundation International. BJ reports institutional funding from the GBS-CIDP Foundation International, Stichting GBS, Horizon 2020 (EU), Grifols, CSL-Behring, Annexon, Hoffmann-la Roche, Hansa Biopharma, BÜHLMANN Laboratories. BJ received institutional royalties, licences or consulting fees from Hansa Biopharma, Annexon and Hoffmann-la Roche. BJ received support from the GBS-CIDP Foundation International to cover travel expenses for their conference. BJ serves an unpaid role in the global Medical advisory board for the GBS-CIDP Foundation International, in the scientific review committee of Stichting MS research, and the scientific review committee of the Erasmus MC. All other authors declare no competing interests., (Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved.)

Background: Guillain-Barré syndrome (GBS), a post-infectious, immune-mediated, acute demyelinating disease of the peripheral nerves and nerve roots, represents the most prevalent and severe acute paralyzing neuropathy. Purinergic P2X7 receptors (P2X7R) play a crucial role in central nervous system inflammation. However, little is known about their role in the immune-inflammatory response within the peripheral nervous system., Methods: Initially, we assessed the expression of purinergic P2X7R in the peripheral blood of patients with GBS using flow cytometry and qRT-PCR. Next, we explored the expression of P2 X7R in CD4 + T cells, CD8 + T cells, and macrophages within the sciatic nerves and spleens of rats using immunofluorescence labeling and flow cytometry. The P2X7R antagonist brilliant blue G (BBG) was employed to examine its therapeutic impact on rats with experimental autoimmune neuritis (EAN) induced by immunization with the P0 180 - 199 peptide. We analyzed CD4 + T cell differentiation in splenic mononuclear cells using flow cytometry, assessed Th17 cell differentiation in the sciatic nerve through immunofluorescence staining, and examined the expression of pro-inflammatory cytokine mRNA using RT-PCR. Additionally, we performed protein blotting to assess the expression of P2X7R and NLRP3-related inflammatory proteins within the sciatic nerve. Lastly, we utilized flow cytometry and immunofluorescence labeling to examine the expression of NLRP3 on CD4 + T cells in rats with EAN., Results: P2X7R expression was elevated not only in the peripheral blood of patients with GBS but also in rats with EAN. In rats with EAN, inhibiting P2X7R with BBG alleviated neurological symptoms, reduced demyelination, decreased inflammatory cell infiltration of the peripheral nerves, and improved nerve conduction. BBG also limited the production of pro-inflammatory molecules, down-regulated the expression of P2X7R and NLRP3, and suppressed the differentiation of Th1 and Th17 cells, thus protecting against EAN. These effects collectively contribute to modifying the inflammatory environment and enhancing outcomes in EAN rats., Conclusions: Suppression of P2X7R relieved EAN manifestation by regulating CD4 + T cell differentiation and NLRP3 inflammasome activation. This finding underscores the potential significance of P2X7R as a target for anti-inflammatory treatments, advancing research and management of GBS., (© 2024. The Author(s).)

Introduction: Guillain-Barré syndrome (GBS) is a group of acute immune-mediated disorders in the peripheral nervous system. Both infectious and noninfectious factors are associated with GBS, which may act as triggers of autoimmune responses leading to neural damage and dysfunction., Areas Covered: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and its vaccines as well as flaviviruses have been associated with GBS, although a robust conclusion has yet to be reached. Immunomodulatory treatments, including intravenous immunoglobulins (IVIg) and plasma exchange (PE), have long been the first-line therapies for GBS. Depending on GBS subtype and severity at initial presentation, the efficacy of IVIg and PE can be variable. Several new therapies showing benefits to experimental animals merit further investigation before translation into clinical practice. We review the state-of-the-art knowledge on the immunopathogenesis of GBS in the context of coronavirus disease 2019 (COVID-19). Immunomodulatory therapies in GBS, including IVIg, PE, corticosteroids, and potential therapies, are summarized., Expert Opinion: The association with SARS-CoV-2 remains uncertain, with geographical differences that are difficult to explain. Evidence and guidelines are lacking for the decision-making of initiating immunomodulatory therapies in mildly affected patients or patients with regional subtypes of GBS.

We present a case of a 24-year-old female recently diagnosed with acute leukemia who came with complaints of fever for 14 days, progressive lower limb weakness, and multiple episodes of vomiting in the last 1 day. In nerve conduction studies, a diagnosis of Guillain-Barré syndrome (GBS) was established. Fever with thrombocytopenia workup revealed a positive dengue nonstructural protein 1 (NS1) and immunoglobulin M (IgM) report. Immunophenotyping confirmed pre-B acute lymphoblastic leukemia (ALL). As leukemia is an immunocompromised state, the peripheral nervous system vulnerability is increased, or infection could precipitate an immune neuropathy. About 10% of adult ALL presents with central nervous system (CNS) leukemias; a higher incidence is seen in mature B ALL. There is some evidence to suggest immunosuppression secondary to intensive chemotherapy (vincristine-induced dying back neuropathy), which was not started in our case. This rare combination in a short period of time with a worsening situation paralyzed the line of management. Few reports described GBS in patients with dengue in adults. The association of Guillan-Barre syndrome and ALL could be coincidental or has a pathophysiological basis and is under basic investigation., (© Journal of the Association of Physicians of India 2024.)

Immune checkpoint molecules play pivotal roles in the regulation of immune homeostasis. Disruption of the immune checkpoints causes autoimmune/inflammatory as well as malignant disorders. Over the past few years, the immune checkpoint molecules with inhibitory function emerged as potential therapeutic targets in oncological conditions. The inhibition of the function of these molecules by using immune checkpoint inhibitors (ICIs) has brought paradigmatic changes in cancer therapy due to their remarkable clinical benefits, not only in improving the quality of life but also in prolonging the survival time of cancer patients. Unfortunately, the ICIs soon turned out to be a "double-edged sword" as the use of ICIs caused multiple immune-related adverse effects (irAEs). The development of inflammatory neuropathies such as Guillain-Barré syndrome (GBS) and Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) as the secondary effects of immunotherapy appeared very challenging as these conditions result in significant and often permanent disability. The underlying mechanism(s) through which ICIs trigger inflammatory neuropathies are currently not known. Compelling evidence suggests autoimmune reaction and/or inflammation as the independent risk mechanism of inflammatory neuropathies. There is a lack of understanding as to whether prior exposure to the risk factors of inflammatory neuropathies, the presence of germline genetic variants in immune function-related genes, genetic variations within immune checkpoint molecules, the existence of autoantibodies, and activated/memory T cells act as determining factors for ICI-induced inflammatory neuropathies. Herein, we highlight the available pieces of evidence, discuss the mechanistic basis, and propose a few testable hypotheses on inflammatory neuropathies as irAEs of immunotherapy., (© 2023 Peripheral Nerve Society.)

The authors describe a patient with a background of metastatic small cell prostate cancer who presented with a rapidly evolving sensorimotor neuropathy with bulbar features closely resembling Guillain-Barré syndrome, with a good initial response to intravenous immunoglobulins and platinum-based chemotherapy. This represented a likely paraneoplastic manifestation of the patient's urological malignancy., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

The efficacy of immunotherapies such as steroids, plasmapheresis, and intravenous immunoglobulin have been proven in various immune-mediated neuropathies. However, these treatments sometimes lack the efficacy in a part of patients with the immune-mediated neuropathies. In addition, anti-myelin associated glycoprotein (MAG) neuropathy is usually refractory to the treatments. Recently, novel therapies targeting a molecule which are associated with pathogenesis of immune-mediated diseases, have been developed. These molecularly targeted therapies are notable in immune-mediated neuropathies as novel drug candidates. In the present article, current treatments and future prospect of novel therapies in immune-mediated neuropathies will be reviewed.

We present the case of a man in his 60s with a 5-month medical history of deceased donor liver transplantation, who developed Guillain-Barré syndrome (GBS) secondary to a primary cytomegalovirus (CMV) infection. This was confirmed by molecular tests and serology antibodies that ruled out other frequent aetiologies. Therapy with intravenous immunoglobulin and valganciclovir was started and the patient gradually improved over the weeks. GBS is the most common aetiology of paralysis worldwide, and it is an autoimmune-mediated neuropathy that is frequently caused by a preceding infection. Few cases of GBS have been reported in the context of liver transplant recipients, and those related to CMV infection are extremely rare. This case highlights the importance of considering GBS as a possible differential diagnosis in patients with solid organ transplantation, and it contributes to the knowledge of other infrequent aetiologies of this condition., Competing Interests: Competing interests: Competing interests: JT is associate editor of NEJM Journal Watch Neurology and is an editorial board member for Multiple Sclerosis and Related Disorders. JG, TM and SR have nothing to disclose., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

About 40% of the Guillain-Barré syndrome (GBS) cases are associated with prodromal infections; occasionally, it has been associated to chronic hepatitis C or its reactivation. A 38-year-old man came to our attention after transaminase elevation occurred during recovery from GBS. All the possible causes of acute hepatitis were excluded except for the positivity of HCVRNA, and a diagnosis of new onset hepatitis C was made. Recalling patient history, we observed that (i) anti-HCV antibodies were negative and liver enzymes were normal 7 weeks before GBS onset; (ii) in the early stages of ICU admission, liver enzymes started to rise, but the elevation remained mild under steroid treatment; (iii) serum aminotransferase peak occurred 11 weeks after GBS onset; and (iv) HCV RNA was already significantly high when anti-HCV antibodies became positive, consistent with an acute hepatitis. Furthermore, anti-HCV seroconversion was likely delayed or blurred by steroids and immunoglobulin infusions. The interval of time between GBS onset and transaminase elevation compared with the patient clinical history allows us to establish a cause-effect relationship between the two diseases. All patients with GBS should be tested for hepatitis C, or its reactivation if already present, and followed up for an early diagnosis and treatment., (© 2023. The Author(s).)

Objective: This article summarizes the clinical features, diagnostic criteria, differential diagnosis, pathogenesis, and prognosis of Guillain-Barré syndrome (GBS), with insights into the current and future diagnostic and therapeutic interventions for this neuromuscular syndrome., Latest Developments: GBS is an acute, inflammatory, immune-mediated polyradiculoneuropathy that encompasses many clinical variants and divergent pathogenic mechanisms that lead to axonal, demyelinating, or mixed findings on electrodiagnostic studies. The type of antecedent infection, the development of pathogenic cross-reactive antibodies via molecular mimicry, and the location of the target gangliosides affect the subtype and severity of the illness. The data from the International GBS Outcome Study have highlighted regional variances, provided new and internationally validated prognosis tools that are beneficial for counseling, and introduced a platform for discussion of GBS-related open questions. New research has been undertaken, including research on novel diagnostic and therapeutic biomarkers, which may lead to new therapies., Essential Points: GBS is among the most frequent life-threatening neuromuscular emergencies in the world. At least 20% of patients with GBS have a poor prognosis and significant residual deficits despite receiving available treatments. Research is ongoing to further understand the pathogenesis of the disorder, find new biomarkers, and develop more effective and specific treatments., (Copyright © 2023 American Academy of Neurology.)

Miller-Fisher syndrome is a rare acquired nerve disease related to Guillain-Barré syndrome. Clinical features include asthenia, ocular muscle weakness with ophthalmoplegia, impaired limb coordination with instability, and absence of tendon reflexes. Swallowing disorders and rarely respiratory failure may be associated. The article aims to summarize, starting from the presentation of a clinical case, the latest updates which, in clinical practice, can be useful for a correct diagnosis and treatment of this condition which concerns both adult and pediatric patients.

Guillain-Barré syndrome is a rare neurological condition. Although some people make a substantial functional recovery, almost half require intensive rehabilitation. Data were collected using a cross-sectional survey which investigated the assessments and interventions used by occupational therapists and physiotherapists for people with Guillain-Barré syndrome. Seventy valid responses were received from 10 countries. The survey highlighted four factors about current practice: (i) practitioners did not identify the use of formal clinical guidelines or protocols for Guillain-Barré Syndrome treatment of the upper limb; (ii) a range of standardized and non-standardized assessment and goal-setting tools are utilized; (iii) interventions include passive and active range of motion exercises, and the prescription of upper limb/hand splints; and (iv) interdisciplinary practice is common in the intensive care unit and during acute phases of Guillain-Barré syndrome, whereas discipline-specific work is more common during rehabilitation. A range of goal-setting and assessment tools are used by occupational therapists and physiotherapists during the hospitalization of people with Guillain-Barré syndrome. The type and duration of interventions vary and may reflect the lack of international protocols for Guillain-Barré syndrome rehabilitation., (© 2023 The Authors. Nursing & Health Sciences published by John Wiley & Sons Australia, Ltd.)

Introduction: We presented a four-case series of COVID-19 related deaths occurred in patients with Guillain-Barré syndrome (GBS) between February 2020 and January 2022 in Italy., Methods: They were extracted from 8,436 medical charts of COVID-19 patients dying. All cases, ranged 48-73 years, showed classical GBS clinical onset - limb weakness, sensory deficits, hypoareflexia - and three of them were admitted in intensive care unit (ICU) for ventilator support., Results: The cerebrospinal fluid showing albumin-cytological dissociation was performed in two cases. Nerve conduction studies supported the diagnosis in all cases. Interstitial pneumonia was documented by chest X-rays or CT scans in all cases: they were treated with intravenous immunoglobulin (IVIg) and the drugs used for COVID-19 infection., Conclusions: Although the mechanism of GBS onset is still unclear in COVID-19, fatal cases may be more frequent than other virus-related GBS, so that strictly monitoring in high-risk patients could dramatically decrease the mortality of GBS.

Background: Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are human autoimmune peripheral neuropathy. Besides humoral immunity, cellular immunity is also believed to contribute to these pathologies, especially CIDP. Sphingosine-1-phosphate receptor 1 (S1PR1) regulates the maturation, migration, and trafficking of lymphocytes. As of date, the therapeutic effect of sphingosine-1-phosphate receptor (S1PR) agonists on patients with GBS or CIDP remains unclear., Methods: To evaluate the effect of siponimod, an agonist of S1PR1 and S1PR5, on experimental autoimmune neuritis (EAN), an animal model of autoimmune peripheral neuropathy, was used. Lewis rats were immunized with 125 μg of synthetic peptide from bovine P2 protein. Rats in the siponimod group were orally administered 1.0 mg/kg siponimod and those in the EAN group were administrated the vehicle on days 5-27 post-immunization (p.i.) daily. The symptom severity was recorded daily. The changes in the expression of cytokines and transcription factors in the lymph nodes and cauda equina (CE) which correlate with the pathogenesis of EAN and recovery of injured nerve were measured using reverse transcription quantitative PCR. Histological study of CE was also performed., Results: Flaccid paralysis developed on day 11 p.i. in both groups. Siponimod relieved the symptom severity and decreased the expression of interferon-gamma and IL-10 mRNAs in lymph nodes and CE compared with that in the EAN group. The expression of Jun proto-oncogene (c-Jun) mRNA increased from the peak to the recovery phase and that of Sonic hedgehog signaling molecule (Shh) and Glial cell line-derived neurotrophic factor (Gdnf) increased prior to increase in c-Jun with no difference observed between the two groups. Histologically, siponimod also reduced demyelinating lesions and inflammatory cell invasion in CE., Conclusions: Siponimod has a potential to ameliorate EAN. Shh and Gdnf, as well as C-Jun played a significant role during the recovery of injured nerves., (© 2023. The Author(s).)

Background: Most previous studies comparing the effectiveness of Plasma Exchange (PE) or intravenous immunoglobulin (IVIG) in treating Guillain-Barre syndrome (GBS) have focused on the short-term outcome at around 1 month., Objective: To compare the long-term efficacy of PE and IVIG at one year in adult patients with GBS., Methods: Eighty-one adult patients with acute GBS were randomized into two groups with a ratio of 2 : 1: PE (N = 54) and IVIG (N = 27). Patients were assessed with the Medical Research Council sum score (MRC sum score), GBS Disability Scale (GDS), and Functional assessment of acute inflammatory neuropathy (FAAIN) at baseline, ten days, one month, three months, and one year. Neurophysiological examinations were performed at baseline and three months following treatment., Results: There were no significant differences between groups in demographic, clinical, and laboratory data. Both treatments produced a significant improvement in all clinical rating scales in both groups that continued up to one year. There were significant differences in the time course of recovery in the MRC and FAAIN scales, with significantly more improvement in the IVIG group at 1 and 3 months, although there was no significant difference in outcome at one year. However the effect size showed measurable differences between the PE and IVIG groups across the different measures at one-year. Electrophysiological studies showed equal improvement in most measures in both groups at three months, with a slightly greater effect in the IVIG group., Conclusion: long term outcomes of IVIG and PE were equivalent. However the effect size showed measurable differences between the PE and IVIG groups across the different measures at one-year follow-up that indicate the superiorty of IVIG. There was also a tendency for improvement to be slightly faster in the IVIG group.

Guillain-Barré syndrome (GBS) is an acute immune-mediated paralytic neuropathy with variable disease course and outcome. In this study, we aimed to investigate the therapeutic effects of celastrol on GBS and uncover its underlying mechanisms. Experimental autoimmune neuritis (EAN) is a typical animal model for GBS, and thus an EAN rat model was established with the injection of celastrol or/and LPS. We assessed the body weights and EAN clinical scores of rats. HE staining, flow cytometry, RT-qPCR, and Western blotting were respectively employed to measure pathological damage, proportions of cells (Th1, Th17, and Treg), Th1/Th17 cell differentiation-related mRNAs (IFN-γ, TBX21, IL-18, RORγT, IL-17, and IL-23) and TLR4/NF-κB/STAT3 pathway-related proteins (TLR4, NF-κB, p-NF-κB, STAT3, and p-STAT3). We found that celastrol attenuated clinical symptoms and pathological damage of GBS in EAN rats. Moreover, celastrol down-regulated Th1 and Th17 cell proportions, and the levels of IFN-γ, TBX21, IL-18, RORγT, IL-17, and IL-23 in EAN rats. Meanwhile, the levels of TLR4, p-NF-κB, and p-STAT3 were decreased by celastrol. Taken together, celastrol could restrain Th1/Th17 cell differentiation through inhibition of the TLR4/NF-κB/STAT3 pathway in EAN rats. Our findings suggest that celastrol may exert therapeutic effects on GBS by suppressing TLR4/NF-κB/STAT3 pathway-mediated Th1/Th17 cell differentiation.

Objective: To compare the efficacy of intravenous immunoglobulin (IVIG) added to antiretroviral therapy (ART) and ART alone in people living with human immunodeficiency virus-associated Guillain-Barré syndrome (HIV-associated GBS)., Methods: The study was a retrospective analysis of clinical records of HIV-associated GBS patients from department of neurology at West China Hospital between January 2015 and October 2020. Patients treated with ART alone were compared with those treated with IVIG + ART. The primary outcome was the GBS disability score at 4 weeks, which was assessed with multivariable ordinal regression. Additional outcomes include the GBS disability scale at 1 week, improvement of ≥ 1 point on the GBS disability score at 1 and 4 weeks, median change in the MRC sum score at 1 and 4 weeks, number of patients who were able to walk independently at 4 weeks., Results: Two hundred and fifty-two (252) individuals living with HIV were recruited in the study. According to the inclusion and exclusion criteria, 21 HIV-associated GBS patients were finally included, of whom 8 were treated with IVIG + ART and 13 were treated with ART alone. At the fourth weeks after treatment, the GBS disability scale grade was significantly lower in patients treated with IVIG + ART than those with ART alone (1 vs. 2, P = 0.02). The adjusted OR for a lower GBS disability scale was 10.6 (95 % CI 1.15 to 98.05; P = 0.03) for the IVIG + ART group. Moreover, 6 of 8 (75 %) patients treated with IVIG + ART were able to walk independently at four weeks after treatment., Conclusions: The introduction of IVIG combined with ART may be efficacious in the treatment of HIV-infected GBS and may provide better clinical outcomes., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022. Published by Elsevier B.V.)

The patient, a 50-year-old woman, presented with fever and diarrhea in early July, X. One week later, she noticed muscle weakness in both lower extremities, which upon examination was found to be dominant in the distal muscles, with associated loss of tendon reflexes. We diagnosed the case as Guillain-Barré syndrome. After admission, the patient experienced decreased oxygenation, and a chest X-ray indicated elevation of the left hemidiaphragm. The phrenic nerve conduction studies revealed laterality of the amplitude of compound muscle action potential, and diaphragmatic ultrasonographic examination revealed decreased left diaphragmatic wall motion. We diagnosed the patient with unilateral diaphragmatic nerve palsy and initiated intravenous immunoglobulin and methylprednisolone treatment. After 2 weeks, the patient demonstrated good clinical recovery, increased diaphragmatic nerve amplitude, and improved diaphragmatic movement. We evaluated the longitudinal clinical course of unilateral diaphragmatic nerve palsy in the patient using nerve conduction tests and diaphragmatic echocardiography. The longitudinal evaluation allowed us to assess the pathological condition more sensitively so that the prognosis could be predicted accurately.

Introduction: The treatment of Guillain-Barré Syndrome (GBS) with intravenous immunoglobulin (IVIg) or plasma exchange (PE) reduces time to clinical recovery. Although sometimes used in clinical practice, the benefit of a second treatment cycle is of unproven benefit., Aims: Our aim was to compare GBS prognosis in patients treated with one or two cycles of IVIg or PE., Methods: We selected patients with electrophysiological studies compatible with acute inflammatory demyelinating polyneuropathy or acute motor-sensory axonal neuropathy, from January 2018 to December 2020 in our hospital. Our primary outcome was any improvement in the Guillain-Barré Syndrome Disability Score (GBS-DS) at a mean of twelve weeks. We compared patients treated with one or two treatment cycles with a binary regression., Results: We included twenty-six patients, 65.4% with the classical presentation and 30.8% were treated with two cycles. Patients treated with two cycles presented a higher basal GBS-DS (median 4; IQR 1-5) compared with the group of patients treated with one cycle (median 3; IQR 1-5), p = 0.01. The remaining basal characteristics were similar between groups. The two-cycle treatment regimen did not associate with an improvement in GBS-DS (OR 0.28, 95% CI 0.03-2.35, p = 0.24). Likewise there was no benefit in the need for intensive care unit (OR 2.0, 95% CI 0.37-10.92, p = 0.42) or mechanical invasive ventilation (OR 10.2, 95% CI 0.86-120.96, p = 0.66)., Discussion: Our analysis reinforces the recent literature data regarding the absence of benefit of two treatment cycles in patients with GBS., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Background and Purpose: Intravenous immunoglobulin (IVIg) is recommended in Guillain-Barré syndrome (GBS), but its efficacy may vary in different subtypes. We report the outcomes of patients with GBS following IVIg treatment compared to the natural course (NC). We also compare the effect of IVIg treatment in different subtypes of GBS., Methods: From a cohort of 528 GBS subjects, we have extracted 189 patients who received IVIg and compared their outcomes with 199 age- and peak disability-matched patients who did not receive IVIg, plasmapheresis, or corticosteroid. Disability was assessed using the 0-6 Guillain-Barré Syndrome Disability Scale (GBSDS). Clinical and neurophysiological subtypes were recorded. The primary outcome was functional disability at 6 months, which was categorized as complete (GBSDS ≤ 1), partial (GBSDS 2-3), or poor (GBSDS &gt; 3). The secondary outcomes were in-hospital death, duration of hospitalization, and mechanical ventilation., Results: In-hospital death (2.6% vs. 2%, p = 0.74) and 3-month poor recovery (20.7% vs. 18%) were similar in the IVIg and NC groups. At 6 months, however, a lesser proportion of patients in the IVIg group had poor recovery (2.2% vs. 8.3%, p = 0.026). The outcomes of IVIg and NC were compared in 72 acute motor axonal neuropathy (AMAN) and 256 acute inflammatory demyelinating polyradiculoneuropathy (AIDP) patients. IVIg therapy did not alter the outcome in AMAN but resulted in a lesser proportion of poor recovery at 6 months in AIDP (0.8% vs. 6.6%, p = 0.03)., Conclusions: IVIg is beneficial in AIDP variants of GBS but not in the AMAN subtype. A customized treatment may be cost-effective until a randomized controlled trial is conducted in AMAN., (© 2022 European Academy of Neurology.)

Background: Guillain-Barré syndrome (GBS) is an immune-mediated polyradiculoneuropathy with the classic presentation of acute onset neurological symptoms preceded by an infective illness, followed by progressive limb weakness. Unilateral facial paralysis is rarely seen in GBS., Case Presentation: We reported a child presented with unilateral facial paralysis, limited outward movement of one eye and unilateral lower limb weakness, who was later diagnosed to have GBS. Through reviewing the patients with similar presentation reported previously, we found that the onset time of unilateral facial weakness in relation to other presentations of GBS seemed to be variable, which could be later or earlier than other symptoms, or concomitant. Most of the patients had a relatively good outcome within 2 weeks to 12 months of follow-up., Conclusions: Unilateral facial paralysis may be a feature of GBS, albeit a rare thing. Recognising the clinical patterns of such atypical variants of GBS allows for more timely and accurate diagnosis, and for treatment to be initiated without delay., (© 2022 International Society for Developmental Neuroscience.)

Background and Objective: Intravenous immunoglobulin (IVIg) at a standard dosage is the treatment of choice for Guillain-Barré syndrome. The pharmacokinetics, however, is highly variable between patients, and a rapid clearance of IVIg is associated with poor recovery. We aimed to develop a model to predict the pharmacokinetics of a standard 5-day IVIg course (0.4 g/kg/day) in patients with Guillain-Barré syndrome., Methods: Non-linear mixed-effects modelling software (NONMEM ® ) was used to construct a pharmacokinetic model based on a model-building cohort of 177 patients with Guillain-Barré syndrome, with a total of 589 sequential serum samples tested for total immunoglobulin G (IgG) levels, and evaluated on an independent validation cohort that consisted of 177 patients with Guillain-Barré syndrome with 689 sequential serum samples., Results: The final two-compartment model accurately described the daily increment in serum IgG levels during a standard IVIg course; the initial rapid fall and then a gradual decline to steady-state levels thereafter. The covariates that increased IgG clearance were a more severe disease (as indicated by the Guillain-Barré syndrome disability score) and concomitant methylprednisolone treatment. When the current dosing regimen was simulated, the percentage of patients who reached a target ∆IgG > 7.3 g/L at 2 weeks decreased from 74% in mildly affected patients to only 33% in the most severely affected and mechanically ventilated patients (Guillain-Barré syndrome disability score of 5)., Conclusions: This is the first population-pharmacokinetic model for standard IVIg treatment in Guillain-Barré syndrome. The model provides a new tool to predict the pharmacokinetics of alternative regimens of IVIg in Guillain-Barré syndrome to design future trials and personalise treatment., (© 2022. The Author(s).)

Objectives: To evaluate the safety of immune checkpoint inhibitor use in patients with pre-existing neurological autoimmune diseases., Methods: In this retrospective case-series, we examined exacerbations of underlying disease and the occurrence of immune-related adverse events in 5 patients who had been diagnosed with a neurological autoimmune disease prior to receiving immune checkpoint inhibitor therapy for advanced malignancy., Results: Two patients had a prior diagnosis of myasthenia gravis, two had Guillain-Barré syndrome, and one had chronic idiopathic demyelinating polyneuropathy. Only one patient experienced a flare of neurological autoimmune disease. Four of the five patients experienced immune-related adverse events unrelated to their neurological disease., Conclusions: In this case-series, exacerbations of neurological autoimmune disease were less common and less severe than expected. Further research is needed to determine which individuals are at greatest risk of neurological autoimmune disease complication while receiving immune checkpoint inhibitor therapy., (Copyright © 2022. Published by Elsevier B.V.)

Guillain-Barrè syndrome (GBS) is an acute immune-mediated neuropathy, possibly triggered by a recent infection or vaccination, and driven by an immune attack targeting the peripheral nervous system. GBS typically leads to ascending limb weakness, often with sensory and cranial nerve involvement 1-2 weeks after immune stimulation, but emergency and neurology physicians should be aware of its important clinical heterogeneity. In rare cases, bilateral facial nerve palsy can be the main clinical manifestation, as the case of the variant formerly known as bilateral facial weakness with paresthesias. An increasing number of case reports of GBS in patients receiving COVID-19 vaccination have been reported both during the pre-clinical phase and after large-scale authorities' approval. We report two cases of bifacial palsy with paresthesias, a rare variant of GBS, both occurring after the first dose of COVID-19 vaccine Vaxzevria™ (formerly COVID-19 vaccine AstraZeneca), showing a favorable outcome after high-dose immunoglobulin therapy, and discuss the literature of GBS post-COVID-19 vaccination., (© 2022. The Author(s).)

Guillain-Barré syndrome (GBS) has occasionally occurred in people who have received coronavirus disease 2019 (COVID-19) vaccines. Dysgeusia is rare symptom of GBS. We herein report a rare case of sensory ataxic GBS with dysgeusia just after the second dose of the Pfizer-BioNTech COVID-19 vaccine. Although autoantibodies against glycolipids were not detected, immunotherapy with intravenous immunoglobulin and methylprednisolone pulse therapy effectively ameliorated the symptoms. Our report suggests that the COVID-19 vaccine may induce various clinical subtypes of GBS, including a rare variant with sensory ataxia and dysgeusia.

Background: Intravenous immunoglobulin (IVIg) have been administrated for the long time in patients with several autoimmune neuromuscular diseases. Eczematous eruption has been described as IVIg-induced adverse effect., Objective: The purpose of this study is to clarify the incidence and characteristic of IVIg-induced eczematous eruption in autoimmune neuromuscular disease., Methods: We retrospectively collected the data from 92 patients with autoimmune neuromuscular diseases, including 35 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 8 patients with multifocal motor neuropathy (MMN), 25 patients with myositis, 15 patients with Guillain-Barré syndrome (GBS), and 9 patients with myasthenia gravis (MG), who have administrated IVIg in Yamaguchi University Hospital., Results: There are 10 patients (6 CIDP/4 MMN), who had an eczematous skin reaction after IVIg infusion. The frequencies of IVIg-induced eczematous eruption were significantly higher in patients with multifocal acquired demyelinating sensory and motor (MADSAM) and MMN than in patients with GBS, myositis, and MG. In addition, corticosteroids or immunosuppressive drugs had been administrated before IVIg treatment more frequently in patients with myositis and MG than in those with MADSAM and MMN., Conclusion: MADSAM or MMN patients had more frequently IVIg-induced eczematous eruption than other autoimmune neuromuscular diseases. Pathophysiology of MADAM and MMN is considered to be cell-mediated immunity against the peripheral nerve and the accumulation of IgG in both epidermis and dermis of the hand after IVIg may induce the infiltration of inflammatory cells around the vessels in the skin, causing eczematous eruption in MADSAM and MMN.

A 9-year-old girl was admitted to our hospital with severe plantar pain, 7 days after the onset of Campylobacter jejuni enteritis. On admission, extremity strength and the deep tendon reflex were normal; however, there was difficulty in walking owing to plantar pain. Motor nerve conduction test showed no abnormalities. No spinal cord protein cell dissociation. Lumbar spine-enhanced MRI showed a 4th and 5th lumbar vertebrae nerve root contrast-enhanced effect. Gabapentin was effective in minimizing her pain, eventually enabling the patient to walk. Antiganglioside antibody tests on admission showed multiple positive results. Six months after the initial onset of symptoms, she had recovered completely. She was suspected with sensory Guillain-Barré syndrome (GBS). GBS subsequent to Campylobacter jejuni enteritis has been recognized as an acute motor axonal neuropathy; hence, this report is considered to be valuable.

Guillain-Barré syndrome (GBS) is an acute autoimmune polyradiculoneuropathy affecting 1-2 subjects per 100,000 every year worldwide. It causes, in its classic form, symmetric weakness in the proximal and distal limb muscles with common involvement of the cranial nerves, particularly facial weakness. Respiratory function is compromised in a case in four. Randomised controlled trials have demonstrated the benefit of therapeutic plasma exchange in hastening time to recovery. Intravenous immunoglobulin was subsequently shown to be as efficacious as plasma exchange in adult subjects. In children, few trials have shown the benefit of intravenous immunoglobulin versus supportive care. Pharmacokinetic studies suggested a relationship between increase in immunoglobulin G level post-infusion and outcome, implying administration of larger doses may be beneficial in subjects with poor prognosis. However, a subsequent trial of a second dose of immunoglobulin in such subjects failed to show improved outcome, while demonstrating a higher risk of thromboembolic side-effects. Monoclonal antibody therapy has more recently been investigated for GBS, after multiple studies in animal models, with different agents and variable postulated mechanisms of action. Eculizumab, a humanised monoclonal antibody against the complement protein C5, was tested in in two randomised, double-blind, placebo-controlled phase 2 trials. Neither showed benefit versus immunoglobulins alone on disability level at 4 weeks, although one study importantly suggested possible, clinically highly relevant, late effects on normalising function. A phase 3 trial is in progress. Preliminary results of a placebo-controlled ongoing study of ANX005, a humanised recombinant antibody against C1q inhibiting the complement cascade, have been promising., (© 2022. The Author(s).)

A 9-year-old boy presented with unbalanced gait, back pain and lower limb weakness. His physical examination revealed almost absent lower limbs reflexes and cerebro-spinal fluid (CSF) showed albuminocytologic dissociation. The brain and spine MRI with contrast illustrated abnormal enhancement-suggestive of Guillain-Barré syndrome.The case had limited distribution and it did not progress beyond the presenting clinical involvements. They did not need immunotherapy, self-recovered, managed conservatively using painkillers and gabapentin along with physiotherapy-with a wait and see approach. The child is now almost back to normal after 8-12 weeks., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Rationale: Sleep disturbance is commonly noted after Guillain-Barré syndrome (GBS) and is often caused by persistent discomfort after disease survival. Intravascular laser irradiation of blood (ILIB) has been shown to be effective in pain modulation owing to the influence of nociceptive signals in the peripheral nervous system. We investigated the application of ILIB on post-Oxford -AstraZeneca vaccination GBS and evaluated its effect on sleep quality., Patient Concerns: A 48-year-old woman was subsequently diagnosed with GBS after Oxford-AstraZeneca vaccination. The patient was discharged after a 5-day course of intravenous immunoglobulin administration. However, 1 week after discharge, the previously relieved symptoms flared with accompanying sleep disturbance., Diagnosis and Interventions: The patient was diagnosed with post-vaccination GBS, and persistent pain and sleep disturbances persisted after disease survival. ILIB was performed., Outcomes: We used the Pittsburgh Sleep Quality Index before and after intravascular laser irradiation. There was a marked improvement in the sleep duration, efficiency, and overall sleep quality. The initial score was 12 out of 21 and the final score was 7 out of 21., Lessons: We found that ILIB was effective in pain modulation in post-vaccination GBS and significantly improved sleep quality., Competing Interests: The authors have no funding and conflicts of interests to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Neurological manifestations are common in SARS-CoV-2 infection, including life-threatening acute muscle weakness, due to neuromuscular disorders such as acute transverse myelitis (TM) and Guillain-Barré syndrome (GBS). These syndromes can rarely coexist and present as an overlap syndrome. Here, we report a patient who developed acute symmetrical proximal lower limb weakness 5 days after diagnosis of COVID-19. GBS was diagnosed due to the presence of motor signs, albumin-cytological dissociation in cerebrospinal fluid examination and axonal damage according to nerve condition tests. However, abnormal areas on MRI of the thoracic spine and lack of improvement with intravenous immunoglobulin supported a diagnosis of TM. Therefore, a possible overlap between GBS and TM was established. To our knowledge, this is the third case report of GBS/TM overlap syndrome after COVID-19. The patient's full and rapid recovery with intravenous corticosteroids and plasmapheresis supports our diagnosis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: The effect of Glucocorticoids (GCs) on the treatment of Guillain-Barré syndrome (GBS) has been controversial. There is no information on whether specific subtypes of GBS respond differently to GCs. In this setting, we aimed to discuss whether GCs treating yield different effects in the distinct subtypes (acute inflammatory demyelinating polyneuropathy, AIDP; acute motor axonal neuropathy, AMAN). And further, we analyzed the impact of different doses on the outcome., Methods: Medical records of 448 patients with a diagnosis of classic GBS admitted to 31 tertiary hospitals, located in 14 provinces of Southern China, from 1 January 2013 to 30 September 2016, were retrospectively collected. And 251 patients treated with GCs alone (AIDP=189, AMAN=62) were reviewed and analyzed., Results: After GCs treatment, the Hughes score of AIDP patients was significantly lower than that of AMAN patients at discharge (P=0.005) and 3 months after onset (P<0.001). Further analysis revealed that among AIDP patients, the high-dose group had significantly shorter hospital stay (P=0.023), lower Hughes score at nadir (P<0.001), at discharge (P=0.005), and 3 months after onset (P<0.001), compared with the low-dose group. However, for AMAN patients, the outcome difference between groups was nonsignificant., Conclusion: Our data suggest that the high doses of GCs may result, at least in part, from the side of the duration of hospital stay and short-term outcome, favorable outcomes in AIDP patients. Therefore, we cannot completely deny the priority of GCs in the treatment of GBS, because the effect of different doses of GCs varies in treating different subtypes. More studies are needed in the future to further validate this issue., Trial Registration: ChiCTR-RRC-17014152 . Registered 26 December 2017- Retrospectively registered., (© 2022. The Author(s).)