JAMA neurology最新文献

{"title":"Neuromodulation for Children With Hemiparesis and Perinatal Stroke","authors":"Alicia J. Hilderley, Mary Dunbar, John Andersen, Darcy Fehlings, Megan Metzler, Helen L. Carlson, Ephrem Zewdie, Jacquie Hodge, Kathleen O’Grady, Lisa Carsolio, Nomazulu Dlamini, Adrianna Giuffre, Lauran Cole, Hsing-Ching Kuo, Anna Bourgeois, Asha Hollis, Meghan Maiani, Patrick Ciechanski, Zeanna Jadavji, Brandon Craig, Dion Kelly, Joanna Keough, James Wrightson, Linda Fay, Lauren Switzer, Maya Pajevic, Alana Ramsey, Michael Sametz, Brian L. Brooks, Maryna Yaskina, Jesse Batara, Michael D. Hill, Adam Kirton","doi":"10.1001/jamaneurol.2024.4898","DOIUrl":"https://doi.org/10.1001/jamaneurol.2024.4898","url":null,"abstract":"ImportanceCurrent upper-extremity therapies provide inconsistent outcomes for children with unilateral cerebral palsy. Noninvasive brain stimulation, specifically transcranial direct current stimulation, may enhance motor gains when combined with therapy.ObjectiveTo determine whether the addition of neurostimulation to upper-extremity therapy enhances motor function in children with perinatal stroke and unilateral cerebral palsy.Design, Setting, and ParticipantsThis multicenter, randomized, sham-controlled phase 3 trial was conducted from July 2017 through March 2023. Investigators, treating therapists, outcome assessors, parents, and participants were blinded to intervention allocation. The study took place at 3 tertiary care Canadian pediatric rehabilitation hospitals. From a population-based sample of children 6 to 18 years old with perinatal stroke and disabling unilateral cerebral palsy, 196 children were approached and 107 were excluded.InterventionParticipants were randomly assigned in permuted blocks of 2 (1:1) to receive daily sham or cathodal stimulation to the contralesional motor cortex during 10 days of high-dose, child-centered intensive upper-extremity therapy.Main Outcomes and MeasuresThe primary end points were changes from baseline to 6 months posttherapy in affected hand function and attainment of child-identified functional goals assessed by the Assisting Hand Assessment and Canadian Occupational Performance Measure. Safety was assessed, including any decrease in the function of either hand. Analysis was intention to treat.ResultsEighty-nine children were enrolled with 45 randomized to sham (62% male, 38% female; mean [SD] age, 10.7 [2.8] years) and 44 to stimulation (52% male, 48% female; mean [SD] age, 10.7 [2.1] years). Eighty-three participants had complete outcome data (42 sham, 41 stimulation). High proportions of children in both groups demonstrated significant functional gains sustained at 6 months (<jats:italic>P</jats:italic> < .001) with large effect size (Cohen <jats:italic>d</jats:italic> > 1). There were no differences between groups for mean (SD) change in hand function (5.2 [5.3] vs 4.6 [5.7]; <jats:italic>P</jats:italic> = .63) or goal attainment (3.0 [2.0] vs 3.6 [2.3]; <jats:italic>P</jats:italic> = .25). Procedures were safe and well tolerated with no serious adverse events.Conclusions and RelevanceIn this study, results showed that patient-centered intensive motor learning programs could produce marked and sustained improvements in upper-extremity function in children with perinatal stroke and unilateral cerebral palsy. The addition of 1 milliampere contralesional motor cortex transcranial direct current stimulation did not improve outcomes compared with sham stimulation.Trial RegistrationClinicalTrials.gov Identifier: <jats:ext-link xmlns:xlink=\"http://www.w3.org/1999/xlink\" ext-link-type=\"uri\" xlink:href=\"https://clinicaltrials.gov/study/NCT03216837?term=NCT03216837&rank","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":"27 1","pages":""},"PeriodicalIF":29.0,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143077162","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk of Attempted and Completed Suicide in Persons Diagnosed With Headache","authors":"Holly Elser, Dóra Körmendiné Farkas, Cecilia Hvitfeldt Fuglsang, Sissel Toft Sørensen, Henrik Toft Sørensen","doi":"10.1001/jamaneurol.2024.4974","DOIUrl":"https://doi.org/10.1001/jamaneurol.2024.4974","url":null,"abstract":"ImportanceAlthough past research suggests an association between migraine and attempted suicide, there is limited research regarding risk of attempted and completed suicide across headache disorders.ObjectiveTo examine the risk of attempted and completed suicide associated with diagnosis of migraine, tension-type headache, posttraumatic headache, and trigeminal autonomic cephalalgia (TAC).Design, Setting, and ParticipantsThis was a population-based cohort study of Danish citizens from 1995 to 2020. The setting was in Denmark, with a population of 5.6 million people. Persons 15 years and older who were diagnosed with headache were matched by sex and birth year to persons without headache diagnosis with a ratio of 5:1. Data analysis was conducted from May 2023 to May 2024.ExposuresFirst-time headache diagnoses identified from inpatient hospitalizations, emergency department visits, and outpatient specialty clinic visits using <jats:italic>International Statistical Classification of Diseases and Related Health Problems, Tenth Revision </jats:italic>(<jats:italic>ICD-10</jats:italic>) codes.Main Outcomes and MeasuresDiagnostic codes from the <jats:italic>ICD-10</jats:italic> were used to identify attempted suicide from the Danish National Patient Registry and the Danish Psychiatric Central Research Register and completed suicides from the Danish Register of Causes of Death. Absolute risks (ARs) and risk differences (RDs) for attempted and completed suicide were calculated using the cumulative incidence function. Hazard ratios (HRs) for attempted and completed suicide associated with headache diagnosis were computed adjusting for age, sex, year, education, income, baseline comorbidities, and accounting for competing risk of death.ResultsIn total, 119 486 persons (83 046 female [69.5%]) diagnosed with headache were identified and matched with 597 430 persons (415 230 female [69.5%]) drawn from the general population. Participants’ median (IQR) age was 40.1 (29.1-51.6) years. The 15-year AR of attempted suicide among persons diagnosed with headache was 0.78% (95% CI, 0.72%-0.85%) vs 0.33% (95% CI, 0.31%-0.35%) in the comparison cohort (RD, 0.45%; 95% CI, 0.39%-0.53%). The 15-year AR of completed suicide among persons diagnosed with headache was 0.21% (95% CI, 0.17%-0.24%) vs 0.15% (95% CI, 0.13%-0.16%) in the comparison cohort (RD, 0.06%; 95% CI, 0.02%-0.10%). The hazards of attempted suicide (HR, 2.04; 95% CI, 1.84-2.27) and completed suicide (HR, 1.40; 95% CI, 1.17-1.68) were elevated among persons with headache vs comparison cohort members. Findings were consistent across headache types, with stronger associations for TACs and posttraumatic headache.Conclusions and RelevanceResults of this cohort study revealing the robust and persistent association of headache diagnoses with attempted and completed suicide suggest that behavioral health evaluation and treatment may be important for these patients.","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":"15 1","pages":""},"PeriodicalIF":29.0,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143077161","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ischemic Stroke Due to Compression of a Wandering Internal Carotid Artery by the Hyoid Bone.","authors":"François Bouille, Maxime De Malherbe, Fernando Pico","doi":"10.1001/jamaneurol.2024.5028","DOIUrl":"https://doi.org/10.1001/jamaneurol.2024.5028","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":""},"PeriodicalIF":20.4,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143080028","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Quality Improvement Intervention for Reducing Acute Treatment Times in Ischemic Stroke: A Cluster Randomized Clinical Trial.","authors":"Daniël Hansen, Sanne J den Hartog, Nikki van Leeuwen, Jelis Boiten, Wouter Dinkelaar, Pieter J van Doormaal, Frank Eijkenaar, Bart J Emmer, Adriaan C G M van Es, H Zwenneke Flach, Rob Gons, M Heleen den Hertog, Farshad Imani, Paula M Janssen, Hans Kortman, Nyika D Kruyt, Laurien S Kuhrij, Christiaan van der Leij, T H Rob Lo, Aad van der Lugt, Geert Lycklama À Nijeholt, Jasper M M Martens, Paul J Nederkoorn, Jurgen Piet, Michel J M Remmers, Yvo B W E M Roos, Suzanne M Silvis, Lotte J Stolze, Wouter Stomp, Julia H van Tuijl, Martine T B Truijman, Sarah E Vermeer, Marianne A A van Walderveen, Ido R van den Wijngaard, H Bart Van der Worp, Lonneke Yo, Diederik W J Dippel, Hester F Lingsma, Bob Roozenbeek","doi":"10.1001/jamaneurol.2024.4304","DOIUrl":"10.1001/jamaneurol.2024.4304","url":null,"abstract":"<p><strong>Importance: </strong>Efficient care processes are crucial to minimize treatment delays and improve outcome after endovascular thrombectomy (EVT) in patients with ischemic stroke. A potential means to improve care processes is performance feedback.</p><p><strong>Objective: </strong>To evaluate the effect of performance feedback to hospitals on treatment times for EVT.</p><p><strong>Design, setting, and participants: </strong>This cluster randomized clinical trial was conducted from January 1, 2020, to June 30, 2022. Participants were consecutive adult patients with ischemic stroke who underwent EVT in 13 Dutch hospitals. No patients were excluded. Data analysis took place from March to May 2023.</p><p><strong>Intervention: </strong>The intervention consisted of feedback on hospital performance using structure, process, and outcome indicators. Indicator scores were based on data from a national quality registry and compared with a benchmark. Performance feedback was provided through a dashboard for local quality improvement teams who developed and implemented improvement plans based on the feedback. Every 6 months, 3 to 4 randomly selected hospitals switched to the intervention condition.</p><p><strong>Main outcome and measures: </strong>The primary outcome was time from door to groin puncture for all patients treated with EVT. Secondary outcomes included door-to-needle time, National Institutes of Health Stroke Scale (NIHSS) score at day 2, expanded Treatment in Cerebral Infarction (eTICI) score, and modified Rankin Scale (mRS) score at 3 months. The effect of the intervention was estimated with multivariable linear mixed models.</p><p><strong>Results: </strong>A total of 4747 patients were included (intervention: 2431; control: 2316). Their mean (SD) age was 72 (13) years; 2337 (49.2%) were female and 2410 (50.8%) were male. The median (IQR) baseline NIHSS score was 14 (8-19). Median (IQR) door-to-groin puncture time under the intervention condition was 47 (25-71) minutes, compared with 52 (29-75) minutes under the control condition. The adjusted absolute reduction was 5 minutes (β = -4.8; 95% CI, -9.5 to -0.1; P = .04), corresponding to a relative reduction of 9.2% (95% CI, -18.3% to -0.2%).</p><p><strong>Conclusion and relevance: </strong>This study found that performance feedback provided through a dashboard used by local quality improvement teams reduced door-to-groin puncture time for EVT. Implementation of performance feedback in hospitals providing EVT can improve the quality of care for ischemic stroke.</p><p><strong>Trial registration: </strong>The Netherlands Trial Register: NL9090.</p>","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"160-167"},"PeriodicalIF":20.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11811792/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142828321","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Blood-Based Biomarkers for Identifying Disease Activity in AQP4-IgG-Positive Neuromyelitis Optica Spectrum Disorder.","authors":"Su-Hyun Kim, Ana Beatriz Ayroza Galvão Ribeiro Gomes, Patrick Schindler, Jae-Won Hyun, Ki Hoon Kim, Dong-Eun Lee, Vinicius Andreoli Schoeps, Aline de Moura Brasil Matos, Natalia Trombini Mendes, Samira Luisa Dos Apóstolos-Pereira, Dagoberto Callegaro, Jasmine Lerner, Pascal Benkert, Jens Kuhle, Klemens Ruprecht, Friedemann Paul, Anne-Katrin Pröbstel, Ho Jin Kim","doi":"10.1001/jamaneurol.2024.4400","DOIUrl":"10.1001/jamaneurol.2024.4400","url":null,"abstract":"<p><strong>Importance: </strong>The temporal dynamics of serum glial fibrillary acidic protein (sGFAP) and serum neurofilament light chain (sNfL) as biomarkers of disease activity for neuromyelitis optica spectrum disorder (NMOSD) remain underexplored.</p><p><strong>Objective: </strong>To determine optimal timing for assessing sGFAP and sNfL, establish cutoff values differentiating between attacks and remissions in NMOSD, and evaluate these findings across independent cohorts.</p><p><strong>Design, setting, and participants: </strong>This retrospective, longitudinal, multicenter cohort study was conducted among patients with aquaporin-4 antibody (AQP4-IgG)-positive NMOSD. Patients with available stored serum samples were included, totaling 181 patients with 625 samples. Discovery cohort samples were collected from February 2008 to October 2023 and validation cohort samples were collected from January 2013 to October 2023. A combined analysis of both cohorts was conducted from November 2023 to March 2024.</p><p><strong>Exposures: </strong>sNfL and sGFAP concentrations, measured by a single-molecule array assay.</p><p><strong>Main outcomes and measures: </strong>The primary outcomes were the optimal timing of assessing sGFAP and sNfL and the adjusted cutoff values for evaluating disease activity in NMOSD.</p><p><strong>Results: </strong>The discovery cohort consisted of 366 samples from 78 Korean patients (median [IQR] age, 35 [30-42] years; 73 female patients [95%]), while the validation cohort included 190 samples from 34 German patients (median [IQR] age, 54 [39-61] years; 32 female patients [94%]) and 69 samples from 69 Brazilian patients (median [IQR] age, 46 [35-55] years; 62 female patients [90%]). Six-month postattack temporal biomarker dynamics were analyzed in 202 samples from 74 patients in the discovery cohort: sGFAP levels peaked within the first week and sNfL levels peaked at 5 weeks postattack. The optimal time frames for evaluating attacks were within 1 week for sGFAP and from 1 to 8 weeks for sNfL, with remission defined as at least 6 months postattack. z Score cutoffs of 3.0 for sGFAP and 2.1 for sNfL effectively distinguished between attack and remission phases, indicated by area under the curve values of 0.95 (95% CI, 0.88-1.02) and 0.87 (95% CI, 0.82-0.91), respectively. The discovery cohort time frames and cutoff values were applied to the validation cohort, achieving 71% sensitivity and 94% specificity for sNfL and 100% sensitivity and specificity for sGFAP in the German and Brazilian cohorts.</p><p><strong>Conclusions and relevance: </strong>This longitudinal cohort study established optimal timing and thresholds for sGFAP and sNfL, which were consistent in independent cohorts, supporting these biomarkers' effectiveness in distinguishing NMOSD attacks from remission.</p>","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"168-175"},"PeriodicalIF":20.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11811799/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142876775","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Improved Measurement of Treatment Effect in Multiple Sclerosis Clinical Trials.","authors":"Alessio Signori, Francesca Bovis, Maurizio Fedriga, Noemi Montobbio, Marta Ponzano, Irene Schiavetti, Maria Pia Sormani","doi":"10.1001/jamaneurol.2024.4396","DOIUrl":"10.1001/jamaneurol.2024.4396","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"204-206"},"PeriodicalIF":20.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11811791/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142876858","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neurological Pupil Index and Intracranial Hypertension in Patients With Acute Brain Injury: A Secondary Analysis of the ORANGE Study.","authors":"Matteo Petrosino, Elisa Gouvêa Bogossian, Paola Rebora, Stefania Galimberti, Randall Chesnut, Pierre Bouzat, Mauro Oddo, Fabio Silvio Taccone, Giuseppe Citerio","doi":"10.1001/jamaneurol.2024.4189","DOIUrl":"10.1001/jamaneurol.2024.4189","url":null,"abstract":"<p><strong>Importance: </strong>Invasive intracranial pressure (ICP) is the standard of care in patients with acute brain injury (ABI) with impaired consciousness. The Neurological Pupil Index (NPi) obtained by automated pupillometry is promising for noninvasively estimating ICP.</p><p><strong>Objectives: </strong>To evaluate the association between repeated NPi and invasive ICP values.</p><p><strong>Design, setting, and participants: </strong>This study is a secondary analysis of the Outcome Prognostication of Acute Brain Injury With the Neurological Pupil Index (ORANGE), a multicenter, prospective, observational study of patients with ABI performed from October 1, 2020, to May 31, 2022, with follow-up at 6 months after ABI. The ORANGE study was performed at neurologic intensive care units of tertiary hospitals in Europe and North America. In ORANGE, 514 adult patients receiving mechanical ventilatory support were admitted to the intensive care unit after ABI.</p><p><strong>Exposure: </strong>Invasive ICP monitoring and automated pupillometry assessment every 4 hours during the first 7 days, considered as a standard of care.</p><p><strong>Main outcomes and measures: </strong>Association between ICP and NPi values over time, using bayesian joint models, with linear and logistic mixed-effects longitudinal submodels.</p><p><strong>Results: </strong>The study included 318 adult patients (median [IQR] age, 58 [43-69] years; 187 [58.8%] male) who required intensive care unit admission, intubation, and mechanical ventilatory support due to acute traumatic brain injury (n = 133 [41.8%]), intracerebral hemorrhage (n = 104 [32.7%]), or aneurysmal subarachnoid hemorrhage (n = 81 [25.5%]) and had automatic infrared pupillometry used as part of the standard evaluation practice and ICP monitoring. A total of 8692 ICP measurements were collected, with a median (IQR) of 31 (18-37) evaluations per patient. The median (IQR) NPi and ICP for the study population were 4.1 (3.5-4.5) and 10 (5-14) mm Hg, respectively. In a linear mixed model, the mean change in the NPi value, as a continuous variable, was -0.003 (95% credible interval [CrI], -0.006 to 0.000) for each 1-mm Hg ICP increase. No significant association between ICP and abnormal NPi (<3; odds ratio, 1.01; 95% CrI, 0.99-1.03) or absent NPi (0; odds ratio, 1.03; 95% CrI, 0.99-1.06) was observed.</p><p><strong>Conclusions and relevance: </strong>Although an abnormal NPi could indicate brainstem dysfunction, in this large and heterogeneous population of patients, NPi values were not significantly associated overall with ICP values. Repeated NPi measurements may not be a sufficient replacement for invasive monitoring.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov Identifier: NCT04490005.</p>","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"176-184"},"PeriodicalIF":20.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11811798/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142800641","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Presentation, Investigation Findings, and Outcomes of IgG4-Related Pachymeningitis: A Systematic Review.","authors":"Sara Terrim, João Vitor Mahler, Flávio Vieira Marques Filho, Leandro Tavares Lucato, Henrique Mayrink Giardini, Tarso Adoni, Guilherme Diogo Silva","doi":"10.1001/jamaneurol.2024.3947","DOIUrl":"10.1001/jamaneurol.2024.3947","url":null,"abstract":"<p><strong>Importance: </strong>Immunoglobulin G4 (IgG4)-related disease is an increasingly recognized fibroinflammatory condition that can involve multiple organs, including the pachymeninges. The understanding of IgG4-related pachymeningitis (IgG4-RP) remains limited because of its rarity and the predominance of knowledge derived from case reports and case series.</p><p><strong>Objective: </strong>To systematically review and synthesize the clinical presentation, investigation findings, and prognosis of IgG4-RP to better understand its diagnosis and management.</p><p><strong>Evidence review: </strong>A comprehensive systematic review was conducted following guidelines from the Preferred Reporting Items for Systematic Reviews and Meta-analyses. PubMed/MEDLINE, Embase, and Scopus were searched from their inception until May 30, 2023, using terms related to IgG4-related disease and pachymeningitis without language or publication restrictions. Case reports and series that met the 2020 Revised Comprehensive Diagnostic Criteria or the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria were included. Data on clinical presentations, investigation findings, and treatment outcomes were extracted and summarized.</p><p><strong>Findings: </strong>A total of 148 case reports contributed data from 208 patients. Their median (IQR) age was 52 (39-62) years; 132 patients were male (63.5%) and 76 female (36.5%). Headache and cranial nerve dysfunctions were the most common neurological manifestations. Systemic involvement was identified in nearly half of the patients. Diagnostic imaging often showed preferential involvement of cavernous sinus and middle fossa. Laboratory results highlighted elevated serum IgG4 levels in 97 of 147 patients (65%) of patients and cerebrospinal fluid pleocytosis in 43 of 82 patients (52%). Storiform fibrosis or obliterating phlebitis were uncommon pathological findings. Mortality was below 1% (1/134; 0.7%), but only a third of patients presented complete clinical improvement, and the recurrence rate was 60 patients (40%) in a median (IQR) follow-up time of 9 (1-20) months. Glucocorticoids were the most commonly prescribed treatment, in 143 of 169 patients (85%); rituximab was prescribed as maintenance therapy in 53 of 169 patients (31%).</p><p><strong>Conclusions and relevance: </strong>IgG4-RP commonly presents with headaches and cranial nerve dysfunction, posing diagnostic challenges due to the significant absence of systemic manifestations, low IgG4 serum levels, and atypical pathological findings. Current treatment outcomes are limited by incomplete recovery and frequent relapses underscoring the necessity for new treatment strategies.</p>","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"193-199"},"PeriodicalIF":20.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142647943","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Londonderry Air.","authors":"Calvin G Ludwig","doi":"10.1001/jamaneurol.2024.4481","DOIUrl":"10.1001/jamaneurol.2024.4481","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"121-122"},"PeriodicalIF":20.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142931794","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intracranial Metastatic Tumor Hemorrhage During MRI Scanning.","authors":"Wei Rao, Jin-Long Mao, Wei-Dong Cao","doi":"10.1001/jamaneurol.2024.4531","DOIUrl":"10.1001/jamaneurol.2024.4531","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"202-203"},"PeriodicalIF":20.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142931789","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}