Clinical Neurology and Neurosurgery最新文献

{"title":"A case report and review of the literature on hereditary diffuse leukoencephalopathy with spheroids presenting with stroke-like symptoms","authors":"Jing Zhou ,&nbsp;Yiming Wang ,&nbsp;Yingte Wang ,&nbsp;Jiahao Li ,&nbsp;Airong Yang ,&nbsp;Xianliang Li ,&nbsp;Zhiming Li","doi":"10.1016/j.clineuro.2025.109019","DOIUrl":"10.1016/j.clineuro.2025.109019","url":null,"abstract":"<div><div>Hereditary diffuse leukoencephalopathy with spheroids (HDLS), a rare autosomal dominant disorder caused by CSF1R (Colony Stimulating Factor 1 Receptor) variants, manifests clinically as adult-onset cognitive-motor decline with progressive white matter degeneration. Notably, its phenotypic variability remains undercharacterized, with only limited case reports available globally. We systematically investigated a 47-year-old male presenting with acute stroke-like hemiparesis that progressed to fronto-subcortical dementia (MMSE=14) and extrapyramidal symptoms within six months. Comprehensive diagnostic evaluations included multimodal 3 T MRI and whole-exome sequencing (WES) targeting CSF1R. Neuroimaging revealed hallmark frontoparietal white matter rarefaction accompanied by persistent diffusion restriction (DWI+/ADC−). WES identified a de novo CSF1R nonsense variant (c.2462 G&gt;A, p.Trp821Ter), which truncates the tyrosine kinase domain. This variant was classified as pathogenic, confirming a definitive HDLS diagnosis. This stroke<strong>-</strong>onset HDLS expands the phenotypic spectrum, establishing persistent diffusion restriction as an early diagnostic biomarker. CSF1R sequencing proves critical for sporadic case identification regardless of family history. Study limitations include absence of pedigree segregation analysis.</div></div>","PeriodicalId":10385,"journal":{"name":"Clinical Neurology and Neurosurgery","volume":"256 ","pages":"Article 109019"},"PeriodicalIF":1.8,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144338386","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pick's disease presenting as progressive apraxia of speech: Atypical clinical and neuroimaging features in three autopsy-confirmed cases","authors":"Katherine E. Jones ,&nbsp;Jonathan Graff-Radford ,&nbsp;Rene L. Utianski ,&nbsp;Joseph R. Duffy ,&nbsp;Heather M. Clark ,&nbsp;Mary M. Machulda ,&nbsp;Dennis W. Dickson ,&nbsp;Jennifer L. Whitwell ,&nbsp;Keith A. Josephs ,&nbsp;Hugo Botha","doi":"10.1016/j.clineuro.2025.109018","DOIUrl":"10.1016/j.clineuro.2025.109018","url":null,"abstract":"<div><h3>Objectives</h3><div>Patients with progressive apraxia of speech (PAOS) often develop atypical parkinsonian features suggestive of corticobasal syndrome (CBS) or progressive supranuclear palsy (PSP), and typically have an underlying 4-repeat tauopathy at autopsy. We describe three cases of PAOS with underlying Pick’s disease, a 3-repeat tauopathy, who lacked CBS or PSP features during life.</div></div><div><h3>Methods</h3><div>We reviewed patients enrolled in the Neurodegenerative Research Group’s ongoing studies on speech and language disorders and identified those with PAOS who had autopsy-confirmed Pick’s disease. All patients had comprehensive neurologic, speech-language, and neuropsychological assessments, as well as multimodal neuroimaging, during life.</div></div><div><h3>Results</h3><div>Three female patients presented with phonetic PAOS without parkinsonism. Patient 1 had speech onset at age 54, later developed behavioral variant frontotemporal dementia (bvFTD), and died at 64. Patient 2 had speech onset at 47, early bvFTD features, prominent frontal and temporal involvement, and died at 53. Patient 3 had speech onset at 58, minimal behavioral changes, primarily frontal involvement on imaging, and died at 63.</div></div><div><h3>Conclusion</h3><div>Our findings highlight that Pick's disease can present with PAOS and may be distinguished from 4R-tau PAOS by an absence of motoric CBS/PSP features and, in some cases, by prominent temporal hypometabolism with bvFTD development. These atypical features may prove useful in the antemortem identification of Pick's disease as a cause of PAOS.</div></div>","PeriodicalId":10385,"journal":{"name":"Clinical Neurology and Neurosurgery","volume":"256 ","pages":"Article 109018"},"PeriodicalIF":1.8,"publicationDate":"2025-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144314064","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Distinct symptom recoveries based upon highest level of care in patients with sport-related concussion or traumatic brain injury and Glasgow Coma Scale 13–15","authors":"Shawn R. Eagle ,&nbsp;Bryan Lamb ,&nbsp;Daniel Huber ,&nbsp;Michael A. McCrea ,&nbsp;Sergey Tarima ,&nbsp;Terri A. deRoon-Cassini ,&nbsp;David O. Okonkwo ,&nbsp;Lindsay D. Nelson","doi":"10.1016/j.clineuro.2025.109017","DOIUrl":"10.1016/j.clineuro.2025.109017","url":null,"abstract":"<div><div>There are limited data directly comparing recovery across traumatic brain injury subpopulations. We compared symptom recovery profiles between patients with Glasgow Coma Scale (GCS) 13–15 traumatic brain injury (TBI) from the same region in three cohorts: (1) Participants with sport-related concussion (SRC), (2) participants evaluated and discharged from the level 1 trauma center emergency department (ED), and (3) participants who required 1 + night(s) in the inpatient unit (IP). The current analysis aggregates data from two prospective cohort studies at the same institution resulting in a combined dataset of 395 patients with TBI (224 with SRC, 95 discharged from the ED, and 75 admitted [IP]). The primary outcome measure of interest was self-reported TBI symptom duration (in days). Two multivariable Cox proportional hazards models evaluated differences in symptom recovery between groups while controlling for recovery risk factors, including age, sex, race/ethnicity, acute symptom severity, psychological disorder history, loss of consciousness, and post-traumatic amnesia. The second model included only ED and IP, due to availability of additional predictor variables in these samples (e.g., education, cause of injury, peripheral injury severity). In unadjusted models, hazards of symptom recovery were lower with increasing levels of care (IP vs. ED HR=.40, p &lt; .001; IP vs. SRC HR=.11, p &lt; .001, ED vs. SRC HR=.28, p &lt; .001). Controlling for covariates, longer symptom recovery in the trauma center subsamples versus SRC persisted (IP vs. SRC HR=.26, p = .018, ED vs. SRC HR=.52, p = .021), whereas differences between ED and IP became nonsignificant (HR=.86, p = .782). Acute symptom severity (HR=0.98; p &lt; 0.001–0.010) and psychiatric history (HR=0.27–0.36; p ≤ 0.034) were independent predictors of symptom duration in both models. The results of this study suggest that patients with TBI and GCS 13–15 seen at a level 1 trauma center vary significantly in symptom recovery and severity in comparison to those with SRC, regardless of population differences in age, sex and psychiatric history.</div></div>","PeriodicalId":10385,"journal":{"name":"Clinical Neurology and Neurosurgery","volume":"256 ","pages":"Article 109017"},"PeriodicalIF":1.8,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144297991","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Corrigendum to \"Imaging monitoring of Balamuthia granulomatous amoebic encephalitis\" [Clin. Neurol. Neurosurg. 254 (2025) 108917].","authors":"Jinzhi Mei, Fang Sheng, Chenmei Zhang, Xiaorong Chen","doi":"10.1016/j.clineuro.2025.109015","DOIUrl":"https://doi.org/10.1016/j.clineuro.2025.109015","url":null,"abstract":"","PeriodicalId":10385,"journal":{"name":"Clinical Neurology and Neurosurgery","volume":" ","pages":"109015"},"PeriodicalIF":1.8,"publicationDate":"2025-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144301228","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Deep brain stimulation for refractory epilepsy: A meta-analysis of stimulation parameters","authors":"Pooya Vahedi ,&nbsp;Shahab Lotfinia ,&nbsp;Alireza Zali ,&nbsp;Farzad Ashrafi ,&nbsp;Mahrooz Roozbeh ,&nbsp;Hossein Pakdaman ,&nbsp;Yasmin Tabatabavakili ,&nbsp;Mehrdad Roozbeh","doi":"10.1016/j.clineuro.2025.108992","DOIUrl":"10.1016/j.clineuro.2025.108992","url":null,"abstract":"<div><h3>Background</h3><div>Epilepsy affects approximately 50 million people worldwide, with around 30 % not responding to antiepileptic drugs. Neuromodulation therapies, such as deep brain stimulation (DBS), are increasingly crucial for managing poorly controlled epilepsy.</div></div><div><h3>Methods</h3><div>This study conducted a meta-analysis following PRISMA guidelines, systematically searching PubMed, Scopus, and Web of Science databases for studies on DBS in refractory epilepsy patients. Out of an initial 568 papers screened by title and abstract, 49 studies met the inclusion criteria, involving a total of 682 patients.</div></div><div><h3>Results</h3><div>Various DBS interventions were analyzed, targeting regions such as the anterior nucleus of the thalamus, centromedian thalamic nucleus, and hypothalamus, with diverse stimulation parameters, including voltage, frequency, and stimulation type. The analysis revealed that these parameters significantly impacted treatment success, with moderate variability among studies.</div></div><div><h3>Conclusions</h3><div>This meta-analysis underscores the importance of tailored DBS parameters to improve outcomes in patients with drug-resistant epilepsy, highlighting DBS as a promising treatment approach.</div></div>","PeriodicalId":10385,"journal":{"name":"Clinical Neurology and Neurosurgery","volume":"256 ","pages":"Article 108992"},"PeriodicalIF":1.8,"publicationDate":"2025-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144306308","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk analysis index-measured frailty as a critical predictor of outcomes in patients with non-normal pressure hydrocephalus undergoing first-time shunt surgery: A nationwide study","authors":"Gilberto Perez Rodriguez Garcia ,&nbsp;S. Farzad Maroufi ,&nbsp;Fnu Ruchika ,&nbsp;Danisha Kumar ,&nbsp;Christian Bowers ,&nbsp;Sarah T. Menacho","doi":"10.1016/j.clineuro.2025.109012","DOIUrl":"10.1016/j.clineuro.2025.109012","url":null,"abstract":"<div><h3>Objective</h3><div>To assess the predictive utility of the Risk Analysis Index (RAI) for in-hospital mortality, discharge disposition, extended length of stay (eLOS), and shunt failure in patients with non-NPH hydrocephalus (including obstructive and communicating subtypes) undergoing first-time shunt placement.</div></div><div><h3>Methods</h3><div>We performed a retrospective cohort study using Nationwide Inpatient Sample data. Non-NPH patients undergoing first-time shunt placement (ICD-10-CM G91.2) were stratified by RAI score (Normal vs. Frail), a metric previously validated for use with NIS data. Patients were further grouped by payer, race, income quartile, and hydrocephalus subtype. Primary outcomes included extended length of stay, non-home discharge, and in-hospital mortality.</div></div><div><h3>Results</h3><div>In a cohort of 2525 patients (mean age 43.8 years; 50.5 % female), the majority were White (68.4 %) and privately insured (39.6 %), with 92.0 % treated in large, urban teaching hospitals. Frailty was identified in 21.2 % of the cohort and was strongly associated with eLOS (OR 1.23–2.00), NHD (OR 1.74–2.69), and in-hospital mortality (OR 1.66–14.17). The RAI demonstrated the highest predictive value for mortality (AUC = 0.775) and age for NHD (AUC = 0.733; p &lt; 0.01). Additionally, obstructive hydrocephalus (OR 2.12–3.25) was linked to increased odds of eLOS.</div></div><div><h3>Conclusion</h3><div>RAI-measured frailty, advanced age, and specific non-NPH subtypes predicted adverse shunt outcomes. These findings support the RAI as a valuable tool for risk stratification and personalized perioperative planning in patients with non-NPH hydrocephalus, particularly in subacute presentations. Its use may help guide shared decision-making and resource preparedness.</div></div>","PeriodicalId":10385,"journal":{"name":"Clinical Neurology and Neurosurgery","volume":"256 ","pages":"Article 109012"},"PeriodicalIF":1.8,"publicationDate":"2025-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144263730","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Determinants of adult Ventriculoperitoneal Shunt failure: Insights from a large neurosurgical centre","authors":"James Stewart ,&nbsp;John Warner-Levy ,&nbsp;Sebastian Bate ,&nbsp;Catherine McMahon ,&nbsp;Dominic Slade ,&nbsp;Matthew Bailey","doi":"10.1016/j.clineuro.2025.109013","DOIUrl":"10.1016/j.clineuro.2025.109013","url":null,"abstract":"<div><h3>Background</h3><div>Ventriculoperitoneal Shunts (VPS) are commonly inserted and often lifesaving. Complication and failure rates are persistently high. Research to identify risk factors is disproportionately skewed to paediatric VPS and has returned inconsistent results. There has been minimal change in failure rates for decades.</div></div><div><h3>Objectives</h3><div>To identify modifiable risk factors for adult VPS failure, to identify non-modifiable risk factors which predict future failure, and to describe the timing of failure.</div></div><div><h3>Methods</h3><div>Single-centre retrospective cohort study reviewing all VPS surgery between December 2021 and December 2023. Minimum of one year follow-up. Data collected included patient demographics, underlying indication for VPS, and technical factors. Univariable and multivariable analysis used to investigate for risk factors for implant failure. Kaplan-Meier plots generated. Modality of failure recorded. We evaluate the use of a post-operative CT based grading system to predict future failure.</div></div><div><h3>Results</h3><div>202 operations (including 49 revisional surgeries) performed. Average follow-up excluding failed shunts and patients who died was 719 days. No patients died from VPS failure. Shunt survival at one-year was 71.5 %. Average time to failure was 62.48 days. Surgeon experience, use of non-programmable valves, previous permanent CSF diversion surgery for any reason other than insertion of the same VPS, ventricular catheter location, and the presence of a CNS tumour or aqueduct stenosis were significantly associated with failure. Failure was significantly higher after revisional surgery.</div></div><div><h3>Conclusion</h3><div>We have described the epidemiology of adult VPS failure, identified risk factors for failure, and described factors not associated with failure, guiding decision making and the technical performance of surgery.</div></div>","PeriodicalId":10385,"journal":{"name":"Clinical Neurology and Neurosurgery","volume":"256 ","pages":"Article 109013"},"PeriodicalIF":1.8,"publicationDate":"2025-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144297990","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of preoperative nutritional status on morbidity and mortality in geriatric patients undergoing spine surgery: Role of geriatric nutritional risk index","authors":"Aladine A. Elsamadicy ,&nbsp;Paul Serrato ,&nbsp;Selma Belkasim ,&nbsp;Shaila D. Ghanekar ,&nbsp;Justice Hansen ,&nbsp;Syed I. Khalid ,&nbsp;Sheng-fu Larry Lo ,&nbsp;Daniel M. Sciubba","doi":"10.1016/j.clineuro.2025.109000","DOIUrl":"10.1016/j.clineuro.2025.109000","url":null,"abstract":"<div><h3>Background</h3><div>Malnutrition is a risk factor for poor surgical outcomes amongst the elderly. However, the association between malnutrition and outcomes in geriatric patients undergoing spine surgery has been understudied. The aim of this study was to assess associations between malnutrition and 30-day morbidity and mortality in geriatric patients undergoing spine surgery.</div></div><div><h3>Methods</h3><div>A retrospective cohort study was performed using the American College of Surgeons National Surgical Quality Improvement Program database. All geriatric patients (≥ 65 years old) were categorized by Geriatric Nutritional Risk Index (GNRI) score: Normal, Malnourished, and Severely Malnourished. Patient demographics, comorbidities, and adverse events (AEs) were assessed. Multivariable logistic regression analyses identified independent predictors of 30-day postoperative AEs, extended length of stay (LOS), non-routine discharge (NRD), unplanned readmission, and mortality.</div></div><div><h3>Results</h3><div>Of the 79,160 patients identified, 58,906 (74.4 %) had Normal nutritional status, 12,184 (15.4 %) were Malnourished, and 8070 (10.2 %) were Severely Malnourished. The Malnourished and Severely Malnourished cohorts had significantly increased proportion of AEs (<em><u>p &lt; 0.001</u></em>) and a longer LOS (<em><u>p &lt; 0.001</u></em>). The Malnourished and Severely Malnourished cohorts had significantly increased proportions of NRD (<em><u>p &lt; 0.001</u></em>), unplanned readmissions (<em><u>p &lt; 0.001</u></em>), and 30-day mortality (<em><u>p &lt; 0.001</u></em>). On multivariable analysis, Malnourished and Severely Malnourished statuses, respectively, were independently associated with any AE (<em><u>p &lt; 0.001</u></em>), extended LOS (<em><u>p &lt; 0.001</u></em>), 30-day unplanned readmission (<em><u>p &lt; 0.001</u></em>), NRD (<em><u>p &lt; 0.001</u></em>), and 30-day mortality (<em><u>p &lt; 0.001</u></em>).</div></div><div><h3>Conclusion</h3><div>Our study suggests that preoperative GNRI-defined malnutrition may increase the risk of adverse postoperative outcomes following spine surgery in geriatric patients.</div></div>","PeriodicalId":10385,"journal":{"name":"Clinical Neurology and Neurosurgery","volume":"256 ","pages":"Article 109000"},"PeriodicalIF":1.8,"publicationDate":"2025-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144263731","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Characterization of methylation profile in biofluid cell-free DNA and identification of differentially methylated genes for phenotypic representations in Parkinson's disease","authors":"Aiqin Zhu ,&nbsp;Fenglin Wang ,&nbsp;Jie Meng ,&nbsp;Xiaotao Li ,&nbsp;Hongxiu Liang ,&nbsp;Yuhua Liang ,&nbsp;Lele Song","doi":"10.1016/j.clineuro.2025.109014","DOIUrl":"10.1016/j.clineuro.2025.109014","url":null,"abstract":"<div><h3>Background</h3><div>The potential biomarkers for Parkinson’s Disease (PD) in blood circulating cell-free DNA (cfDNA) have been infrequently explored. This study aims to identify specific methylation markers in blood cfDNA that could aid in the diagnosis of PD.</div></div><div><h3>Methods</h3><div>A total of twenty patients with PD and nine healthy participants were recruited for this study. Cerebrospinal fluid (CSF) and/or blood samples were collected from the patients. Whole-genome bisulfite sequencing (WGBS) was conducted to analyze cfDNA methylation patterns in both CSF and blood samples. Data analysis and figure plotting were performed using R software.</div></div><div><h3>Results</h3><div>We identified 1322 differentially methylated genes (DMGs) in CSF and 118 DMGs in blood cfDNA between patients with PD and healthy participants, with 16 DMGs being common across both sample types. Significant correlations were observed within the CSF regarding methylation signatures related to sex,age,occupational nature,dietary habits(meat-only, vegetarian or mixed),tea consumption,family history of disease, hypertension,diabetes,postural instability and gait disorder,hyposmia,as well as glossolalia. Conversely, significant correlations found in blood included associations between methylation signatures and factors such as duration of residence at high altitudes, cigarette use, diabetes prevalence, dysphagia occurrence, family history of PD, scores on the Unified Parkinson’s Disease Rating Scale (MDS-UPDRS), and results from the Hamilton Anxiety Scale (HAMA). Furthermore, potential diagnostic markers comprising common DMGs present in both CSF and blood cfDNA alongside phenotypic factors were identified. The CSF markers consisted of five methylated genes—APMAP,C19orf25,TMA16,NIT2,and NDST2—and three phenotypic factors including duration of residence at high altitude locations, hyperhidrosis, and family history of PD. In contrast, the blood cfDNA markers encompassed eight differentially methylated genes—QPCT,TMA16,APMAP,NIT2,DCP2,ATP,TNFAIP1,and ZNF700—and two phenotypic factors which included tea consumption and hyposmia.</div></div><div><h3>Conclusions</h3><div>For the first time, specific methylation signatures related to PD have been identified in blood samples. Furthermore, correlations were observed between methylation signatures in CSF and cfDNA from blood with various patient phenotype factors. Diagnostic markers for PD based on CSF and blood cfDNA methylation patterns, along with phenotypic characteristics, have been established.</div></div>","PeriodicalId":10385,"journal":{"name":"Clinical Neurology and Neurosurgery","volume":"256 ","pages":"Article 109014"},"PeriodicalIF":1.8,"publicationDate":"2025-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144291234","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Emergent role of complement inhibitors in myasthenic crisis: Understanding why, when and how","authors":"Elena Rossini ,&nbsp;Stefania Morino ,&nbsp;Matteo Garibaldi ,&nbsp;Luca Leonardi ,&nbsp;Laura Tufano ,&nbsp;Antonio Lauletta ,&nbsp;Francesca Forcina ,&nbsp;Giovanni Antonini ,&nbsp;Laura Fionda","doi":"10.1016/j.clineuro.2025.109011","DOIUrl":"10.1016/j.clineuro.2025.109011","url":null,"abstract":"<div><div>We report the case of a 32-year-old woman with an 11-year history of thymomatous AChR antibody-positive generalized myasthenia gravis (gMG, MGFA IIIb) who experienced a myasthenic crisis (MC) during the postpartum period, requiring mechanical ventilation (MV). Initial treatment with therapeutic plasma exchange (TPE) and high-dose prednisone was ineffective, and pyridostigmine worsened airway secretions. Due to contraindications for IVIg, she was treated with the complement inhibitor eculizumab, following prophylactic meningococcal vaccination and antibiotic therapy. Six days after starting eculizumab, the patient showed marked improvement in muscle strength, respiratory function, and was successfully extubated. She was discharged from the intensive care unit (ICU) and continued bi-weekly Eculizumab infusions without adverse events. A systematic literature review identified 19 additional cases of MC treated with eculizumab, examining response timing, treatment efficacy, adverse events, and follow-up data. This case, along with the literature review, supports the possibility of using anti-complement therapy in patients unresponsive to conventional therapies in MC setting.</div></div>","PeriodicalId":10385,"journal":{"name":"Clinical Neurology and Neurosurgery","volume":"256 ","pages":"Article 109011"},"PeriodicalIF":1.8,"publicationDate":"2025-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144263729","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}