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Shimizu F, Koga M, Mizukami Y, Watanabe K, Sato R, Takeshita Y, Maeda T, Kanda T, Nakamori M. Small Nuclear Ribonucleoprotein Autoantibody Associated With Blood-Nerve Barrier Breakdown in Guillain-Barré Syndrome. NEUROLOGY(R) NEUROIMMUNOLOGY & NEUROINFLAMMATION 2025; 12:e200405. [PMID: 40388674 PMCID: PMC12092546 DOI: 10.1212/nxi.0000000000200405] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 10/15/2024] [Accepted: 03/11/2025] [Indexed: 05/21/2025]
Abstract
BACKGROUND AND OBJECTIVES Breakdown of the blood-nerve barrier (BNB) is observed in patients with Guillain-Barré syndrome (GBS); however, the molecular mechanism underlying this phenomenon remains unclear.The aim of this study was to identify antibodies against the BNB-endothelial cells that initiate BNB breakdown in patients with GBS. METHODS We purified IgGs from the serum samples of patients with GBS (n = 77) during the acute phase, disease controls ([DCs], n = 51), and healthy controls ([HCs], n = 24). Human peripheral nerve microvascular endothelial cells (PnMECs) were incubated with IgG. Molecular changes in PnMECs after GBS-IgG exposure were evaluated using RNA-seq and a high-content imaging system. U1-small nuclear ribonucleoprotein (U1-snRNP) autoantibodies were detected using an ELISA. The clinical information of U1-snRNP antibody-positive GBS patients was verified. RESULTS GBS-IgGs significantly increased NF-κB nuclear translocation and permeability of the 10-kDa dextran in PnMECs compared with DC-IgGs or HC-IgGs. RNA-seq analyses of PnMECs demonstrated that NF-κB p65 in the center of the network analysis, snRNPs as upstream genes of NF-κB p65, and CXCR5 as downstream genes of NF-κB p65 were important molecules after GBS-IgG exposure. The protein level of claudin-5 and U1-snRNP was significantly reduced while that of CXCR5 was significantly increased after incubation with IgG from patients with GBS, compared with that from HCs. The rate of U1-snRNP antibody positivity was 36% (28 of 77) in patients with GBS, 7% (2 of 28) in DCs, and 0% (0 of 16) in HCs. The serum titer of snRNP antibody decreased after treatment. Both cerebral spinal fluid protein and albumin quotient (QALB)/QALBLIM were higher in snRNP antibody-positive GBS patients than in snRNP antibody-negative GBS patients. IgG from U1-snRNP antibody-positive GBS patients decreased the barrier function and claudin-5 expression more than that from HCs in an in vitro BNB coculture model. The reduction in U1-snRNP antibody decreased the biological effect of IgG from GBS patients with U1-snRNP antibody on the increased permeability of PnMECs. DISCUSSION U1-snRNP autoantibodies are associated with the breakdown of BNB in GBS, through the reduction of U1-snRNP and claudin-5 and the induction of NF-κB activation in BNB-endothelial cells. A temporary autoantibody response against snRNP may be boosted by the periodic response to infection in GBS.
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Affiliation(s)
- Fumitaka Shimizu
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Ube, Japan
| | - Michiaki Koga
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Ube, Japan
- Faculty of Medicine and Health Sciences, Yamaguchi University Graduate School of Medicine, Ube, Japan
| | | | - Kenji Watanabe
- Center for Gene Research, Yamaguchi University, Ube, Japan
| | - Ryota Sato
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Ube, Japan
| | - Yukio Takeshita
- Department of Neurotherapeutics, Yamaguchi University School of Medicine, Ube, Japan
- BBB Research Center, Yamaguchi University School of Medicine, Ube, Japan; and
- Research Institute for Cell Design Medical Science, Yamaguchi University, Ube, Japan
| | - Toshihiko Maeda
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Ube, Japan
| | - Takashi Kanda
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Ube, Japan
| | - Masayuki Nakamori
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Ube, Japan
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Liu AN, Yang JY, Chen XY, Wu SS, Ji Zhi SN, Zheng SM. Refractory Crohn's disease complicated with Guillain-Barré syndrome: A case report. World J Clin Cases 2025; 13:103618. [DOI: 10.12998/wjcc.v13.i18.103618] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2024] [Revised: 01/24/2025] [Accepted: 02/17/2025] [Indexed: 02/27/2025] Open
Abstract
BACKGROUND Inflammatory bowel disease (IBD) comprises a group of chronic inflammatory gastrointestinal disorders, including Crohn's disease (CD) and ulcerative colitis, with uncertain etiologies. The natural course of IBD can be accompanied by extraintestinal manifestations involving the skin, mucous membranes, musculoskeletal structures, eyes, cardiovascular system and nervous system. Guillain-Barré syndrome (GBS) is a type of peripheral neuropathy. However, the etiology and pathogenesis of IBD combined with GBS are unclear, and only a few clinical cases have been reported. Here, we report a case of refractory CD complicated by GBS and review the previous literature to improve the understanding of these diseases.
CASE SUMMARY A 34-year-old man had a 9-year history of refractory CD. He became unresponsive to multiple drugs and experienced recurrent intestinal fistulas. After several abdominal surgeries and treatment with ustekinumab, he achieved clinical remission. Unfortunately, he developed GBS during maintenance treatment with ustekinumab. According to previous reports, in some patients with IBD combined with GBS, GBS may be a comorbidity, an extraintestinal manifestation of IBD, or an adverse reaction to IBD therapeutic drugs. After a comprehensive evaluation, we suspected that GBS might have been a comorbidity in this patient. To avoid fatal disease relapse after medication discontinuation, we concluded that ustekinumab should not be withdrawn. On the basis of a joint decision between doctors and the patient, we decided to continue maintenance treatment with ustekinumab along with intravenous immunoglobulin, dexamethasone and traditional Chinese medicine acupuncture, which resulted in a steady improvement in his GBS symptoms and sustained remission of CD.
CONCLUSION When IBD is complicated by a neurological disease, it is first necessary to analyze the patient's condition and then choose the corresponding treatment strategy. If the neurological disease is a specific comorbidity, treatment of both IBD and the comorbid disease should be considered. For IBD patients with extraintestinal manifestations involving the nervous system, neurological manifestations tend to resolve when the active IBD is controlled. When an adverse drug reaction is suspected, the medication should be discontinued, and symptomatic treatment should be administered.
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Affiliation(s)
- A-Niu Liu
- Department of Gastroenterology and Hepatology, Chengdu Medical College, Chengdu 610500, Sichuan Province, China
- Department of Gastroenterology and Hepatology, The General Hospital of Western Theater Command, Chengdu 610083, Sichuan Province, China
| | - Jia-Yi Yang
- Department of Radiology, Wuxi People’s Hospital, Nanjing Medical University, Nanjing 214023, Jiangsu Province, China
| | - Xing-Yu Chen
- Department of Gastroenterology and Hepatology, The General Hospital of Western Theater Command, Chengdu 610083, Sichuan Province, China
| | - Shan-Shan Wu
- Department of Gastroenterology and Hepatology, The General Hospital of Western Theater Command, Chengdu 610083, Sichuan Province, China
| | - Se-Niu Ji Zhi
- Department of Gastroenterology and Hepatology, The General Hospital of Western Theater Command, Chengdu 610083, Sichuan Province, China
| | - Shu-Mei Zheng
- Department of Gastroenterology and Hepatology, The General Hospital of Western Theater Command, Chengdu 610083, Sichuan Province, China
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Higgins TJ, Cureton KD, Jacob AK, Kane SV, Klein CJ, Chen J. Anesthesia Experience for Guillain-Barre Syndrome in Endoscopy Procedures: A Retrospective Case Series. Mayo Clin Proc Innov Qual Outcomes 2025; 9:100609. [PMID: 40256620 PMCID: PMC12008634 DOI: 10.1016/j.mayocpiqo.2025.100609] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/22/2025] Open
Abstract
Objective To determine whether patients with previous Guillain-Barre syndrome (GBS) encountered anesthetic complications that necessitated an unanticipated escalation of care during endoscopic procedures. Patients and Methods We reviewed 309 patients diagnosed with GBS who underwent 537 gastrointestinal endoscopic procedures at all Mayo Clinic geographic sites from January 1, 2012 to May 25, 2023. Our study included patients with GBS from acute onset, chronic/relapsing to full/partial recovery phases. We assessed whether GBS correlated with unanticipated escalations of care, defined as intraprocedural escalation between types of sedation or unanticipated hospital admission after an endoscopic procedure. Results No case exhibited anesthesia-related complications or required escalations of care. A total of 85% of cases (458) were performed without a secured airway. Within 6 months of GBS acute onset, 61% of cases (16 of 26) required general endotracheal anesthesia. Nine of 26 acute onset cases involved patients already intubated or with tracheostomies, primarily to place percutaneous endoscopic gastrostomy feeding tubes to advance care outside of the intensive care unit. 33 cases received paralyzing doses of succinylcholine; 3 involved patients with reported residual muscle weakness. Conclusion Acute onset GBS cases frequently present with bulbar dysfunction, respiratory distress, and muscle weakness. Patients within 6 months of acute onset should delay elective endoscopic procedures, whereas urgent/emergent ones should be scheduled on a case-by-case basis. Beyond 6 months, most patients exhibit dramatic functional recovery, allowing for administration of sedation without a protected airway. Regardless of time since onset, residual GBS symptoms-particularly respiratory distress and bulbar dysfunction-can help risk stratify patients for periprocedural aspiration risks.
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Affiliation(s)
- Timon J. Higgins
- Department of Anesthesiology & Perioperative Medicine, Mayo Clinic, Rochester, MN
| | | | - Adam K. Jacob
- Department of Anesthesiology & Perioperative Medicine, Mayo Clinic, Rochester, MN
| | - Sunanda V. Kane
- Department of Gastroenterology & Hepatology, Mayo Clinic, Rochester, MN
| | | | - James Chen
- Department of Anesthesiology & Perioperative Medicine, Mayo Clinic, Rochester, MN
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Wagner W, Krawiec M, Iwanicki A, Krupa P, Gębka-Kępińska B, Lubiński M, Białka S. Where did the attack come from? The mysterious case of paralysis mimicking Guillain-Barré syndrome: Case report. Medicine (Baltimore) 2025; 104:e42118. [PMID: 40419896 DOI: 10.1097/md.0000000000042118] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 05/28/2025] Open
Abstract
RATIONALE Tick-borne encephalitis (TBE) can present with neurological symptoms mimicking Guillain-Barré syndrome (GBS), posing a significant diagnostic challenge. Misidentification may lead to delayed or inappropriate treatment, increasing the risk of complications. PATIENT CONCERNS A 49-year-old man presented with flu-like symptoms and progressive paralysis of the left upper limb following a tick bite. His condition rapidly deteriorated despite initial therapy, including facial nerve palsy, further paralysis of the left upper limb, and swallowing difficulties. DIAGNOSES The initial clinical picture, cerebrospinal fluid analysis showing elevated protein, and electromyography suggested GBS. INTERVENTIONS The patient underwent 6 plasmapheresis sessions for suspected GBS, with no neurological improvement. OUTCOMES Due to a lack of improvement and further magnetic resonance imaging findings, a repeated lumbar puncture and serological testing revealed the correct diagnosis of TBE, confirmed by the presence of IgM and IgG antibodies against the TBE virus. After stabilization and intensive care unit discharge, the patient was transferred to a neurology ward and later to a rehabilitation unit. Significant neurological improvement was observed, although partial left upper limb paresis and facial nerve palsy persisted. He was eventually discharged with continued outpatient neurology and cardiology follow-up. LESSONS This case highlights the importance of detailed medical history, including tick exposure, and maintaining a broad differential diagnosis in acute flaccid paralysis. Early consideration of infectious etiologies, particularly in endemic regions, is essential to prevent unnecessary interventions and improve patient outcomes.
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Affiliation(s)
- Wiktor Wagner
- Student Scientific Society at the Department of Anaesthesiology and Intensive Therapy in Zabrze, Medical University of Silesia in Katowice, Katowice, Poland
| | - Michał Krawiec
- Student Scientific Society at the Department of Anaesthesiology and Intensive Therapy in Zabrze, Medical University of Silesia in Katowice, Katowice, Poland
| | - Adam Iwanicki
- Student Scientific Society at the Department of Anaesthesiology and Intensive Therapy in Zabrze, Medical University of Silesia in Katowice, Katowice, Poland
| | - Paweł Krupa
- Student Scientific Society at the Department of Anaesthesiology and Intensive Therapy in Zabrze, Medical University of Silesia in Katowice, Katowice, Poland
| | - Barbara Gębka-Kępińska
- Department and Clinic of Neurology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia in Katowice, Katowice, Poland
| | - Mateusz Lubiński
- Department of Anesthesiology and Intensive Therapy, Faculty of Medical Sciences in Zabrze, Medical University of Silesia in Katowice, Katowice, Poland
| | - Szymon Białka
- Department of Anesthesiology and Intensive Therapy, Faculty of Medical Sciences in Zabrze, Medical University of Silesia in Katowice, Katowice, Poland
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Chen L, Yang H, Wang C, Wu H, Zhu Z, Shi Z. Clinical and electrophysiological characteristics and blood markers for short-term prognosis prediction in severe Guillain-Barré syndrome: a retrospective cohort study. Mult Scler Relat Disord 2025; 100:106532. [PMID: 40413968 DOI: 10.1016/j.msard.2025.106532] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/13/2024] [Revised: 05/16/2025] [Accepted: 05/18/2025] [Indexed: 05/27/2025]
Abstract
This study aimed to investigate the clinical, electrophysiological characteristics and blood inflammatory markers in severe Guillain-Barré syndrome (GBS) and their correlation with short-term prognosis. Data from 95 patients with severe GBS were classified into two groups based on the Hughes functional grading scale (HFGS) on day 28: those with poor prognosis (>3) and those with prognosis (≤3). Clinical characteristics, nerve conduction studies and blood parameters were compared at admission between the two groups. Logistic regression analysis identified predictive factors for GBS, and receiver operating characteristic (ROC) curves were used to evaluate the predictive efficacy. A nomogram model combining these predictive factors was constructed and evaluated using ROC and calibration curves and Hosmer⁃Lemeshow goodness-of-fit test. The poor prognosis group exhibited bulbar paralysis and an elevated modified Erasmus GBS Outcome Score (mEGOS) (P < 0.05). Nerve conduction studies revealed increased numbers of inexcitable motor nerves (IMN) in the poor prognosis group. Blood analysis showed significantly elevated neutrophil-to-lymphocyte ratio (NLR) during acute disease stage (P < 0.05) compared with the good prognosis group. ROC curve analysis indicated that mEGOS, NLR value, IMN number, and their combination had area under the curve (AUC) values of 0.818, 0.757, 0.870, and 0.947, with sensitivities of 78.4 %, 76.5 %, 75.0 %, and 92.2 %, and specificities of 77.3 %, 77.3 %, 61.3 %, and 86.4 % respectively, for short-term prognosis prediction. The nomogram model demonstrated an area under the ROC curve of 0.990, reflecting good potential clinical effect. The calibration curve showed good agreement between actual observations and nomogram predictions. The findings indicate that early assessment of the occurrence of bulbar palsy, mEGOS, IMN numbers, and NLR value can predict poor prognosis, with their combination providing improved accuracy for short-term prognosis in patients with severe GBS.
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Affiliation(s)
- Li Chen
- Department of Neurology, Huanhu Hospital Affiliated to Tianjin Medical University, Tianjin 300060, PR China
| | - Hui Yang
- Tianjin First Aid Center, Tianjin 300011, PR China
| | - Change Wang
- Department of Neurology, Huanhu Hospital Affiliated to Tianjin Medical University, Tianjin 300060, PR China
| | - Hao Wu
- Department of Neurology, Huanhu Hospital Affiliated to Tianjin Medical University, Tianjin 300060, PR China
| | - Zilong Zhu
- Department of Neurology, Huanhu Hospital Affiliated to Tianjin Medical University, Tianjin 300060, PR China
| | - Zhihong Shi
- Department of Neurology, Huanhu Hospital Affiliated to Tianjin Medical University, Tianjin 300060, PR China.
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Sprenger-Svačina A, Svačina MKR, Otlu HG, Gao T, Sheikh KA, Zhang G. Endoneurial immune interplay in peripheral nerve repair: insights and implications for future therapeutic interventions. Front Neurosci 2025; 19:1602112. [PMID: 40415889 PMCID: PMC12098419 DOI: 10.3389/fnins.2025.1602112] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/28/2025] [Accepted: 04/16/2025] [Indexed: 05/27/2025] Open
Abstract
The mechanisms underlying axonal injury and repair in peripheral nerves, whether due to traumatic damage or autoimmune neuropathies, are complex and not yet fully understood. Recent research indicates that an orchestrated interplay between damaged neurons, Schwann cells, and especially endoneurial immune cells such as macrophages or T cells is crucial to achieve satisfactory nerve recovery. Following axonal injury, degenerating axons and reactive Schwann cells release chemoattractants and cytokines that recruit immune cells into the endoneurium. Among them, macrophages play a pivotal role by clearing axonal and myelin debris and subsequently creating a pro-regenerative microenvironment that supports axonal outgrowth. There is evidence that the timely switch of a pro-inflammatory M1 toward a pro-regenerative M2 macrophage polarization state is crucial for satisfactory nerve recovery, and supportive cellular and humoral factors that influence the endoneurial microenvironment, such as T cells and their cytokines, can substantially impact this fragile recovery process. The latter explains the limited nerve recovery in immune neuropathies, where a pathologic pro-inflammatory shift within the endoneurial immune cell signature hampers axonal outgrowth. This review aims to provide insights into cellular and humoral determinants of the endoneurial microenvironment during nerve damage and repair, which are assumed to hold substantial potential for future therapeutic interventions, especially since current strategies to enhance peripheral nerve recovery are limited to either surgical interventions in traumatic neuropathies or immunomodulatory drugs in immune neuropathies that often fail to achieve satisfactory functional results.
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Affiliation(s)
- Alina Sprenger-Svačina
- Neuromuscular Research Laboratory, Department of Neurology, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX, United States
- Department of Neurology, Faculty of Medicine, University Hospital of Cologne, Cologne, Germany
| | - Martin K. R. Svačina
- Neuromuscular Research Laboratory, Department of Neurology, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX, United States
- Philipps University Marburg and Department of Neurology, University Hospital Gießen and Marburg, Marburg, Germany
| | - Husniye G. Otlu
- Neuromuscular Research Laboratory, Department of Neurology, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX, United States
- Vocational Health Sciences, Laboratory Techniques Program, Malatya Turgut Ozal University, Malatya, Türkiye
| | - Tong Gao
- Neuromuscular Research Laboratory, Department of Neurology, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX, United States
| | - Kazim A. Sheikh
- Neuromuscular Research Laboratory, Department of Neurology, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX, United States
| | - Gang Zhang
- Neuromuscular Research Laboratory, Department of Neurology, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX, United States
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van Tilburg SJ, Huizinga R, Kuitwaard K, Sassen SDT, Walgaard C, van Doorn PA, Jacobs BC, Koch BCP. If it does not help, it might hurt: Pharmacodynamics of a second IVIg course in Guillain-Barré syndrome. Ann Clin Transl Neurol 2025; 12:966-975. [PMID: 40084606 DOI: 10.1002/acn3.52313] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/24/2024] [Accepted: 12/25/2024] [Indexed: 03/16/2025] Open
Abstract
OBJECTIVES Intravenous immunoglobulin (IVIg) is an effective treatment for Guillain-Barré syndrome (GBS), but recovery varies between patients. This study aims to evaluate the pharmacokinetics (PK) and pharmacodynamics (PD) of a single and a second IVIg dose (SID) in patients with GBS. METHODS Data were analyzed from the SID-GBS trial, a double-blind, randomized, placebo-controlled study. Patients with poor prognosis (modified Erasmus GBS Outcome Score, mEGOS ≥6) after a standard course of IVIg (0.4 g/kg for 5 days) were randomized to receive either SID or placebo. Serum IgG levels were measured at standard serial time points and clinical outcomes were assessed using the GBS disability score and Medical Research Council sum score. PK modeling was performed to predict IVIg exposure and its association with clinical outcomes. RESULTS Serum IgG concentration after a single and double course of IVIg was variable, but accurately described by the current PK model. Lower ΔIgG and IVIg exposure were associated with poorer clinical outcomes. SID increased the IgG concentration, but did not result in an improvement in clinical outcome. Serious adverse events, including thromboembolic events, occurred more frequently in the SID group and were associated with lower IVIg exposure. INTERPRETATION SID increases serum IgG levels in GBS patients as predicted by the current PK model, but does not improve clinical outcomes and increases the risk of serious adverse events. Model-informed precision dosing may guide individualization of treatment.
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Affiliation(s)
- Sander J van Tilburg
- Department of Immunology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
| | - Ruth Huizinga
- Department of Immunology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
| | - Krista Kuitwaard
- Department of Neurology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
- Department of Neurology, Albert Schweitzer Hospital, Dordrecht, The Netherlands
| | - Sebastiaan D T Sassen
- Department of Hospital Pharmacy, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
| | - Christa Walgaard
- Department of Immunology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
- Department of Neurology, IJsselland Hospital, Capelle aan den IJssel, The Netherlands
| | - Pieter A van Doorn
- Department of Neurology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
| | - Bart C Jacobs
- Department of Immunology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
- Department of Neurology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
| | - Birgit C P Koch
- Department of Hospital Pharmacy, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
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Broachwala M, Banks DW, Jevotovsky DS, Oehlermarx W, Durbhakula S. Burning Mouth Syndrome Following Covid Vaccination: A Case Report. Clin Case Rep 2025; 13:e70329. [PMID: 40308482 PMCID: PMC12041126 DOI: 10.1002/ccr3.70329] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2024] [Accepted: 02/09/2025] [Indexed: 05/02/2025] Open
Abstract
This is the first reported case of burning mouth syndrome resulting from the Pfizer-BioNTech COVID-19 vaccine. It highlights the efficacy of a unique multimodal treatment regimen, including zinc supplementation, acupuncture, and topical capsaicin.
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Affiliation(s)
- Mustafa Broachwala
- Department of Anesthesiology and PainUniversity of California San DiegoSan DiegoCaliforniaUSA
| | - Dylan W. Banks
- Department of Physical Medicine and RehabilitationNew York UniversityNew YorkNew YorkUSA
| | - David S. Jevotovsky
- Department of Physical Medicine and RehabilitationNew York UniversityNew YorkNew YorkUSA
| | - Whitman Oehlermarx
- Department of Physical Medicine and RehabilitationNew York UniversityNew YorkNew YorkUSA
| | - Shravani Durbhakula
- Department of AnesthesiologyVanderbilt University Medical CenterNashvilleTennesseeUSA
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Silva A, Prior R, D'Antonio M, Swinnen JV, Van Den Bosch L. Lipid metabolism alterations in peripheral neuropathies. Neuron 2025:S0896-6273(25)00262-4. [PMID: 40311611 DOI: 10.1016/j.neuron.2025.04.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/10/2025] [Revised: 03/14/2025] [Accepted: 04/07/2025] [Indexed: 05/03/2025]
Abstract
Alterations in lipid metabolism are increasingly recognized as central pathological hallmarks of inherited and acquired peripheral neuropathies. Correct lipid balance is critical for cellular homeostasis. However, the mechanisms linking lipid disturbances to cellular dysfunction and whether these changes are primary drivers or secondary effects of disease remain unresolved. This is particularly relevant in the peripheral nervous system, where the lipid-rich myelin integrity is critical for axonal function, and even subtle perturbations can cause widespread effects. This review explores the role of lipids as structural components as well as signaling molecules, emphasizing their metabolic role in peripheral neurons and Schwann cells. Additionally, we explore the genetic and environmental connections in both inherited and acquired peripheral neuropathies, respectively, which are known to affect lipid metabolism in peripheral neurons or Schwann cells. Overall, we highlight how understanding lipid-centric mechanisms could advance biomarker discovery and therapeutic interventions for peripheral nerve disorders.
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Affiliation(s)
- Alessio Silva
- KU Leuven, University of Leuven, Department of Neurosciences, Experimental Neurology and Leuven Brain Institute (LBI), Leuven, Belgium; VIB, Center for Brain & Disease Research, Laboratory of Neurobiology, Leuven, Belgium.
| | - Robert Prior
- KU Leuven, University of Leuven, Department of Neurosciences, Experimental Neurology and Leuven Brain Institute (LBI), Leuven, Belgium; VIB, Center for Brain & Disease Research, Laboratory of Neurobiology, Leuven, Belgium; Department of Ophthalmology, University Hospital Bonn, Medical Faculty, Bonn, Germany
| | - Maurizio D'Antonio
- Biology of Myelin Unit, Division of Genetics and Cell Biology, IRCCS Ospedale San Raffaele, Milan, Italy
| | - Johannes V Swinnen
- Laboratory of Lipid Metabolism and Cancer, Department of Oncology, KU Leuven, Leuven, Belgium
| | - Ludo Van Den Bosch
- KU Leuven, University of Leuven, Department of Neurosciences, Experimental Neurology and Leuven Brain Institute (LBI), Leuven, Belgium; VIB, Center for Brain & Disease Research, Laboratory of Neurobiology, Leuven, Belgium.
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Yang F, Tian Z, Lu Y, Li Y, Liu K. Miller-Fisher syndrome subtype with isolated bilateral mydriasis: a pediatric case report. BMC Neurol 2025; 25:166. [PMID: 40240898 PMCID: PMC12004657 DOI: 10.1186/s12883-025-04180-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2024] [Accepted: 04/07/2025] [Indexed: 04/18/2025] Open
Abstract
BACKGROUND 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. CONCLUSION 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.
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Affiliation(s)
- Fengqi Yang
- Comprehensive pediatrics, Kunming Children's Hospital, No.28, Shulin Street, Kunming, 650103, Yunnan Province, China
| | - Zhu Tian
- Comprehensive pediatrics, Kunming Children's Hospital, No.28, Shulin Street, Kunming, 650103, Yunnan Province, China
| | - Yanhong Lu
- Comprehensive pediatrics, Kunming Children's Hospital, No.28, Shulin Street, Kunming, 650103, Yunnan Province, China
| | - Yang Li
- Comprehensive pediatrics, Kunming Children's Hospital, No.28, Shulin Street, Kunming, 650103, Yunnan Province, China
| | - Kai Liu
- Comprehensive pediatrics, Kunming Children's Hospital, No.28, Shulin Street, Kunming, 650103, Yunnan Province, China.
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Nie X, He W, Qi W, Ma Y, Zhu G, Hao L, Guo X. Polyneuritis cranialis combined with Horner's syndrome: a rare variant of Guillain Barré syndrome. BMC Neurol 2025; 25:162. [PMID: 40240975 PMCID: PMC12001671 DOI: 10.1186/s12883-025-04169-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/11/2024] [Accepted: 04/01/2025] [Indexed: 04/18/2025] Open
Abstract
BACKGROUND Polyneuritis cranialis (PNC), a rare variant of Guillain-Barré syndrome (GBS), is usually characterized by ocular and pharyngeal weakness without obvious numbness or weakness of the limbs or ataxia. Horner's syndrome is extremely rare in patients with PNC. Here, we describe a case of GBS presenting with acute PNC and unilateral Horner syndrome. CASE PRESENTATION A 53-year-old male presented with headache, abducent paresis, peripheral-type facial palsy, bulbar type dysarthria, decreased gag reflex and tongue palsy. Neurological examination showed Cranial Nerve V, VI, VII, IX, X and XII were affected, and Horner's syndrome was observed. Cerebrospinal fluid analysis showed albuminocytologic dissociation. Sensorimotor conduction velocity and needle electromyography of limbs were normal. Magnetic resonance imaging of brain was normal. Finally, the patient was diagnosed as PNC combined with Horner's syndrome. The patient received plasma exchange and intravenous immunoglobulin, which relieved the symptoms rapidly. CONCLUSION GBS presenting only as Horner syndrome and PNC is a challenge for etiological diagnosis. Clinicians need to know enough to distinguish GBS and its variants from other potential similar diseases.
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Affiliation(s)
- Xiangtao Nie
- Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, 1st You Yi Road, Yu Zhong District, Chongqing, 400016, China
| | - Wei He
- Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, 1st You Yi Road, Yu Zhong District, Chongqing, 400016, China
| | - Wenjing Qi
- Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, 1st You Yi Road, Yu Zhong District, Chongqing, 400016, China
| | - Yongbo Ma
- Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, 1st You Yi Road, Yu Zhong District, Chongqing, 400016, China
| | - Geke Zhu
- Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, 1st You Yi Road, Yu Zhong District, Chongqing, 400016, China
| | - Lei Hao
- Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, 1st You Yi Road, Yu Zhong District, Chongqing, 400016, China.
| | - Xiuming Guo
- Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, 1st You Yi Road, Yu Zhong District, Chongqing, 400016, China.
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12
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Murala MST, Gairola V, Sayedahmed EE, Mittal SK. Next-Generation Adenoviral Vector-Based Vaccines for Severe Acute Respiratory Syndrome Coronavirus-2. Vaccines (Basel) 2025; 13:406. [PMID: 40333307 PMCID: PMC12031563 DOI: 10.3390/vaccines13040406] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2025] [Revised: 04/08/2025] [Accepted: 04/11/2025] [Indexed: 05/09/2025] Open
Abstract
This review systematically revises adenovirus (Ad) biology, vector structure, immune responses, and currently available Ad vector COVID-19 vaccines. It analyzes the challenges associated with the Ad vector-based vaccines, including preexisting vector immunity and other side effects. Moreover, this review explores novel and innovative strategies to overcome these constraints for developing next-generation vaccines for broad protection to cover emerging SARS-CoV-2 variants. The future refinement of Ad vaccine platforms will be pivotal in achieving durable immunity against emerging variants for global preparedness.
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Affiliation(s)
| | | | | | - Suresh K. Mittal
- Department of Comparative Pathobiology, Purdue Institute of Inflammation, Immunology and Infectious Disease, College of Veterinary Medicine, Purdue University, West Lafayette, IN 47907-2027, USA; (M.S.T.M.); (V.G.); (E.E.S.)
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13
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Kiper P, Chevrot M, Godart J, Cieślik B, Kiper A, Regazzetti M, Meroni R. Physical Exercise in Guillain-Barré Syndrome: A Scoping Review. J Clin Med 2025; 14:2655. [PMID: 40283485 PMCID: PMC12028042 DOI: 10.3390/jcm14082655] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/17/2025] [Revised: 03/24/2025] [Accepted: 04/10/2025] [Indexed: 04/29/2025] Open
Abstract
Background: Guillain-Barré Syndrome (GBS) is a rare post-infectious, immune-mediated inflammatory disorder of the peripheral nervous system that can manifest in multiple distinct forms. It significantly impacts patients' quality of life, causing both short-term and long-term impairments, including reduced strength, respiratory deficits, functional limitations, decreased endurance, and increased fatigue. This scoping review aimed to assess the impact of physical activity on strength, functional independence, and fatigue in patients with GBS, as well as to identify effective types of physical activity for rehabilitation programs. Methods: A literature search was conducted in March 2024 and updated in June 2024 across PubMed, Embase, Cochrane, Scopus, and Web of Science using predefined inclusion and exclusion criteria. We included full-text, peer-reviewed articles written in English, French, Polish, or Italian that focused on strength, fatigue, and functional independence within the context of physical exercise. Results: A total of 1021 papers were eligible for screening, and after the screening process, 16 papers were included in this review. Conclusions: Physical exercise may enhance strength, reduce fatigue, and promote functional independence in GBS. Recommended interventions often include muscle strengthening, functional training, and endurance exercises. Larger, high-quality studies and further research into chronic fatigue mechanisms are needed to refine long-term rehabilitation strategies.
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Affiliation(s)
- Pawel Kiper
- Healthcare Innovation Technology Lab, IRCCS San Camillo Hospital, 30126 Venice, Italy
| | - Manon Chevrot
- Department of Health, LUNEX International University of Health Exercise and Sports, L-4671 Differdange, Luxembourg
| | - Julie Godart
- Department of Health, LUNEX International University of Health Exercise and Sports, L-4671 Differdange, Luxembourg
| | - Błażej Cieślik
- Healthcare Innovation Technology Lab, IRCCS San Camillo Hospital, 30126 Venice, Italy
| | | | - Martina Regazzetti
- Healthcare Innovation Technology Lab, IRCCS San Camillo Hospital, 30126 Venice, Italy
| | - Roberto Meroni
- Department of Health, LUNEX International University of Health Exercise and Sports, L-4671 Differdange, Luxembourg
- Luxembourg Health & Sport Sciences Research Institute ASBL, L-4671 Differdange, Luxembourg
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14
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Ghosh R, León-Ruiz M, Mondal A, Dubey S, Benito-León J. Guillain-Barré syndrome following acute hepatitis E virus infection: A novel Indian case report with acute inflammatory demyelinating polyneuropathy pattern and anti-GM1 antibodies. NEUROLOGY PERSPECTIVES 2025; 5:100187. [PMID: 40256458 PMCID: PMC12006973 DOI: 10.1016/j.neurop.2025.100187] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Grants] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Indexed: 04/22/2025]
Affiliation(s)
- R. Ghosh
- Department of General Medicine, Burdwan Medical College and Hospital, Burdwan, West Bengal, India
| | - M. León-Ruiz
- Section of Clinical Neurophysiology, Department of Neurology, La Paz University Hospital, Madrid, Spain
| | - A.S. Mondal
- Department of Neuromedicine, Sammilani Medical College and Hospital, Bankura, West Bengal, India
| | - S. Dubey
- Department of Neuromedicine, Bangur Institute of Neurosciences, Kolkata, West Bengal, India
| | - J. Benito-León
- Department of Neurology, 12 de Octubre University Hospital, Madrid, Spain
- Instituto de Investigación Sanitaria Hospital 12 de Octubre (imas12), Madrid, Spain
- Centro de Investigación Biomédica en Red Sobre Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain
- Department of Medicine, Complutense University, Madrid, Spain
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15
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Chen LM, Wang J, Peng XL, Fan MX, Peng HL, Hu Y. Clinical Features and Prognostic Factors of Pediatric Guillain-Barré Syndrome With Anti-Sulfatide Antibody. Pediatr Neurol 2025; 165:31-39. [PMID: 39938234 DOI: 10.1016/j.pediatrneurol.2025.01.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/26/2024] [Revised: 01/04/2025] [Accepted: 01/14/2025] [Indexed: 02/14/2025]
Abstract
BACKGROUND The study analyzed the clinical features and risk factors for poor prognosis in children with Guillain-Barré syndrome (GBS) spectrum disorders positive for anti-sulfatide antibodies. METHODS Clinical and follow-up data of 43 children diagnosed with GBS spectrum disorders positive for serum and/or cerebrospinal fluid anti-sulfatide antibodies and treated at the Children's Hospital of Chongqing Medical University between July 2018 and April 2023 were analyzed. A 1:1 matching was performed for a comparative analysis of clinical features. RESULTS Respiratory tract prodromal infection was common in the positive anti-sulfatide antibody group (53.4%, 23 of 43). The main presenting symptoms were limb weakness (67.4%, 29 of 43), pain (67.4%, 29 of 43), ataxia (32.5%, 14 of 43), and cranial nerve involvement (62.8%, 27 of 43). The clinical classification was predominantly classical GBS (76.7%, 33 of 43), with a high prevalence of acute inflammatory demyelinating polyneuropathy (41.2%, 20 of 33). Brainstem and medulla lesions were the main cranial magnetic resonance imaging (MRI) findings (16.7%, six of 36), and spinal cord MRI (32.5%, 14 of 34) showed cauda equina or partial nerve root enhancement. The following features showed a significant difference in prevalence between the anti-sulfatide-antibody-positive and -negative groups: gender, cranial nerve involvement, nerve root tension sign, abnormal brain MRI, GBS disability score (GBS-DS) at discharge, difference in GBS-DS between admission and discharge, and GBS-DS at one-month follow-up. Shorter time to peak was identified as an independent risk factor for poor short-term prognosis in GBS spectrum disorders with positive anti-sulfatide antibodies. CONCLUSIONS GBS spectrum disorders with positive anti-sulfatide antibodies have a relatively specific clinical phenotype. Shorter time to peak was an independent risk factor for poor prognosis.
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Affiliation(s)
- La-Mei Chen
- Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Child Neurodevelopment and Cognition Disorders, Chongqing, China
| | - Juan Wang
- Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Child Neurodevelopment and Cognition Disorders, Chongqing, China
| | - Xiao-Ling Peng
- Guangdong Provincial Key Laboratory of Interdisciplinary Research and Application for Data Science, BNU-HKBU United International College, Zhuhai, China
| | - Ming-Xuan Fan
- Guangdong Provincial Key Laboratory of Interdisciplinary Research and Application for Data Science, BNU-HKBU United International College, Zhuhai, China
| | - Hai-Lun Peng
- Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Child Neurodevelopment and Cognition Disorders, Chongqing, China
| | - Yue Hu
- Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Child Neurodevelopment and Cognition Disorders, Chongqing, China.
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16
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Khatami SS, Ghorbani Shirkouhi S, Høilund-Carlsen PF, Revheim ME, Alavi A, Blaabjerg M, Andalib S. Peripheral Nervous System Complications after COVID-19 Vaccination. J Integr Neurosci 2025; 24:26632. [PMID: 40152570 DOI: 10.31083/jin26632] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/03/2024] [Revised: 11/14/2024] [Accepted: 12/04/2024] [Indexed: 03/29/2025] Open
Abstract
While vaccination against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) remains crucial, neurological complications have been detected following the coronavirus disease 2019 (COVID-19) vaccination. The neurological complications of COVID-19 vaccination can be seen in both the central nervous system (CNS) and the peripheral nervous system (PNS). In this study, we reviewed PNS complications after COVID-19 vaccination, their underlying mechanisms, diagnosis, and management. Inflammatory polyneuropathy, small fiber neuropathy, Parsonage-Turner syndrome (PTS), cranial mononeuropathies, and myasthenia gravis (MG) have been reported following COVID-19 vaccination. Inflammatory polyneuropathy following COVID-19 vaccination should be diagnosed early based on clinical presentation and treated with supportive care, and immunoglobulin or plasmapheresis to prevent respiratory distress if required. It is important to differentiate peripheral from central facial paralysis after COVID-19 vaccination to rule out upper motor neuron damage, including stroke. Diagnosis of small fiber neuropathy in the setting of COVID-19 vaccination should be suspected in patients with dysesthesia, dysautonomia, and lower extremity paresthesia. A skin biopsy of the proximal or distal lower limb should generally be considered for diagnosing small fiber neuropathy following COVID-19 vaccination. Even though pain at the injection site is one of the most common symptoms after COVID-19 vaccination, shoulder pain lasting more than 3 weeks should raise the suspicion of severe complications such as PTS. In addition to a proper physical examination as a reliable diagnosis tool, needle electromyography can be considered to help the diagnosis of PTS following COVID-19 vaccination. In our opinion, despite complications after COVID-19 vaccination, the benefit of vaccination immunity should not be forgotten.
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Affiliation(s)
| | - Samaneh Ghorbani Shirkouhi
- Student Research Committee, School of Medicine, Shahroud University of Medical Sciences, 3614773943 Shahroud, Iran
| | - Poul Flemming Høilund-Carlsen
- Department of Nuclear Medicine, Odense University Hospital, University of Southern Denmark, 5000 Odense, Denmark
- Department of Clinical Research, Faculty of Health Sciences, University of Southern Denmark, 5230 Odense, Denmark
| | - Mona-Elisabeth Revheim
- The Intervention Center, Division of Technology and Innovation, Oslo University Hospital, 0450 Oslo, Norway
- Institute of Clinical Medicine, University of Oslo, 0313 Oslo, Norway
| | - Abass Alavi
- Department of Radiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Morten Blaabjerg
- Research Unit of Neurology, Department of Clinical Research, Faculty of Health Sciences, University of Southern Denmark, 5230 Odense, Denmark
- Department of Neurology, Odense University Hospital, 5000 Odense, Denmark
| | - Sasan Andalib
- Research Unit of Neurology, Department of Clinical Research, Faculty of Health Sciences, University of Southern Denmark, 5230 Odense, Denmark
- Department of Neurology, Odense University Hospital, 5000 Odense, Denmark
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17
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Ye L, Yue WR, Shi H, Li JR, Qun YY. Case Report: Successful immune checkpoint inhibitor rechallenge after sintilimab-induced Guillain-Barré syndrome. Front Immunol 2025; 16:1546886. [PMID: 40176803 PMCID: PMC11961408 DOI: 10.3389/fimmu.2025.1546886] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/17/2024] [Accepted: 02/27/2025] [Indexed: 04/04/2025] Open
Abstract
Immune checkpoint inhibitors (ICIs) have revolutionized hepatocellular carcinoma (HCC) treatment, while immune-related adverse events (IRAEs) pose significant challenges. We report a 60-year-old male with unresectable HCC who developed Guillain-Barré syndrome (GBS), a rare but severe neurologic complication, after three cycles of sintilimab plus bevacizumab biosimilar and conventional transarterial chemoembolization (c-TACE). The patient presented with progressive ascending weakness, reaching symmetric quadriparesis with proximal muscle strength of 2/5 in upper limbs and 1/5 in lower limbs. Following sintilimab discontinuation, treatment with intravenous immunoglobulin (2 g/kg) and oral prednisone (30 mg/day) achieved complete neurological recovery within one month. Given the patient's favorable initial tumor response and strong request, immunotherapy was cautiously reinstated using tislelizumab after thorough clinical evaluation. Following four cycles of treatment, significant tumor response enabled successful conversion surgery with major pathological response (necrosis rate >70%). With 26-month survival and no evidence of recurrence, this case demonstrates the potential feasibility of ICI rechallenge with an alternative PD-1 inhibitor following sintilimab-induced GBS. Our experience suggests that ICI-related neurological adverse events may be drug-specific rather than class-specific, potentially providing valuable treatment options for patients showing favorable tumor response despite experiencing severe IRAEs, though larger studies are needed for validation.
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Affiliation(s)
- Lin Ye
- Department of Hepatobiliary and Pancreatic Surgery, Affiliated Hospital of Guilin Medical University, Guilin, China
| | - Wan Rong Yue
- Department of Pathology, Guilin People's Hospital, Guilin, China
| | - Hao Shi
- Department of Hepatobiliary and Pancreatic Surgery, Affiliated Hospital of Guilin Medical University, Guilin, China
| | - Jian Ren Li
- Department of Hepatobiliary and Pancreatic Surgery, Affiliated Hospital of Guilin Medical University, Guilin, China
| | - Yu Ya Qun
- Department of Hepatobiliary and Pancreatic Surgery, Affiliated Hospital of Guilin Medical University, Guilin, China
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18
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Kang X, Wang G, Liu B, Wang Z. Knowledge mapping of Guillain-Barré syndrome from January 2013 to October 2023: A bibliometric analysis. Medicine (Baltimore) 2025; 104:e41830. [PMID: 40101082 PMCID: PMC11922404 DOI: 10.1097/md.0000000000041830] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2025] [Accepted: 02/21/2025] [Indexed: 03/20/2025] Open
Abstract
BACKGROUND With the COVID-19 pandemic and the serious sequelae, foreign factor-induced Guillain-Barré syndrome (GBS) has become a research focus in autoimmune peripheral neuropathies. The study employs a bibliometric system to illustrate the research hotspots and trends in GBS based on pertinent literature from January 2013 to October 2023. METHODS The Web of Science Core Collection retrieved articles on GBS from January 1, 2013, to October 28, 2023. These articles were then visualized and statistically evaluated using VOSviewer, CiteSpace software, R version 4.2.1, and Microsoft Office Excel 2019. RESULTS A total of 4269 articles on GBS were gathered. The United States of America produced the most publications (28.55%, 1219/4269), followed by China (14.22%, 607/4269). The world's leading country was the United States of America, with the most publications, the most substantial international cooperation, and the highest centrality (0.17). Union of French Research Universities (UDICE)-French Research Universities in France was the most productive organization (189 articles). Lancet was the highest cocited journal (2428), and Professor Jacobs, Bart C., was the most prolific author (93). The most significant increases were shown for the keywords coronavirus, respiratory failure, and coronavirus disease 2019. The novel coronavirus is an emerging virus that may cause GBS, indicating a promising area of research. CONCLUSIONS The study on GBS was illustrated using bibliometrics, and it covers trends in international collaboration, publications, and research hotspots. These findings allow the scientific community to pinpoint the novel ideas and directions that will drive future GBS research.
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Affiliation(s)
- Xue Kang
- School of Clinical Medicine, Ningxia Medical University, Yinchuan, China
| | - Guowei Wang
- NHC Key Laboratory of Diagnosis and Treatment on Brain Functional Diseases, Chongqing, China
- The First Affiliated Hospital of Chongqing Medical University, Chongqing, China
| | - Bin Liu
- College of Biology, Hunan University, Changsha, China
| | - Zhenhai Wang
- Institute of Medical Sciences, General Hospital of Ningxia Medical University, Yinchuan, China
- Diagnosis and Treatment Engineering Technology Research Center of Nervous System Diseases of Ningxia Hui Autonomous Region, Yinchuan, China
- Neurology Center, General Hospital of Ningxia Medical University, Yinchuan, China
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19
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Aziz H, Kanwar D. Gullian Barre syndrome secondary to heat stroke: a case report. Intern Emerg Med 2025; 20:523-528. [PMID: 39392537 DOI: 10.1007/s11739-024-03793-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/01/2024] [Accepted: 10/04/2024] [Indexed: 10/12/2024]
Abstract
Heat waves are increasingly common in subcontinent countries, including Pakistan. Heat stroke (HS) is a critical condition resulting from extreme temperatures, often culminating in multiorgan dysfunction. Clinical manifestations of heat-related emergencies can include altered mentation, fever, and profound weakness. We present a compelling case of a previously healthy 49-year-old male who developed altered mentation, fever, and acute kidney injury following intense physical exertion amidst a severe heat wave. Although initially managed as heat stroke, the patient soon exhibited dysarthria, palatal weakness, and bilateral lower limb weakness with areflexia, prompting a detailed neurological assessment. Electromyography and nerve conduction studies revealed the acute motor-sensory axonal neuropathy (AMSAN) variant of Guillain-Barré Syndrome (GBS), an uncommon but severe sequel of heat stroke. GBS, an autoimmune disorder, typically presents with ascending bilateral lower limb flaccid paralysis, often following an acute infectious trigger. Treatment with plasmapheresis led to remarkable neurological recovery. This case represents the first documented instance of heat stroke-induced GBS in Pakistan, highlighting both the distinctive clinical features and the therapeutic complexities of this rare condition.
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Affiliation(s)
- Hassan Aziz
- Internal Medicine Resident PGY 02, The Indus Hospital Karachi, Karachi, Pakistan.
- Internal Medicine, The Indus Hospital Karachi, Karachi, Pakistan.
| | - Dureshahwar Kanwar
- FCPS Neurology, Diplomate American Board of Neurophysiology, Assistant Professor, Section of Neurology, Department of Medicine, Clinical Neurophysiology Lab, Aga Khan University Hospital, Karachi, Pakistan
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20
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Sahu M, Praharaj D, Bhadoria AS. Vaccination Strategies for a Liver Transplant Recipient. J Clin Exp Hepatol 2025; 15:102421. [PMID: 39588050 PMCID: PMC11585777 DOI: 10.1016/j.jceh.2024.102421] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2023] [Accepted: 09/29/2024] [Indexed: 11/27/2024] Open
Abstract
Patients with cirrhosis and liver transplant recipients are at increased risk of infections. Malnutrition, multiple hospital admissions, immune dysfunction related to cirrhosis, and immunosuppressive agents used for liver transplantation predispose the recipient to various life-threatening infections. Some of these infections are preventable with vaccines. With the COVID-19 pandemic, there has been an accelerated research in vaccination technology and platforms, which in turn may also improve awareness of physicians regarding this healthy and often ignored aspect of management of patients with cirrhosis and transplant recipients. The organ transplant candidates should complete the recommended vaccination schedule as early as possible (especially patients with compensated cirrhosis) or at least during their pretransplant work-up so as to prevent or reduce the severity of various infections.
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Affiliation(s)
- Monalisa Sahu
- Department of Infectious Diseases, Yashoda Hospitals, Hyderabad, India
| | - Dibyalochan Praharaj
- Department of Gastroenterology, Kalinga Institute of Medical Sciences, Bhubaneswar, India
| | - Ajeet S. Bhadoria
- Department of Community and Family Medicine, All India Institute of Medical Sciences, Rishikesh, India
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21
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Gabay Moreira D. Spinal Cord Infarction Presenting with Abnormal F Waves. Neurodiagn J 2025; 65:64-75. [PMID: 39913861 DOI: 10.1080/21646821.2024.2448359] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/19/2024] [Accepted: 12/26/2024] [Indexed: 03/14/2025]
Abstract
A 42-year-old female presented with acute onset of asymmetric lower extremity weakness, bilateral lower extremity areflexia and sensory loss, and urinary incontinence. Initial investigation with a spine MRI for a spinal cord process was negative, leading to further investigation with nerve conduction studies/electromyography (NCS/EMG). This revealed absent F waves bilaterally in the fibular nerves, and there was only one isolated F wave with decreased amplitude and increased latency in the left tibial nerve and had lack of heterogeneity in the right tibial nerve. Given the early presentation of her symptoms, this led to a working diagnosis of Guillain-Barré Syndrome, which was treated with plasmapheresis without improvement. After plasmapheresis, the diagnosis was revisited, and a repeat spine MRI revealed an extensive lower thoracic and lumbar spinal cord infarction. In this unique scenario, the properties and abnormalities of F waves are discussed and reviewed, along with the influence of acute central nervous system lesions on their configuration.
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Affiliation(s)
- Daniel Gabay Moreira
- Department of Neurology, University of Connecticut School of Medicine, Farmington, Connecticut, USA
- Department of Neurology, Hartford Hospital, Hartford, Connecticut, USA
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22
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Refai S, Mattar A, Patel N, Hull SD, Virgilio R. An Atypical Case of Miller Fisher Syndrome: Classic Clinical Triad Despite Negative Anti-GQ1b Antibody. Cureus 2025; 17:e80529. [PMID: 40225479 PMCID: PMC11993312 DOI: 10.7759/cureus.80529] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/22/2024] [Accepted: 03/13/2025] [Indexed: 04/15/2025] Open
Abstract
Miller Fisher syndrome (MFS), a variant of Guillain-Barré syndrome (GBS), was diagnosed in a 30-year-old patient following a mononucleosis infection. The progression of this patient's disease began with the classic triad of symptoms (areflexia, ophthalmoplegia, and ataxia) beginning at the head and progressing downward. Most notably, the diaphragm was spared. Ophthalmoplegia, ataxia, and areflexia were among the most defining of symptoms; however, this patient also presented with restlessness in his extremities and urinary hesitancy. Diagnostic workup of this patient included testing for anti-GQ1B antibodies in addition to a thorough history and physical exam. Initial treatment with intravenous immunoglobulin (IVIG) was unsuccessful. Plasmapheresis was ultimately initiated; however, the patient failed to complete treatment due to iatrogenic complications. The patient failed to return to follow-up appointments to determine recovery status. This case underscores the critical importance of a thorough and systematic diagnostic approach in managing atypical presentations of GBS variants.
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Affiliation(s)
- Sami Refai
- Internal Medicine, Edward Via College of Osteopathic Medicine, Auburn, USA
| | - Ammar Mattar
- Internal Medicine, Edward Via College of Osteopathic Medicine, Auburn, USA
| | - Neel Patel
- Internal Medicine, Edward Via College of Osteopathic Medicine, Auburn, USA
| | - Stephen D Hull
- Internal Medicine, Grandview Medical Center, Birmingham, USA
| | - Richard Virgilio
- Clinical Affairs, Edward Via College of Osteopathic Medicine, Auburn, USA
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23
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Lima MFDO, Nogueira VB, Maury W, Wilson ME, Júnior METD, Teixeira DG, Bezerra Jeronimo SM. Altered Cellular Pathways in the Blood of Patients With Guillain-Barre Syndrome. J Peripher Nerv Syst 2025; 30:e70012. [PMID: 40099626 DOI: 10.1111/jns.70012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/24/2024] [Revised: 02/03/2025] [Accepted: 03/03/2025] [Indexed: 03/20/2025]
Abstract
BACKGROUND AND AIMS Guillain-Barré syndrome (GBS) is a rare disorder, with a global incidence ranging from 1 to 2 individuals per 100,000 people/year. Infections and vaccines have been implicated as causes triggering GBS. The aim of the study was to identify host genes involved in the pathogenesis of GBS when Zika (ZIKV) and Chikungunya viruses (CHIKV) were introduced in Brazil. METHODS A case-control study of GBS was performed when ZIKV and CHIKV were introduced into a naïve population. GBS was studied during both acute and postacute phases. RNA sequencing was conducted using whole blood. RESULTS GBS typically manifested a week after rash and fever; acute inflammatory demyelinating polyradiculoneuropathy was more frequent. None of the GBS cases had a poor outcome. Serological assays for ZIKV and CHIKV revealed high titers of immunoglobulin G for both viruses in 9 out of 11 subjects. Metatranscriptomic analyses unveiled an increased abundance of reads attributed to Pseudomonas tolaasii and Toxoplasma gondii in the acute phase. Analysis of differentially expressed host genes during the acute phase revealed altered expression of genes associated with axogenesis, synapse assembly, and presynapse organization. Moreover, genes upregulated during acute GBS were primarily related to inflammation and the inflammasome pathways, including AIM2, NLR family genes and LRR-protein genes, and IL-10. INTERPRETATION These findings suggest that inflammasome activation via AIM2 could play a role in tissue damage during GBS. Further investigation into the general activation of innate inflammatory responses is warranted to elucidate their potential contribution to the pathology of GBS.
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Affiliation(s)
| | - Viviane Brito Nogueira
- Graduate Program in Health Sciences, Health Sciences Center, Federal University of Rio Grande do Norte, Natal, Brazil
| | - Wendy Maury
- Department of Microbiology and Immunology, University of Iowa, Iowa City, Iowa, USA
| | - Mary Edythe Wilson
- Department of Microbiology and Immunology, University of Iowa, Iowa City, Iowa, USA
- Department of Internal Medicine, University of Iowa, Iowa City, Iowa, USA
- Veterans' Affairs Medical Center, Iowa City, Iowa, USA
| | - Mário Emílio Teixeira Dourado Júnior
- Institute of Tropical Medicine of Rio Grande do Norte, Federal University of Rio Grande do Norte, Natal, Brazil
- Onofre Lopes University Hospital and Department of Integrative Medicine, Federal University of Rio Grande do Norte, Natal, Rio Grande do Norte, Brazil
| | - Diego Gomes Teixeira
- Institute of Tropical Medicine of Rio Grande do Norte, Federal University of Rio Grande do Norte, Natal, Brazil
| | - Selma Maria Bezerra Jeronimo
- Graduate Program in Health Sciences, Health Sciences Center, Federal University of Rio Grande do Norte, Natal, Brazil
- Institute of Tropical Medicine of Rio Grande do Norte, Federal University of Rio Grande do Norte, Natal, Brazil
- Department of Biochemistry, Biosciences Center, Federal University of Rio Grande do Norte, Natal, Rio Grande do Norte, Brazil
- Institute of Science and Technology of Tropical Diseases, Natal, Rio Grande do Norte, Brazil
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Chen S, Ou R, Wei Q, Zhao B, Chen X. Sequential administration of efgartigimod shortened the course of Guillain-Barré syndrome: a case series. Ther Adv Neurol Disord 2025; 18:17562864251314746. [PMID: 40012687 PMCID: PMC11863258 DOI: 10.1177/17562864251314746] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/20/2024] [Accepted: 01/06/2025] [Indexed: 02/28/2025] Open
Abstract
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.
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Affiliation(s)
- Sihui Chen
- Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Ruwei Ou
- Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Qianqian Wei
- Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Bi Zhao
- Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Xueping Chen
- Department of Neurology, West China Hospital, Sichuan University, No. 37 Guoxue Road, Chengdu, Sichuan 610041, China
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Guo F, Yao QY, Wu XH, Guo HX, Su XL, Zhou JF, Ren LJ, Zhou YX. Clinical characteristics of Guillain-Barré syndrome in Shenzhen: a retrospective study. BMC Neurol 2025; 25:72. [PMID: 39984908 PMCID: PMC11844154 DOI: 10.1186/s12883-025-04061-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/23/2024] [Accepted: 01/28/2025] [Indexed: 02/23/2025] Open
Abstract
BACKGROUND Guillain-Barré syndrome (GBS) is a group of immune-mediated peripheral neuropathies that causes acute flaccid paralysis. The global incidence of GBS was 0.6-4/100 000, and the incidence in China was 0.698/100 000. Although the diagnosis and treatment of GBS has made rapid progress, approximately 20% of patients with GBS are still unable to walk alone within 6 months after the onset of GBS, and 40% of patients have sequelae, such as weakened strength, limb pain, and numbness, seriously affecting their life and work. We aimed to retrospectively analyze the clinical characteristics of patients with GBS in Shenzhen, China and analyze the factors affecting disease severity to provide a reference for the precise treatment of GBS. METHODS Clinical data of inpatients diagnosed with GBS in several hospitals in Shenzhen from April 2010 to October 2021 were obtained from an electronic medical record system (HIS system). The clinical characteristics of patients with GBS and the factors affecting disease severity were analyzed. RESULTS A total of 146 patients were identified for this study, and 13 were lost during follow-up. During the follow-up period, three patients had acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP). One hundred and thirty patients with complete data, comprising 90 males (69.23%) and 40 females (30.77%) with a median age of 39.50 ± 23.75 years, were included in the statistical analyses. Acute inflammatory demyelinating polyneuropathy (AIDP) was the most common electrophysiological variant (106 cases [81.54%]). Miller-Fisher syndrome (MFS), acute motor axonal neuropathy (AMAN), and acute motor-sensory axonal neuropathy (AMSAN) were noted in 21 (16.15%), 2 (1.54%), and 1 (0.77%) patients, respectively. The clinical course of the disease was mainly mild in 95 cases (73.08%), while 35 patients (26.92%) experienced severe disease. Logistic multivariate regression analysis showed that age ≥ 60 years old and having pneumonia may be associated with the severity of the disease. CONCLUSIONS AIDP is the most common electrophysiological variant of GBS in Shenzhen. Most cases of GBS in our setting are mild, and the long-term prognosis is favorable. Old age (≥ 60 years) and having pneumonia are independent risk factors for severe GBS.
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Affiliation(s)
- Feng Guo
- Shenzhen Guangming District People's Hospital, Guangdong Shenzhen, 518000, China
| | - Qin-Yuan Yao
- Community Primary Medical Management, Department of The Second Affiliated Hospital, School of Medicine, The Chinese University of Hong Kong, Longgang District People's Hospital of Shenzhen, Guangdong Shenzhen, 518172, China
| | - Xiao-Hong Wu
- The First Affiliated Hospital of Shenzhen University, The Second Hospital of Shenzhen, Guangdong Shenzhen, 51800, China
| | - Hui-Xia Guo
- Peking University Shenzhen Hospital, Guangdong Shenzhen, 51800, China
| | - Xiao-Lin Su
- Shenzhen People's Hospital, Guangdong Shenzhen, 51800, China
| | - Jin-Fang Zhou
- LongGang District Central Hospital of Shenzhen, Guangdong Shenzhen, 518000, China
| | - Li-Jie Ren
- The First Affiliated Hospital of Shenzhen University, The Second Hospital of Shenzhen, Guangdong Shenzhen, 51800, China.
| | - Yan-Xia Zhou
- The First Affiliated Hospital of Shenzhen University, The Second Hospital of Shenzhen, Guangdong Shenzhen, 51800, China.
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Basch RE, Tierney M, Worden L, Sanders S, Ng EA. EBV and Concomitant Acute Motor and Sensory Axonal Neuropathy in a Healthy 15-Year-Old Female. Neuropediatrics 2025. [PMID: 39904368 DOI: 10.1055/a-2532-4172] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2025]
Abstract
Acute motor and sensory axonal neuropathy (AMSAN) is a rare and severe form of acute axonal injury caused by immune damage to the axonal membrane. AMSAN is an axonal variant of GBS. GBS occurs from immune injury to the myelin sheath, axonal variants of GBS (AMSAN and AMAN) differ in that insult is to the axonal membrane. AMSAN is seldom seen, especially in pediatric and adolescent patients. Unlike acute motor axonal neuropathy (AMAN), which has been well-described in literature to be secondary to Campylobacter jejuni infection, there is no known etiology of AMSAN. Here, we present a case of an otherwise healthy 15-year-old female who presented with new-onset facial and bulbar weakness that rapidly progressed to functional paralysis requiring intubation. With no clear diagnosis and after failure in improvement with high-dose steroids, Intravenous Immunoglobulin (IVIG), and plasma exchange transfusion, diagnosis was finally made with electromyography (EMG) and nerve conduction study (NCS). In addition, extensive laboratory work was completed and was only notable for primary acute EBV infection. This case represents a new presenting symptom of AMSAN, a unique finding of concomitant primary EBV infection, the possibility of primary Epstein-Barr virus (EBV) infection as the triggering event in AMSAN and stresses the importance of EMG and NCS when evaluating patients with weakness.
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Affiliation(s)
- Rebecca E Basch
- Division of General Pediatrics, Connecticut Children's, Hartford, Connecticut, United States
- Department of Pediatrics, University of Connecticut School of Medicine, Farmington, Connecticut, United States
| | - McKenna Tierney
- Division of General Pediatrics, Connecticut Children's, Hartford, Connecticut, United States
- Department of Pediatrics, University of Connecticut School of Medicine, Farmington, Connecticut, United States
| | - Lila Worden
- Department of Pediatrics, University of Connecticut School of Medicine, Farmington, Connecticut, United States
- Division of Neurology, Connecticut Children's, Hartford, Connecticut, United States
| | - Sara Sanders
- Department of Pediatrics, University of Connecticut School of Medicine, Farmington, Connecticut, United States
- Division of Critical Care, Connecticut Children's, Hartford, Connecticut, United States
| | - Elizabeth A Ng
- Department of Pediatrics, University of Connecticut School of Medicine, Farmington, Connecticut, United States
- Division of Neurology, Connecticut Children's, Hartford, Connecticut, United States
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27
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Sohutskay DO, Goodwin JD, Smith KM. 51-Year-Old Woman With Bilateral Facial Palsy and Lower Extremity Weakness. Mayo Clin Proc 2025; 100:346-351. [PMID: 39755967 DOI: 10.1016/j.mayocp.2024.03.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/20/2024] [Revised: 02/28/2024] [Accepted: 03/01/2024] [Indexed: 01/07/2025]
Affiliation(s)
- David O Sohutskay
- Resident in Internal Medicine, Mayo Clinic School of Graduate Medical Education, Rochester, MN
| | - Jacob D Goodwin
- Resident in Internal Medicine, Mayo Clinic School of Graduate Medical Education, Rochester, MN
| | - Kelsey M Smith
- Advisor to residents and Consultant in Neurology, Mayo Clinic, Rochester, MN.
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28
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Almeida MP, Winck JC, Marques A. InspireGBs - Inspiratory muscle training in people with Guillain-Barré syndrome: A feasibility study. PM R 2025; 17:187-194. [PMID: 39140186 DOI: 10.1002/pmrj.13251] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2023] [Revised: 05/20/2024] [Accepted: 06/13/2024] [Indexed: 08/15/2024]
Abstract
BACKGROUND Guillain-Barré syndrome (GBS) is a rare immune-mediated peripheral nerve disease often preceded by infections. Respiratory muscle weakness is a common complication in this population, leading to decreased vital capacity, weakened coughing ability, atelectasis, and pulmonary infections. Inspiratory muscle training (IMT) has been widely used to enhance inspiratory muscle strength and pulmonary function in various diseases; however, its application in GBS is unknown. OBJECTIVE To assess the safety, feasibility, and preliminary effectiveness of an IMT protocol-InspireGBs-in people with GBS. METHODS A pre/post feasibility study was conducted. Feasibility was determined by participant recruitment/retention, adherence, time spent in each session, and adverse events. Secondary outcome was inspiratory muscle strength. InspireGBs consisted of twice daily sessions 5 times/week, three sets of 10 breaths in each session, for 6 weeks. Initial resistance was set at 50% of participant's baseline maximal inspiratory pressure (MIP) and with a weekly increase of 10% calculated from the previous week's training intensity, if tolerated, otherwise the increase was 5%. RESULTS Eleven patients (63% male; 63 ± 9 years) were recruited from inpatient rehabilitation and 10 completed the study. Recruitment and retention rates were high (79% and 91%, respectively). Excellent adherence rate (96%) was obtained with no reported adverse effects or safety issues. Sessions lasted from 4 to 6 minutes. The MIP improved (pretraining: 39 [26.5-50] cm H2O vs. posttraining: 61 [56.3-64.5] cm H2O, p = .005 and pretraining: 38 [30.5-53.8] % of predicted vs. posttraining: 60 [54.28-71.58] % of predicted, p = .009) with the InspireGBs. CONCLUSION InspireGBs is safe, feasible, and may be effective in improving inspiratory muscle strength in individuals with GBS. A randomized controlled trial is now needed to strengthen these findings.
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Affiliation(s)
- Miguel P Almeida
- North Rehabilitation Centre, Local Health Unit Gaia e Espinho, Vila Nova de Gaia, Portugal
- Lab3R - Respiratory Research and Rehabilitation Laboratory, School of Health Sciences and Institute of Biomedicine (iBiMED), University of Aveiro, Aveiro, Portugal
| | | | - Alda Marques
- Lab3R - Respiratory Research and Rehabilitation Laboratory, School of Health Sciences and Institute of Biomedicine (iBiMED), University of Aveiro, Aveiro, Portugal
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29
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Li Z, Huang F, Hao S. Guillain-Barré syndrome in patients with multiple myeloma: three cases report and literature review. BMC Neurol 2025; 25:36. [PMID: 39856633 PMCID: PMC11760682 DOI: 10.1186/s12883-025-04045-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2024] [Accepted: 01/16/2025] [Indexed: 01/27/2025] Open
Abstract
BACKGROUND Multiple myeloma (MM) with Guillain-Barré syndrome (GBS) is relatively rare, and the specific mechanism is still unclear. The previous infection, surgery, and medication use may have contributed to the occurrence of GBS. The use of bortezomib in patients with MM can easily lead to peripheral neuropathy, which is similar to the symptoms of GBS, making it challenging to diagnose GBS. CASES PRESENTATION Three patients with IgA type MM experienced lower limb weakness during treatment. Combined with lumbar puncture, nerve conduction studies, and other tests, the diagnosis was confirmed as GBS. All three patients had a history of spinal surgery before the onset of GBS, and had been treated with bortezomib which induced peripheral neuropathy. Two of the three patients had a clear history of upper respiratory tract infection before the onset of GBS. After treatment with intravenous immunoglobulin, one patient died and two patients showed improvement in GBS symptoms. CONCLUSION Patients with MM often have concurrent infections and spinal surgery, which may contribute to the occurrence of GBS. The symptoms of bortezomib-induce peripheral neuropathy overlap with those of GBS, which can easily lead to misdiagnosis or missed diagnosis of GBS. Timely lumbar puncture and nerve conduction studies may help to diagnose GBS and improve the prognosis.
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Affiliation(s)
- Zhichao Li
- Department of Hematology, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China
| | - Fang Huang
- Department of Hematology, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China
| | - Siguo Hao
- Department of Hematology, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China.
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30
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Hassan IN, Yaqub S, Ibrahim M, Aljaili G, Abuassa N. A rare neurological complication of dengue: Guillain-Barré Syndrome in a dengue fever patient. IDCases 2025; 39:e02160. [PMID: 39911475 PMCID: PMC11795545 DOI: 10.1016/j.idcr.2025.e02160] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/11/2024] [Accepted: 01/15/2025] [Indexed: 02/07/2025] Open
Abstract
Background Dengue fever is a common viral illness in tropical and subtropical regions, typically presenting with fever, myalgia, and hemorrhagic symptoms. While neurological complications are rare, Guillain-Barré Syndrome (GBS) is a known but uncommon sequelae of viral infections, including dengue. Case presentation We report the case of a previously healthy 32-year-old male who developed acute flaccid paralysis secondary to GBS following a dengue fever infection. The patient initially presented with classic dengue symptoms-fever, severe headache, retro-orbital pain, and myalgia. Following resolution of the acute dengue phase, he developed ascending muscle weakness, areflexia, and numbness in both legs. Electromyography confirmed a diagnosis of GBS, and cerebrospinal fluid analysis revealed albuminocytologic dissociation. The patient was treated with intravenous immunoglobulin (IVIG), leading to significant clinical improvement, with gradual recovery of motor function. Discussion This case highlights the rare but significant neurological complication of GBS following dengue fever. Clinicians should maintain a high index of suspicion for GBS in patients recovering from dengue, especially in endemic regions. Early diagnosis and treatment with IVIG or plasmapheresis are critical for improving outcomes in these patients. Conclusion Dengue fever can lead to severe neurological sequelae such as GBS, and early recognition and intervention can prevent long-term disability. This case underscores the need for vigilance in identifying such complications in patients recovering from viral infections.
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Affiliation(s)
| | - Siddig Yaqub
- Karary University, Faculty of Medicine, Khartoum, Sudan
| | | | - Ghada Aljaili
- Omdurman Islamic University, Faculty of Medicine, Khartoum, Sudan
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31
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Jung S, Lee HG, Kwon S, Cho SY, Park SU, Jung WS, Moon SK, Park JM, Ko CN. Traditional herbal medicine for Guillain-Barré syndrome: A systematic review and meta-analysis. Heliyon 2025; 11:e41455. [PMID: 39850433 PMCID: PMC11754176 DOI: 10.1016/j.heliyon.2024.e41455] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/02/2024] [Revised: 12/18/2024] [Accepted: 12/23/2024] [Indexed: 01/25/2025] Open
Abstract
Background Guillain-Barré syndrome (GBS) is a rapid-onset disease caused by the immune system damaging the peripheral nervous system. Since most standardized treatments for GBS focus on acute phase treatment, there are limitations to the rehabilitation and management of general conditions. In East Asian countries, herbal medicine has been used to treat GBS and aid rehabilitation. Therefore, herbal medicine is considered a complementary treatment for GBS. Hence, the present study was conducted to investigate the clinical evidence of herbal medicine treatment for GBS and to provide a research strategy for the future. Method PubMed, Embase, Cochrane, CNKI, CiNii, and Science ON were searched from inception to December 4, 2024. Randomized controlled trials (RCTs) comparing conventional Western medicine (CWM) combined with herbal medicine (treatment group) and only CWM (control group), to evaluate the effects of herbal medicine combined with CWM as a treatment for GBS were included. All bibliographic data from the collected studies were summarized in Endnote X9 (Clarivate Analytics). The meta-analysis was conducted using Review Manager (Revman) 5.4.1. software. Effectiveness was assessed by Total Effective Rate (TER), Modified Barthel Index (mBI) score and Manual Muscle Testing (MMT) score. Safety was evaluated as the occurrence of a significant adverse events (AEs). Results Ten RCTs that comprised 764 participants were included. Based on the meta-analysis, TER was found to significantly improve in the treatment group compared with the control group (risk ratios: 1.14, 95 % confidence interval: 1.09 to 1.20, p < 0.00001). The mBI score and MMT score of upper limb and lower limb also significantly improved in the treatment group compared with the control group. No significant AEs were reported in any included study. Conclusions The results of this study suggest that the combination of CWM and herbal medicine may be a better and safer method of physical function recovery and rehabilitation in patients with GBS. Further qualified studies are required to establish this hypothesis.
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Affiliation(s)
- Somin Jung
- Department of Clinical Korean Medicine, Graduate School, Kyung Hee University, Seoul, Republic of Korea
- Department of Cardiology and Neurology, Kyung Hee University College of Korean Medicine, Kyung Hee University Medical Center, Seoul, Republic of Korea
| | - Han-Gyul Lee
- Department of Cardiology and Neurology, Kyung Hee University College of Korean Medicine, Kyung Hee University Medical Center, Seoul, Republic of Korea
| | - Seungwon Kwon
- Department of Cardiology and Neurology, Kyung Hee University College of Korean Medicine, Kyung Hee University Medical Center, Seoul, Republic of Korea
| | - Seung-Yeon Cho
- Stroke and Neurological Disorders Center, Kyung Hee University College of Korean Medicine, Kyung Hee University Hospital at Gangdong, Seoul, Republic of Korea
| | - Seong-Uk Park
- Stroke and Neurological Disorders Center, Kyung Hee University College of Korean Medicine, Kyung Hee University Hospital at Gangdong, Seoul, Republic of Korea
| | - Woo-Sang Jung
- Department of Cardiology and Neurology, Kyung Hee University College of Korean Medicine, Kyung Hee University Medical Center, Seoul, Republic of Korea
| | - Sang-Kwan Moon
- Department of Cardiology and Neurology, Kyung Hee University College of Korean Medicine, Kyung Hee University Medical Center, Seoul, Republic of Korea
| | - Jung-Mi Park
- Stroke and Neurological Disorders Center, Kyung Hee University College of Korean Medicine, Kyung Hee University Hospital at Gangdong, Seoul, Republic of Korea
| | - Chang-Nam Ko
- Stroke and Neurological Disorders Center, Kyung Hee University College of Korean Medicine, Kyung Hee University Hospital at Gangdong, Seoul, Republic of Korea
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Hamdeh MA, Mounshar AAMAA, Asad RM, Shubietah A, Basalat NMAJ, Hassouneh JS, Al Murr MJ. Guillain-Barré Syndrome Following Laparoscopic Sleeve Gastrectomy: A Tale of Two Cases. Obes Surg 2025; 35:345-349. [PMID: 39680291 DOI: 10.1007/s11695-024-07635-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2024] [Revised: 10/23/2024] [Accepted: 12/10/2024] [Indexed: 12/17/2024]
Abstract
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.
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Affiliation(s)
- Mo'ath Abu Hamdeh
- Faculty of Medicine and Health Sciences, An-Najah National University, Nablus, Occupied Palestinian Territories.
- Department of Internal Medicine, An-Najah National University Hospital, Nablus, Occupied Palestinian Territories.
| | - Ali Abdelhai Mohammad Abdallah Abu Mounshar
- Faculty of Medicine and Health Sciences, An-Najah National University, Nablus, Occupied Palestinian Territories
- Neurology Department, Jordan University of Science and Technology, Irbid, Jordan
| | - Rasmea Mohammad Asad
- College of Medicine, Hebron University, Hebron, Occupied Palestinian Territories
| | - Abdalhakim Shubietah
- Faculty of Medicine and Health Sciences, An-Najah National University, Nablus, Occupied Palestinian Territories
- Department of Internal Medicine, An-Najah National University Hospital, Nablus, Occupied Palestinian Territories
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Chen Y, Wu C, Weng W, Wei Y. Predictors of the Short-Term Outcomes of Guillain-Barré Syndrome: Exploring Electrodiagnostic and Clinical Features. Brain Behav 2025; 15:e70257. [PMID: 39832157 PMCID: PMC11745152 DOI: 10.1002/brb3.70257] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/14/2024] [Revised: 12/13/2024] [Accepted: 12/15/2024] [Indexed: 01/22/2025] Open
Abstract
BACKGROUND AND OBJECTIVES Guillain-Barré syndrome (GBS), an acute inflammatory disorder of the peripheral nervous system, is characterized by muscle weakness and paralysis. Prompt identification of patients at a high risk of poor outcomes is crucial for timely intervention. In this study, we combined clinical data with nerve conduction study and electromyography data to identify the predictors of GBS outcomes. METHODS We retrospectively analyzed the data of patients with GBS who had received treatment at Chang Gung Memorial Hospital, Taiwan, between 1998 and 2022. Comprehensive clinical and electrophysiological data were collected. Statistical analyses were performed to identify the predictors of poor outcomes. The patients were stratified into two groups by their scores on the GBS Disability Scale: good (score ≤ 2) and poor (score > 2) outcome groups. RESULTS The study finally included 24 GBS patients (mean age: 53.0 ± 20.9 years; female-to-male ratio: 2.3; good outcome group: 13; poor outcome group: 11). Compared with the good outcome group, the poor outcome group was old (43.0 ± 20.4 vs. 64.0 ± 15.7, p = 0.011), had a short time-to-treatment period (12.9 ± 7.8 vs. 6.5 ± 5.4 days, p = 0.033), exhibited more prevalent mechanical ventilation use (0 vs. 36.4%, p = 0.017), and had a prolonged hospitalization duration (14.7 ± 10.2 vs. 53.1 ± 20.0 days, p < 0.001). Poor outcomes were associated with low compound muscle action potential (CMAP), slow motor nerve conduction velocity (MNCV), abnormal F-wave latency, and more conduction block and temporal dispersion. In the subgroup of acute inflammatory demyelinating polyradiculoneuropathy (AIDP), there were 19 patients, out of which 10 had good outcomes, while nine had poor outcomes. The clinical features that differentiate between good and poor outcomes in the AIDP subgroup were similar to those observed in all GBS patients. Notably, the motor conduction features, including distal and proximal CMAP and MNCV of the median and tibial nerves (all p < 0.05), were particularly important electrodiagnostic features of outcome discrimination in the AIDP subgroup. DISCUSSION Combining clinical data with nerve conduction study and electromyography data can assist in predicting outcomes of both GBS patients and the AIDP subgroup. Poor outcomes are associated with older age, a more abrupt onset pattern, low CMAP, and slow nerve conduction, and abnormal tibial F responses can predict poor outcomes. Early identification of high-risk patients facilitates tailored interventions. This highlights the importance of combining clinical and electrophysiological data in GBS management.
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Affiliation(s)
- Yi‐Hsiang Chen
- Department of NeurologyChang Gung Memorial HospitalKeelungTaiwan
| | - Chia‐Lun Wu
- Department of NeurologyChang Gung Memorial HospitalKeelungTaiwan
- College of MedicineChang Gung UniversityTaoyuanTaiwan
| | - Wei‐Chieh Weng
- Department of NeurologyChang Gung Memorial HospitalKeelungTaiwan
- College of MedicineChang Gung UniversityTaoyuanTaiwan
| | - Yi‐Chia Wei
- Department of NeurologyChang Gung Memorial HospitalKeelungTaiwan
- College of MedicineChang Gung UniversityTaoyuanTaiwan
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Cheng BK, Ko CA, Kuo CL, Nai TC, Lin W, Chou CH, Sung YF. Guillain-Barre Syndrome-Like Polyneuropathy Induced by Immune Checkpoint Inhibitors: A Case Report. ACTA NEUROLOGICA TAIWANICA 2025; 34:35-39. [PMID: 40396801 DOI: 10.4103/ant.ant_112_0090] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/27/2023] [Accepted: 02/19/2024] [Indexed: 05/22/2025]
Abstract
ABSTRACT Over the past decade, immune checkpoint inhibitors (ICIs) have emerged as a crucial therapeutic option for treating various malignancies. Nivolumab, an ICI, is a monoclonal antibody directed against programmed cell death protein 1. However, many studies indicate that ICIs can lead to various immune-related adverse events (irAEs), with neurological irAEs accounting for approximately 1%-5% of these cases. Neurological adverse events such as Guillain-Barre syndrome (GBS) are rare but life threatening. This report presents the case of a 72-year-old male diagnosed with sarcomatoid squamous cell carcinoma of the trachea, who was admitted with rapid-onset weakness of the upper limbs 7 days following the latest nivolumab treatment. These symptoms progressed quickly to quadriplegia and respiratory failure, requiring intubation and mechanical ventilation. Serum antiganglioside antibodies were positive for immunoglobulin (Ig) M-GM1, IgM-GD1b, and IgG-GM3. His clinical symptoms improved significantly after steroid and intravenous immunoglobulin treatment. This rare and atypical case highlights the complexity of ICI-related GBS. Early recognition of associated symptoms is crucial for timely and appropriate treatment.
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Affiliation(s)
- Bo-Kang Cheng
- Department of Neurology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan
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Kegyes D, Moisoiu V, Constantinescu C, Tanase A, Ghiaur G, Einsele H, Tomuleasa C, Lazarus HM, Gale RP. Neuro-toxicities of chemo- and immune-therapies in haematologic malignancies: from mechanism to management. Blood Rev 2025; 69:101254. [PMID: 39674687 DOI: 10.1016/j.blre.2024.101254] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2024] [Revised: 11/28/2024] [Accepted: 12/07/2024] [Indexed: 12/16/2024]
Abstract
Chemo- and immune therapies administered to treat haematologic malignancies frequently cause neurologic injury. The adverse events range from mild cognitive impairment and headaches to severe conditions such as seizures, stroke and encephalitis. We performed a comprehensive literature review and report the types, mechanisms, management and prevention of neuro-toxicity resulting from these therapies in subjects who develop these toxic effects. Our paper will not discuss radiation therapy, as it has already been extensively reviewed by many authors. Our focus will be on recently developed anti-cancer drugs.
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Affiliation(s)
- David Kegyes
- Department of Hematology, Ion Chiricuta Cancer Center, Cluj-Napoca, Romania; Department of Hematology / Department of Personalized Medicine and Rare Diseases - Medfuture Institute for Biomedical Research, Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania
| | - Vlad Moisoiu
- Department of Hematology / Department of Personalized Medicine and Rare Diseases - Medfuture Institute for Biomedical Research, Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania; Department of Neurology and Clinical Neuroscience Center, University Hospital Zurich, Zurich, Switzerland
| | - Catalin Constantinescu
- Department of Hematology, Ion Chiricuta Cancer Center, Cluj-Napoca, Romania; Department of Hematology / Department of Personalized Medicine and Rare Diseases - Medfuture Institute for Biomedical Research, Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania
| | - Alina Tanase
- Department of Stem Cell Transplantation, Fundeni Clinical Institute, Bucharest, Romania
| | - Gabriel Ghiaur
- Department of Hematology / Department of Personalized Medicine and Rare Diseases - Medfuture Institute for Biomedical Research, Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania; Department of Leukemia, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University School of Medicine, Baltimore, MD, United States
| | - Hermann Einsele
- Department of Hematology / Department of Personalized Medicine and Rare Diseases - Medfuture Institute for Biomedical Research, Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania; Department of Internal Medicine, Julius Maximilians University of Wurzburg, Wurzburg, Germany
| | - Ciprian Tomuleasa
- Department of Hematology, Ion Chiricuta Cancer Center, Cluj-Napoca, Romania; Department of Hematology / Department of Personalized Medicine and Rare Diseases - Medfuture Institute for Biomedical Research, Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
| | - Hillard M Lazarus
- Department of Medicine, Case Western Reserve University, Cleveland, OH, USA
| | - Robert Peter Gale
- Centre for Haematology, Imperial College of Science, Technology and Medicine, London, UK; Department of Hematologic Oncology, Sun Yat-sen University Cancer Center, Guangzhou, China
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Cody SG, Adam A, Siniavin A, Kang SS, Wang T. Flaviviruses-Induced Neurological Sequelae. Pathogens 2024; 14:22. [PMID: 39860983 PMCID: PMC11768111 DOI: 10.3390/pathogens14010022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2024] [Revised: 12/24/2024] [Accepted: 12/27/2024] [Indexed: 01/27/2025] Open
Abstract
Flaviviruses, a group of single-stranded RNA viruses spread by mosquitoes or ticks, include several significant neurotropic viruses, such as West Nile virus (WNV), Japanese encephalitis virus (JEV), tick-borne encephalitis virus (TBEV), and Zika virus (ZIKV). These viruses can cause a range of neurological diseases during acute infection, from mild, flu-like symptoms to severe and fatal encephalitis. A total of 20-50% of patients who recovered from acute flavivirus infections experienced long-term cognitive issues. Here, we discuss these major neurotropic flaviviruses-induced clinical diseases in humans and the recent findings in animal models and provide insights into the underlying disease mechanisms.
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Affiliation(s)
- Samantha Gabrielle Cody
- Department of Microbiology & Immunology, University of Texas Medical Branch, Galveston, TX 77555, USA; (S.G.C.); (A.A.); (A.S.); (S.S.K.)
- Institute for Translational Sciences, University of Texas Medical Branch, Galveston, TX 77555, USA
| | - Awadalkareem Adam
- Department of Microbiology & Immunology, University of Texas Medical Branch, Galveston, TX 77555, USA; (S.G.C.); (A.A.); (A.S.); (S.S.K.)
- Sealy Institute for Vaccine Sciences, University of Texas Medical Branch, Galveston, TX 77555, USA
| | - Andrei Siniavin
- Department of Microbiology & Immunology, University of Texas Medical Branch, Galveston, TX 77555, USA; (S.G.C.); (A.A.); (A.S.); (S.S.K.)
| | - Sam S. Kang
- Department of Microbiology & Immunology, University of Texas Medical Branch, Galveston, TX 77555, USA; (S.G.C.); (A.A.); (A.S.); (S.S.K.)
| | - Tian Wang
- Department of Microbiology & Immunology, University of Texas Medical Branch, Galveston, TX 77555, USA; (S.G.C.); (A.A.); (A.S.); (S.S.K.)
- Sealy Institute for Vaccine Sciences, University of Texas Medical Branch, Galveston, TX 77555, USA
- Institute for Human Infections and Immunity, University of Texas Medical Branch, Galveston, TX 77555, USA
- Department of Pathology, University of Texas Medical Branch, Galveston, TX 77555, USA
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Papri N, Mohammed A, Rahman MM, Hasan I, Azam R, Saha T, Shaon FTU, Jahan I, Hayat S, Ara G, Islam B, Islam Z. Pain determinants and quality of life in Guillain-Barre syndrome: a prospective cohort study. BMJ Neurol Open 2024; 6:e000925. [PMID: 39687605 PMCID: PMC11647308 DOI: 10.1136/bmjno-2024-000925] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2024] [Accepted: 11/15/2024] [Indexed: 12/18/2024] Open
Abstract
Background 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. Methods 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. Results 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. Conclusions 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.
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Affiliation(s)
| | | | | | | | | | - Tamal Saha
- Gut-Brain Axis, ICDDRB, Dhaka, Bangladesh
| | | | | | | | - Gulshan Ara
- Nutrition Research Division, ICDDRB, Dhaka, Bangladesh
| | - Badrul Islam
- Sheikh Fazilatunnessa Mujib Memorial KPJ Specialized Hospital & Nursing College, Dhaka, Bangladesh
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Alqahtani S, Al-Dorzi HM, Arishi H, Peeran A, Bin Humaid F, Alenezi FZ, Jose J, Sadat M, Alotaibi N, Arabi YM. Characteristics and outcomes of patients with Guillain-Barré syndrome who were admitted to the intensive care unit: a retrospective observational study. J Int Med Res 2024; 52:3000605241306655. [PMID: 39719074 PMCID: PMC11683809 DOI: 10.1177/03000605241306655] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/01/2024] [Accepted: 11/26/2024] [Indexed: 12/26/2024] Open
Abstract
OBJECTIVE To evaluate characteristics and outcomes in critically ill patients with Guillain-Barré syndrome (GBS). METHODS Consecutive adults with GBS who required intensive care unit (ICU) admission at a tertiary-care hospital between 1999 and 2020 were enrolled into this retrospective cohort study. Demographics, clinical data and patient outcomes were compared between patients who did or did not receive mechanical ventilation (MV). RESULTS During the study period, the number of ICU admissions gradually rose from approximately 900 to 3000 annually. Forty-three patients had GBS and were included, of whom, 27 (62.8%) received MV for a median of 13 days. The MV group stayed longer in the ICU (median, 26 versus 6 days) and in the hospital (median, 120 versus 39 days) than the non-MV group. Most patients in the MV group (22 [81.5%]) required tracheostomy. At maximum follow-up, Hughes Functional Grading scores were 0 (full recovery) in 11 patients (25.5%), 1-3 in 18 (41.8%), 4-5 in 12 (27.9%), and 6 (death) in two (4.6%, both in the MV group), with higher median Hughes score in the MV group (3 versus 0.5). Complications during ICU and hospital stay included: veinous thromboembolism in five (11.6%), gastrointestinal bleeding in three (7.0%), bacteremia in five (11.6%), bedsore in one (2.3%), and GBS-treatment side effects in four (9.4%) patients; all of these complications occurred within the MV group. CONCLUSIONS GBS was an uncommon reason for ICU admission. The findings highlight significant morbidity with GBS, particularly among patients who need MV.
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Affiliation(s)
- Samah Alqahtani
- College of Medicine, King Saud bin Abdulaziz University for Health Sciences, King Abdullah International Medical Research Center, Intensive Care Department, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Riyadh, Saudi Arabia
| | - Hasan M Al-Dorzi
- College of Medicine, King Saud bin Abdulaziz University for Health Sciences, King Abdullah International Medical Research Center, Intensive Care Department, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Riyadh, Saudi Arabia
| | - Hatim Arishi
- College of Medicine, King Saud bin Abdulaziz University for Health Sciences, King Abdullah International Medical Research Center, Intensive Care Department, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Riyadh, Saudi Arabia
| | - Ahmad Peeran
- College of Medicine, King Saud bin Abdulaziz University for Health Sciences, King Abdullah International Medical Research Center, Neurology Department, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Riyadh, Saudi Arabia
| | - Felwa Bin Humaid
- King Abdullah International Medical Research Center, King Saud bin Abdulaziz University for Health Sciences, Ministry of National Guard-Health Affairs, Riyadh, Saudi Arabia
| | - Farhan Zayed Alenezi
- College of Medicine, King Saud bin Abdulaziz University for Health Sciences, King Abdullah International Medical Research Center, Intensive Care Department, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Riyadh, Saudi Arabia
| | - Jesna Jose
- Department of Bioinformatics and Biostatistics, King Abdullah International Medical Research Center, King Saud bin Abdulaziz University for Health Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia
| | - Musharaf Sadat
- College of Medicine, King Saud bin Abdulaziz University for Health Sciences, King Abdullah International Medical Research Center, Intensive Care Department, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Riyadh, Saudi Arabia
| | - Naser Alotaibi
- College of Medicine, King Saud bin Abdulaziz University for Health Sciences, King Abdullah International Medical Research Center, Neurology Department, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Riyadh, Saudi Arabia
| | - Yaseen M Arabi
- College of Medicine, King Saud bin Abdulaziz University for Health Sciences, King Abdullah International Medical Research Center, Intensive Care Department, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Riyadh, Saudi Arabia
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Lee MJ, Yoon SJ. Changes in body composition, physical fitness and quality of life on robotic gait assisted training in patients with Guillain-Barré Syndrome: a case report. Phys Act Nutr 2024; 28:9-14. [PMID: 39934625 PMCID: PMC11811613 DOI: 10.20463/pan.2024.0027] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2024] [Revised: 10/29/2024] [Accepted: 11/18/2024] [Indexed: 02/13/2025] Open
Abstract
PURPOSE This case report evaluates changes in body composition, physical fitness, and quality of life in a patient with Guillain-Barre syndrome (GBS) undergoing training with an end-effector gait robotic device. METHODS A 35-year-old man with GBS completed interval training using a robotic gait assistant three times weekly for four weeks. The training intensity was set at 70-75% (Phase 1) and 80-85% (Phase 2) of the target heart rate, calculated using the heart rate reserve. Evaluations included body composition, physical fitness, isometric strength, gait performance, and quality of life indices. RESULTS Post-intervention, improvements were observed across all parameters. Body weight increased by 1.2%, while body fat percentage and visceral fat decreased by 5.4% and 3.7%, respectively. Muscle mass increased by 3.3%, and isometric strength improved at all tested sites. Gait speed increased from 0.7 to 1.8 km/h (157.1%), and the number of steps per minute increased from 33 to 77 (133.3%). The timed up-and-go (TUG) test improved by 7.1%, and exercise self-efficacy (ESE) scores increased by 29.1%. CONCLUSION These findings align with the principles of neuromuscular adaptation, motor learning, and neuroplasticity. Interval training with a robotic gait device may effectively restore physical function and improve quality of life in patients with GBS. However, the limitations of this single case study highlight the need for randomized controlled trials with larger cohorts and long-term effectiveness assessments.
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Affiliation(s)
- Moon Jin Lee
- Department of Exercise Rehabilitation, Institute of Human Convergence Health Science, Gachon University, Incheon, Republic of Korea
- Department of Physical Education, College of Education, Korea University, Seoul, Republic of Korea
| | - Sung Jin Yoon
- Department of Physical Education, College of Education, Korea University, Seoul, Republic of Korea
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Ubogu EE. Animal models of immune-mediated demyelinating polyneuropathies. Autoimmunity 2024; 57:2361745. [PMID: 38850571 PMCID: PMC11215812 DOI: 10.1080/08916934.2024.2361745] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/25/2024] [Revised: 05/13/2024] [Accepted: 05/26/2024] [Indexed: 06/10/2024]
Abstract
Immune-mediated demyelinating polyneuropathies (IMDPs) are rare disorders in which dysregulated adaptive immune responses cause peripheral nerve demyelinating inflammation and axonal injury in susceptible individuals. Despite significant advances in understanding IMDP pathogenesis guided by patient data and representative mammalian models, specific therapies are lacking. Significant knowledge gaps in IMDP pathogenesis still exist, e.g. precise antigen(s) and mechanisms that initially trigger immune system activation and identification of large population disease susceptibility factors. The initial directional cues for antigen-specific effector or autoreactive leukocyte trafficking into peripheral nerves are also unknown. An overview of current animal models, with emphasis on the experimental autoimmune neuritis and spontaneous autoimmune peripheral polyneuropathy models, is provided. Insights on the initial directional cues for peripheral nerve tissue specific autoimmunity using a novel Major Histocompatibility Complex class II conditional knockout mouse strain are also discussed, suggesting an essential research tool to study cell- and time-dependent adaptive immunity in autoimmune diseases.
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Affiliation(s)
- Eroboghene E Ubogu
- Neuromuscular Immunopathology Research Laboratory, Division of Neuromuscular Disease, Department of Neurology, University of Alabama at Birmingham, Birmingham, AL, USA
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Min YG, Hong YH, Rajabally YA, Sung JJ. Timing of intravenous immunoglobulin treatment and outcome in Guillain-Barré syndrome: Is time nerve? Muscle Nerve 2024; 70:1215-1222. [PMID: 39360628 DOI: 10.1002/mus.28271] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2024] [Revised: 09/17/2024] [Accepted: 09/18/2024] [Indexed: 10/04/2024]
Abstract
INTRODUCTION/AIMS Despite treatment, a considerable proportion of patients with Guillain-Barré syndrome (GBS) experience poor recovery, highlighting a therapeutic need. There is a lack of evidence that treatment timing affects recovery. This study aims to investigate the effects of intravenous immunoglobulin (IVIg) timing on disability and speed of recovery in GBS. METHODS We performed a retrospective study of 136 IVIg-treated GBS patients admitted to two Korean centers between 2010 and 2021. We analyzed the effect of time to IVIg on the GBS disability scale (GBS-DS) and the degree of improvement from nadir (∆GBS-DS) at 1, 3, 6, and 12 months, as well as the time to regain the ability to walk or run unaided. Time to IVIg was treated either as a continuous variable or categorized into 1-week intervals to explore critical time windows. Known prognostic factors, the modified Erasmus GBS Outcome Scores on admission and pre-treatment serum albumin levels were adjusted as covariates. RESULTS Shorter time to IVIg was independently associated with better GBS-DS, greater ∆GBS-DS, and shorter time to walk or run unaided at all time points. The therapeutic effect of IVIg was notably diminished when administered beyond the first 2 weeks of onset. DISCUSSION Our study highlights the timing of IVIg as a modifiable prognostic factor in GBS. The earlier IVIg is initiated, the better the outcomes, with the ideal time window being within the first 2 weeks. These findings underscore the importance of prompt diagnosis and early intervention to optimize recovery in GBS patients.
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Affiliation(s)
- Young Gi Min
- Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea
- Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea
| | - Yoon-Ho Hong
- Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea
- Department of Neurology, Seoul Metropolitan Government Boramae Medical Center, Seoul, Republic of Korea
| | - Yusuf A Rajabally
- Aston Medical School, School of Health and Life Sciences, Birmingham, UK
| | - Jung-Joon Sung
- Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea
- Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea
- Biomedical Research Institute, Seoul National University Hospital, Seoul, Republic of Korea
- Neuroscience Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea
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Patel P, Thakkar K, Shah D, Shah U, Pandey N, Patel J, Patel A. Decrypting the multifaceted peripheral neuropathy based on molecular pathology and therapeutics: a comprehensive review. Arch Physiol Biochem 2024; 130:886-897. [PMID: 38588401 DOI: 10.1080/13813455.2024.2336916] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/24/2023] [Revised: 12/28/2023] [Accepted: 03/26/2024] [Indexed: 04/10/2024]
Abstract
CONTEXT Peripheral neuropathy (PN) is a multifaceted complication characterized by nerve damage due to oxidative stress, inflammatory mediators, and dysregulated metabolic processes. Early PN manifests as sensory changes that develop progressively in a "stocking and glove" pattern. METHODS AND MECHANISMS A thorough review of literature has been done to find the molecular pathology, clinical trials that have been conducted to screen the effects of different drugs, current treatments and novel approaches used in PN therapy. Diabetic neuropathy occurs due to altered protein kinase C activity, elevated polyol pathway activity in neurons, and Schwann cells-induced hyperglycemia. Other causes involve chemotherapy exposure, autoimmune ailments, and chronic ethanol intake. CONCLUSION Symptomatic treatments for neuropathic pain include use of tricyclic antidepressants, anticonvulsants, and acetyl-L-carnitine. Patients will have new hope if clinicians focus on novel therapies including gene therapy, neuromodulation techniques, and cannabidiol as an alternative to traditional medications, as management is still not ideal.
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Affiliation(s)
- Praysha Patel
- Ramanbhai Patel College of Pharmacy, CHARUSAT, Changa, Gujarat, India
| | - Krishna Thakkar
- Ramanbhai Patel College of Pharmacy, CHARUSAT, Changa, Gujarat, India
| | - Div Shah
- Ramanbhai Patel College of Pharmacy, CHARUSAT, Changa, Gujarat, India
| | - Umang Shah
- Ramanbhai Patel College of Pharmacy, CHARUSAT, Changa, Gujarat, India
| | - Nilesh Pandey
- Health Science Center, Louisiana State University, Shreveport, LA, USA
| | - Jayesh Patel
- Consultant, Vascular surgeon, Shree Krishna Hospital, Karamsad, Gujarat, India
| | - Alkeshkumar Patel
- Ramanbhai Patel College of Pharmacy, CHARUSAT, Changa, Gujarat, India
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Rałowska-Gmoch W, Koszewicz M, Łabuz-Roszak B, Budrewicz S, Dziadkowiak E. Diagnostic criteria and therapeutic implications of rapid-onset demyelinating polyneuropathies. Exp Mol Pathol 2024; 140:104942. [PMID: 39500252 DOI: 10.1016/j.yexmp.2024.104942] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/04/2024] [Revised: 10/21/2024] [Accepted: 10/24/2024] [Indexed: 12/20/2024]
Abstract
Guillain-Barré syndrome (GBS) and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) are the most common autoimmune polyneuropathies. Their aetiology is unclear. The pathomechanism includes damage mainly to the myelin sheath and, in the long-term process, secondary axonal loss. Both inflammatory polyneuropathies involve different combinations of motor, sensory and autonomic fibres in the peripheral nerves. The differential diagnosis should be based on clinical and neurophysiological features, and laboratory tests. Numerous studies aim to demonstrate the most common errors in the diagnosis of Guillain-Barré syndrome and acute-onset CIDP. Misdiagnosis can result in the wrong treatment. We still do not have reliable markers to help diagnose the disease or to monitor the effectiveness of the therapy.
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Affiliation(s)
- Wiktoria Rałowska-Gmoch
- Department of Neurology, St. Jadwiga Provincial Specialist Hospital, Institute of Medical Sciences, University of Opole, pl. Kopernika 11a, 45-040 Opole, Poland
| | - Magdalena Koszewicz
- Clinical Neurophysiology Laboratory, University Centre of Neurology and Neurosurgery, Faculty of Medicine, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland
| | - Beata Łabuz-Roszak
- Department of Neurology, St. Jadwiga Provincial Specialist Hospital, Institute of Medical Sciences, University of Opole, pl. Kopernika 11a, 45-040 Opole, Poland
| | - Sławomir Budrewicz
- Clinical Department of Neurology, University Centre of Neurology and Neurosurgery, Faculty of Medicine, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland
| | - Edyta Dziadkowiak
- Clinical Department of Neurology, University Centre of Neurology and Neurosurgery, Faculty of Medicine, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland.
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Kantawala B, Uwishema O, Minova A, Babuya J, Wodajo EK, Asri S, Wojtara M. Guillain-Barré Syndrome in Africa: Understanding, Addressing, and Empowering. Health Sci Rep 2024; 7:e70232. [PMID: 39633838 PMCID: PMC11615645 DOI: 10.1002/hsr2.70232] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2023] [Revised: 11/12/2024] [Accepted: 11/18/2024] [Indexed: 12/07/2024] Open
Abstract
Introduction Guillain-Barré Syndrome (GBS) is a spectrum of peripheral neuropathies characterized by rapid symmetrical limb weakness and sensory symptoms. GBS can be life-threatening and requires intensive care, particularly for patients with imminent respiratory failure. In Africa, limited research and high therapy costs pose challenges. This literature review aims to comprehensively address GBS in Africa to improve understanding and outcomes. Aim This literature review aims to provide an extensive overview of GBS in Africa, encompassing its clinical presentation, impact, management approaches, and challenges faced. It also highlights the need for increased research and awareness to enhance patient care and outcomes. Methods A comprehensive review of existing literature on GBS in Africa was conducted, focusing on clinical presentation, diagnosis, management, and its impact on patients and communities. Data sources included medical databases, research articles, and reports. Data was scoured from databases such as PubMed, Medline and Embase. A total of four hundred and fifty-five articles and case studies were screened, with broader topic margins into GBS and different triggers, demographics, statistics, and variations in treatments across the world. These articles were further screened to match our inclusion criteria which focused on articles published after 2000 and which gave clearer insights into the presentations and situation of GBS in the African continent. Results GBS in Africa is characterized by a range of clinical presentations, with limited diagnostic resources and healthcare infrastructure. Patients often face long intervals between symptom onset and hospitalization, impacting outcomes. The syndrome's impact extends beyond physical symptoms, affecting patients' quality of life, employment, and community roles. Management involves immunotherapy, physiotherapy, and psychosocial support, but high therapy costs and incomplete recovery pose challenges. Research in Africa has grown in recent years but remains limited compared to other regions. Efforts are needed to expand research capacity, introduce early screening programs, and improve healthcare infrastructure. Conclusion GBS presents a significant healthcare challenge in Africa, with the potential for severe clinical outcomes. This literature review underscores the importance of enhancing research, awareness, and healthcare infrastructure. African-led research initiatives offer hope for improved patient outcomes and healthcare system strengthening. By advocating for increased government support and resources, Africa can address the pressing needs of GBS patients and foster a brighter and healthier future for affected individuals on the continent.
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Affiliation(s)
- Burhan Kantawala
- Department of Research and EducationOli Health Magazine Organization, Research and EducationKigaliRwanda
- Faculty of General MedicineYerevan State Medical UniversityYerevanArmenia
| | - Olivier Uwishema
- Department of Research and EducationOli Health Magazine Organization, Research and EducationKigaliRwanda
| | - Anamarija Minova
- Department of Research and EducationOli Health Magazine Organization, Research and EducationKigaliRwanda
- Faculty of MedicineSs. Cyril and MethodiusSkopjeRepublic of North Macedonia
| | - Jonathan Babuya
- Department of Research and EducationOli Health Magazine Organization, Research and EducationKigaliRwanda
- Faculty of Health SciencesBusitema UniversityMbaleUganda
| | - Essey Ketema Wodajo
- Department of Research and EducationOli Health Magazine Organization, Research and EducationKigaliRwanda
- College of Health Sciences, School of MedicineAddis Ababa UniversityAddis AbabaEthiopia
| | - Sucharu Asri
- Department of Research and EducationOli Health Magazine Organization, Research and EducationKigaliRwanda
- Department of General MedicineSGT Medical college Hospital and Research InstituteGurugramIndia
| | - Magda Wojtara
- Department of Research and EducationOli Health Magazine Organization, Research and EducationKigaliRwanda
- University of Michigan Medical SchoolAnn ArborMichiganUSA
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Chen Y, Li K, Lv W, Xie J, Qian Y, Cui C, Deng B. What Is the Impact of the Novel Coronavirus and the Vaccination on Guillain-Barre Syndrome? Mol Neurobiol 2024; 61:9835-9850. [PMID: 37728848 DOI: 10.1007/s12035-023-03638-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2023] [Accepted: 09/04/2023] [Indexed: 09/21/2023]
Abstract
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.
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Affiliation(s)
- Yinuo Chen
- Department of Neurology, First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China
- First Clinical College of Wenzhou Medical University, Wenzhou, China
| | - Kezheng Li
- Department of Neurology, First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China
- First Clinical College of Wenzhou Medical University, Wenzhou, China
| | - Wenjing Lv
- Department of Geriatrics, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Jiali Xie
- Department of Neurology, Shanghai East Hospital, Tongji University, Shanghai, People's Republic of China
| | - Yuqin Qian
- Department of Neurology, Institute of Neurology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Can Cui
- Department of Clinical Sciences Malmö, Lund University, Skåne, Sweden
| | - Binbin Deng
- Department of Neurology, First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China.
- First Clinical College of Wenzhou Medical University, Wenzhou, China.
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Papri N, Doets AY, Luijten L, Mohammad QD, Endtz HP, Lingsma HF, Jacobs BC, Islam Z. Prediction of respiratory failure and prolonged mechanical ventilation in Guillain-Barré syndrome: A prospective cohort study in Bangladesh. J Peripher Nerv Syst 2024; 29:428-440. [PMID: 39581760 DOI: 10.1111/jns.12673] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/10/2024] [Revised: 11/07/2024] [Accepted: 11/08/2024] [Indexed: 12/10/2024]
Abstract
BACKGROUND AND AIMS The aim of this study is to validate and perform a region-specific adjustment of the Erasmus GBS Respiratory Insufficiency Score (EGRIS) and identify potential predictors of prolonged mechanical ventilation (PMV) among Guillain-Barré syndrome (GBS) patients from Bangladesh. METHODS We enrolled GBS patients from four prospective observational cohort studies conducted in Bangladesh. Accuracy of EGRIS to predict the requirement of MV in <7 days of study entry was evaluated. Model performance was assessed by discrimination (ability of the model to differentiate between patients who needed MV or not) and calibration (accuracy of absolute risk estimates). PMV was defined as duration of MV >14 days. Potential predictors for PMV were evaluated by Cox regression. RESULTS A total of 594 GBS patients aged ≥6 years old were enrolled; of whom 541 patients had complete EGRIS data prior to MV and were included in validation analysis. EGRIS correctly distinguished between patients requiring MV or not in 81% pairs (AUC = 0.81). EGRIS overestimated the probability of MV than the observed probability (41% vs. 20%) which was resolved by updating of the model intercept. Inability to flex hip at day 7 of start of MV was the strongest predictor for PMV with predicted probabilities of 82%. INTERPRETATION EGRIS accurately predicts the need for MV in GBS patients from Bangladesh. This study developed a region-specific version of EGRIS and identified predictors of PMV. These findings can assist clinicians to identify patients at high risk of developing respiratory failure and requiring PMV to ensure timely intubation and tracheostomy of the patients in low resource settings.
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Affiliation(s)
- Nowshin Papri
- Laboratory of Gut-Brain Axis, icddr,b, Dhaka, Bangladesh
- Department of Neurology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
| | - Alex Y Doets
- Department of Neurology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
| | - Linda Luijten
- Department of Neurology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
- Department of Neurology, St. Elisabeth-TweeSteden Hospital, Tilburg, The Netherlands
| | - Quazi D Mohammad
- National Institute of Neurosciences and Hospital, Dhaka, Bangladesh
| | - Hubert P Endtz
- Department of Medical Microbiology and Infectious Diseases, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
| | - Hester F Lingsma
- Department of Public Health, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
| | - Bart C Jacobs
- Department of Neurology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
- Department of Immunology, Erasmus MC, University Medical Center, Rotterdam, Netherlands
| | - Zhahirul Islam
- Laboratory of Gut-Brain Axis, icddr,b, Dhaka, Bangladesh
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Stino AM, Reynolds EL, Watanabe M, Callaghan BC. Intravenous immunoglobulin and plasma exchange prescribing patterns for Guillain-Barre Syndrome in the United States-2001 to 2018. Muscle Nerve 2024; 70:1192-1199. [PMID: 39324188 PMCID: PMC11560546 DOI: 10.1002/mus.28265] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2024] [Revised: 09/09/2024] [Accepted: 09/16/2024] [Indexed: 09/27/2024]
Abstract
INTRODUCTION/AIMS Randomized controlled trials show that repeat intravenous immunoglobulin (IVIG) dosing and plasma exchange (PLEX) followed by IVIG (combination therapy) have no additional therapeutic benefit in Guillain-Barre Syndrome (GBS) non-responders. Furthermore, the delineation between GBS and Acute Onset CIDP (A-CIDP) can be particularly challenging and carries therapeutic implications. We aimed to evaluate the presence of repeat IVIG, combination therapy, and diagnostic reclassification from GBS to CIDP. METHODS We performed a retrospective study of a large healthcare database for patients with GBS in the US from 2001 to 2018. We identified individuals initially diagnosed with GBS and later re-classified as CIDP. Multivariable logistic regression models were developed to determine associations between patient factors and repeat IVIG dosing, combination therapy, and diagnostic re-classification from GBS to CIDP. RESULTS We identified 2325 patients with GBS. A total of 39.7% received repeat IVIG and 6.1% received combination therapy. The proportion of individuals initially diagnosed with GBS and then re-classified as CIDP was 32.0%. Repeat IVIG, combination therapy, and diagnostic reclassification remained stable over time. Female sex (OR 0.79, 95% CI 0.65-0.96) and medium-high net worth (OR 0.64, 95% CI 0.45-0.90) associated with repeat IVIG therapy, while Asian ethnicity associated with diagnostic re-classification from GBS to CIDP (OR 1.77, 95% CI 1.09-2.86). DISCUSSION Repeat IVIG dosing was quite common in GBS before newer trials suggesting harm in non-responders, and IVIG/PLEX combination therapy continues to persist despite strong evidence against use in non-responders. Further, nearly one in three patients initially diagnosed with GBS is subsequently diagnosed with CIDP, but the reasons are unclear.
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Affiliation(s)
- Amro M. Stino
- Department of Neurology, University of Michigan, Ann Arbor, MI, USA
| | - Evan L. Reynolds
- Department of Neurology, University of Michigan, Ann Arbor, MI, USA
| | - Maya Watanabe
- Department of Biostatistics, University of Michigan, Ann Arbor, MI, USA
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James L, Mohan A, Mol Thankam J, George J. Guillain Barré Syndrome Mimicking Brain Death: A Case Report. Cureus 2024; 16:e76297. [PMID: 39850179 PMCID: PMC11755197 DOI: 10.7759/cureus.76297] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/20/2024] [Indexed: 01/25/2025] Open
Abstract
Here, we present a case of Guillain-Barré syndrome (GBS) that mimicked brain death. A 66-year-old lady with a medical history of breast cancer (now receiving hormone therapy), hypertension, and hypothyroidism, presented to the emergency department. The patient was admitted to the neuro ICU with absent brainstem and spinal cord responses, concerning for possible brain death. Further evaluation, however, identified the uncommon GBS with respiratory failure. This case emphasizes the need to recognize this severe manifestation of GBS since misinterpreting this as brain death might result in the discontinuation of ventilatory support.
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Affiliation(s)
- Lijo James
- Neurology, Caritas Hospital, Kottayam, IND
| | - Aiswarya Mohan
- Research and Development Cell, Caritas Hospital and Institute of Health Sciences, Kottayam, IND
| | - Jithu Mol Thankam
- Neurocritical Care, Caritas Hospital and Institute of Health Sciences, Kottayam, IND
| | - Jeeva George
- Neurocritical Care, Caritas Hospital and Institute of Health Sciences, Kottayam, IND
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Liu X, Liu L, Zhang J. Causal role of the pyrimidine deoxyribonucleoside degradation superpathway mediation in Guillain-Barré Syndrome via the HVEM on CD4 + and CD8 + T cells. Sci Rep 2024; 14:27418. [PMID: 39521826 PMCID: PMC11550458 DOI: 10.1038/s41598-024-78996-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2024] [Accepted: 11/05/2024] [Indexed: 11/16/2024] Open
Abstract
Immune system regulation is a key indicator of the gut microbiota (GM) influencing disease development. The causal role of the GM in Guillain-Barré syndrome (GBS) and whether it can be mediated by immune cells is unknown. Genome-wide association study (GWAS) summary statistics for the GM were obtained from the Dutch Microbiota Project (n = 7,738) and the FINRISK 2002 (FR02) cohort (n = 5,959). Inverse variance weighting method (IVW) were used as the main method to evaluate the causal relationship between GM and GBS. Subsequently, the mediating effects of 731 immune traits were evaluated. Additionally, we also executed the Bayesian Weighting algorithm for verification. Mendelian randomization (MR) analysis determined the protective effect of the pyrimidine deoxyribonucleoside degradation superpathway on GBS (IVW: P = 0.0019, OR = 0.4508). It is worth noting that in the causal effects of pyrimidine deoxyribonucleoside degradation superpathway on GBS, the mediated proportions of herpesvirus entry mediator (HVEM) ( HVEM on CM CD4 + , HVEM on naive CD4 + , HVEM on CD45RA - CD4 + , HVEM on CM CD8br) in the T cell maturation stage on GBS were -0.0398, -0.0452, -0.0414, -0.0425, accounting for 5.00%, 5.67%, 5.19% and 5.34% of the total effect. 11 types of intestinal bacteria might be involved in the pyrimidine deoxyriboside degradation superpathway, including Staphylococcus A fleurettii, AR31,CAG-274 sp000432155, Photobacterium, Acetobacteraceae, Dysgonomonadaceae, NK4A144,Leptospirae, CAG-81 sp000435795, Leptospirales and CAG-873 sp001701165. This study suggests that there is a causal relationship between pyrimidine deoxyribonucleoside degradation superpathway and GBS, which may be mediated by HVEM on CD4 + and CD8 + T cells. As a bidirectional molecular switch, HVEM plays an important role in T cell regulation. 11 intestinal flora were found to be involved in pyrimidine deoxyribonucleoside degradation superpathway, and their changes may be related to the occurrence of GBS. However, extensive research is still warranted before microbiome sequencing can be used for prevention and targeted treatment of GBS.
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Affiliation(s)
- Xianghua Liu
- Jining No. 1 People's Hospital, Jining, Shandong, China
| | - Lingling Liu
- Clinical Laboratory Department, Jiangnan University Medical Center, JUMC, Wuxi, Jiangsu, China
| | - Jiuchang Zhang
- The Affiliated Hospital of Qingdao University, Qingdao, Shandong, China.
- Jining No. 1 People's Hospital, Jining, Shandong, China.
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Mamarabadi M, Mauney S, Li Y, Aboussouan LS. Evaluation and management of dyspnea as the dominant presenting feature in neuromuscular disorders. Muscle Nerve 2024; 70:916-928. [PMID: 39267292 DOI: 10.1002/mus.28243] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/30/2024] [Revised: 08/18/2024] [Accepted: 08/21/2024] [Indexed: 09/17/2024]
Abstract
Dyspnea is a common symptom in neuromuscular disorders and, although multifactorial, it is usually due to respiratory muscle involvement, associated musculoskeletal changes such as scoliosis or, in certain neuromuscular conditions, cardiomyopathy. Clinical history can elicit symptoms such as orthopnea, trepopnea, sleep disruption, dysphagia, weak cough, and difficulty with secretion clearance. The examination is essential to assist with the diagnosis of an underlying neurologic disorder and determine whether dyspnea is from a cardiac or pulmonary origin. Specific attention should be given to possible muscle loss, use of accessory muscles of breathing, difficulty with neck flexion/extension, presence of thoraco-abdominal paradox, conversational dyspnea, cardiac examination, and should include a detailed neurological examination directed at the suspected differential diagnosis. Pulmonary function testing including sitting and supine spirometry, measures of inspiratory and expiratory muscle strength, cough peak flow, sniff nasal inspiratory pressure, pulse oximetry, transcutaneous CO2, and arterial blood gases will help determine the extent of the respiratory muscle involvement, assess for hypercapnic or hypoxemic respiratory failure, and qualify the patient for noninvasive ventilation when appropriate. Additional testing includes dynamic imaging with sniff fluoroscopy or diaphragm ultrasound, and diaphragm electromyography. Polysomnography is indicated for sleep related symptoms that are not otherwise explained. Noninvasive ventilation alleviates dyspnea and nocturnal symptoms, improves quality of life, and prolongs survival. Therapy targeted at neuromuscular disorders may help control the disease or favorably modify its course. For patients who have difficulty with secretion clearance, support of expiratory function with mechanical insufflation-exsufflation, oscillatory devices can reduce the aspiration risk.
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Affiliation(s)
- Mansoureh Mamarabadi
- Department of Neurology, Penn State Health Milton S. Hershey Medical Center, Hershey, Pennsylvania, USA
| | - Sarah Mauney
- Department of Neurology, Penn State Health Milton S. Hershey Medical Center, Hershey, Pennsylvania, USA
| | - Yuebing Li
- Neuromuscular Center, Neurological Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Loutfi S Aboussouan
- Respiratory and Neurological Institutes, Cleveland Clinic, Cleveland, Ohio, USA
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