Annals of Clinical and Translational Neurology最新文献

{"title":"Temporal Divergence of True and False Alarms in Pediatric Nocturnal Seizure Monitoring.","authors":"Mohammad Shahbakhti, Anemoon T Bosch, Job van der Palen, Franciscus S Leijten, Johannes P van Dijk, Richard H C Lazeron, Roland D Thijs","doi":"10.1002/acn3.70530","DOIUrl":"10.1002/acn3.70530","url":null,"abstract":"<p><p>Seizure detection devices can improve the safety of children with epilepsy, yet high false alarms limit their clinical applicability. Here, we investigated whether specific time periods are disproportionately prone to false alarms. We analyzed 2876 NightWatch alarms occurring between 19:00 and 09:00 in 65 children (ages 3-16, 48% female). Temporal distributions differed significantly between true and false alarms (p < 0.001), with a significant divergence in skewness (p = 0.001); notably, 30% of false alarms occurred between 06:00 and 09:00. This suggests NightWatch performance is adversely affected by wakefulness and morning arousal. Our findings support exploring time- or state-dependent detection thresholds to reduce false alarms. Trial Registration: The PROMISE (NCT03909984) and LICSENSE (NTR4115/NR395) trials were registered at the Dutch Trial Registry.</p>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2026-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13621364/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149072912","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"microRNA-7-5p and α-Synuclein SAA Predict Parkinson's Disease Phenoconversion.","authors":"Shayan Zadegan, Praveen Velammal, Kanmani Muthiah, Madhu Jatty, Christopher Adams","doi":"10.1002/acn3.70539","DOIUrl":"10.1002/acn3.70539","url":null,"abstract":"<p><strong>Objective: </strong>Corroborate blood neuron-derived extracellular vesicle (NDEV) alpha-synuclein (αSyn), the CSF αSyn seed amplification assay (αSyn-SAA), and blood microRNA-7-5p (miR-7-5p) as markers for Parkinson's disease (PD) phenoconversion and determine if combining these markers would help select subjects who would be more likely to phenoconvert.</p><p><strong>Methods: </strong>Using Parkinson Progression Marker Initiative data, time to PD phenoconversion was determined by Hoehn and Yahr scores. Log-rank analysis was used to find cutpoints. Cox regression analysis was used with log[miR-7-5p], log[NDEV αSyn], and the αSyn-SAA. Models included age and sex.</p><p><strong>Results: </strong>In a Cox model with baseline log[miR-7-5p], for each unit increase in log[miR-7-5p], there was 1.57 times increased PD phenoconversion (p = 0.003). In a model with baseline log[NDEV αSyn], only baseline age was associated with increased phenoconversion (p < 0.05). Using Cox regression analysis, the cutpoints for miR-7-5p and NDEV αSyn were 27.39 reads per million mapped (p < 0.01) and 18.36 pg/mL (p = 0.01), respectively. In separate models using log[miR-7-5p] and log[NDEV αSyn] cutpoints, higher levels of log[miR-7-5p] and log[NDEV αSyn] resulted in 2.26 (p < 0.001) and 4.46 (p < 0.05) times increased PD phenoconversion, respectively. A positive αSyn-SAA was associated with 3.62 times increased phenoconversion (p < 0.001). Having log[miR-7-5p] higher than its cutpoint and a positive αSyn-SAA was associated with 4.31 times phenoconversion (p < 0.001).</p><p><strong>Interpretation: </strong>Blood miR-7-5p, NDEV αSyn, and αSyn-SAA could be used to enrich for prodromal patients likely to develop PD earlier in disease-modifying clinical trials and combining them could further enrich for this population.</p>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13614880/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149056609","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Utility of the APE² Score as a Diagnostic Tool for Autoimmune Encephalitis.","authors":"Bijoya Basu, Sophia F Damman, Samhitha M Rai, Hesham Abboud","doi":"10.1002/acn3.70526","DOIUrl":"10.1002/acn3.70526","url":null,"abstract":"<p><strong>Objective: </strong>To retrospectively evaluate the diagnostic performance of the Antibody Prevalence in Epilepsy and Encephalopathy (APE²) score relative to clinician-adjudicated autoimmune encephalitis (AE) and the Graus criteria in a tertiary neuroimmunology referral cohort, including antibody-negative AE.</p><p><strong>Methods: </strong>We conducted a retrospective single-center study of consecutive referrals to a tertiary neuroimmunology clinic (January 2017-May 2023). AE diagnosis was determined by expert consensus based on clinical features, investigations, exclusion of alternative diagnoses, and immunotherapy response. APE² scores and Graus categories, were assigned retrospectively; APE2SM [APE2 seizure/movement] was examined as a post hoc exploratory secondary analysis. Diagnostic performance was assessed using ROC analysis and contingency tables, and logistic regression modeled the probability of AE across scores within this study cohort.</p><p><strong>Results: </strong>Eighty-five patients were included (56.5% female; mean age 53.8 years). Fifty-nine (69.4%) had AE (34 antibody-positive). APE² demonstrated higher discriminative performance than Graus categories in this cohort (AUC 0.894 vs. 0.804) and in antibody-negative patients (AUC 0.905 vs. 0.850). In both models, an APE² cutoff ≥ 4 balanced sensitivity and specificity near or above 80%, whereas in the full patient cohort no Graus criteria threshold achieved a similar balance. Probability modeling showed APE² ≥ 4 crossed 80% in all patients and 60% in antibody-negative patients within this cohort. Exploratory analysis showed that APE2SM had overall discrimination comparable to the original APE² score, with a slightly lower AUC in the full cohort (0.888 vs. 0.894).</p><p><strong>Interpretation: </strong>APE² score demonstrated good diagnostic discrimination and may represent a useful adjunctive tool for early AE evaluation, including antibody-negative cases. The exploratory APE2SM model may further enhance sensitivity; however, prospective multicenter validation is needed before routine clinical implementation.</p>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13614881/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149056635","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ofatumumab Therapy and Long-Term Seizure Outcomes of Patients With Relapsing Anti-NMDA Receptor Encephalitis.","authors":"Jian Wang, Mengjiao Li, Ping Kong","doi":"10.1002/acn3.70537","DOIUrl":"10.1002/acn3.70537","url":null,"abstract":"<p><strong>Objective: </strong>There is a lack of studies on seizure outcomes associated with ofatumumab therapy in patients for relapsing anti-N-methyl-D-aspartate (NMDA) receptor encephalitis. We aimed to evaluate long-term seizure outcomes of ofatumumab therapy for relapsing anti-NMDA receptor encephalitis.</p><p><strong>Methods: </strong>This is a prospective cohort study, Compared patients with relapsing anti-NMDA receptor encephalitis who had received at least 1 ofatumumab dose and a control cohort of non-ofatumumab-treated patients, continuous follow-up for 24 months. The primary outcome was the seizure outcomes (including both seizure reduction and seizure freedom; seizure reduction is defined as a reduction in seizure frequency to 50% or more of the baseline level, seizure freedom is defined as having no seizures) at the follow-up. The secondary outcomes included modified Rankin Scale (mRS) scores and Clinical Assessment Scale in Autoimmune Encephalitis (CASE) scores over the 24-month follow-up.</p><p><strong>Results: </strong>We enrolled 93 patients with relapsing anti-NMDA receptor encephalitis. A total of 76 patients met the requirements after the inclusion and exclusion criteria. The 38 patients received ofatumumab therapy, and the other 38 patients received non-ofatumumab therapy. Follow-up after 6 months, the ofatumumab therapy group demonstrated favorable seizure outcomes (63.16% vs. 36.84%, p < 0.05). Follow-up after 12 months, this improvement of seizure outcomes was more pronounced (78.95% vs. 60.53%, p < 0.05). Follow-up after 24 months, this difference between the two groups widened further (94.74% vs. 78.95%, p < 0.05), compared with the non-ofatumumab therapy group. Follow-up after 6 months, the ofatumumab group demonstrated a faster mRS scores (2.85 ± 0.65 vs. 3.75 ± 0.55, p < 0.05) and CASE scores (11.80 ± 1.65 vs. 13.75 ± 1.55, p < 0.05). Follow-up after 12 months, the ofatumumab group demonstrated a faster mRS scores (1.68 ± 0.45 vs. 2.39 ± 0.35, p < 0.05) and CASE scores (6.75 ± 1.45 vs. 9.39 ± 1.35 p < 0.05). Follow-up after 24 months, the ofatumumab group demonstrated a faster mRS scores (1.25 ± 0.35 vs. 1.68 ± 0.45, p < 0.05) and CASE scores (4.25 ± 0.75 vs. 6.68 ± 1.35, p < 0.05). And no serious adverse events associated with ofatumumab treatment were reported. Furthermore, Multivariate logistic regression analysis identified that early initiation of ofatumumab therapy, psychiatric symptoms, and abnormal brain MRI findings may be associated with seizure outcomes.</p><p><strong>Conclusions: </strong>Ofatumumab showed substantial improvement in long-term seizure outcomes in patients with relapsing anti-NMDA receptor encephalitis, particularly those presenting with seizures as a predominant clinical feature.</p>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13613457/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149035665","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Systemic Extracerebral Atherosclerotic Burden and Dementia Risk After Incident Stroke.","authors":"Zhexuan Yu, Tianxue Chen, Wei Jin, Weifeng Jin, Haitong Wan","doi":"10.1002/acn3.70540","DOIUrl":"10.1002/acn3.70540","url":null,"abstract":"<p><strong>Background: </strong>Stroke survivors are at increased risk of dementia, but clinically accessible markers of risk heterogeneity remain limited. Previous studies have largely examined atherosclerotic disease in individual vascular beds rather than the overall burden of systemic extracerebral atherosclerosis.</p><p><strong>Methods: </strong>We included UK Biobank participants without stroke at baseline who subsequently developed incident stroke and remained under follow-up and dementia-free 1 year after the index event. Extracerebral atherosclerotic vascular disease (ECAVD) was evaluated according to its overall presence, the number of affected vascular beds, and the mutually adjusted contributions of carotid artery stenosis, coronary heart disease (CHD), and peripheral artery disease. Fine-Gray subdistribution hazard models were used with death treated as a competing event. Missing covariate data were addressed using multiple imputation.</p><p><strong>Results: </strong>Among 9571 participants, 584 developed all-cause dementia during follow-up. ECAVD was associated with a higher risk of dementia (subdistribution hazard ratio [sHR], 1.38; 95% confidence interval [CI] 1.13-1.68; p = 0.0014). The adjusted 5-year risks were 4.4% and 3.2% in participants with and without ECAVD, respectively, and the corresponding 10-year risks were 11.1% and 8.1%. Compared with no affected vascular beds, involvement of 1 vascular bed was associated with a higher dementia risk (sHR, 1.41; 95% CI 1.15-1.72), whereas the estimate for ≥ 2 affected beds was not statistically significant (sHR, 1.19; 95% CI 0.76-1.87). The per-category trend was significant (sHR, 1.24; 95% CI 1.07-1.45). In mutually adjusted analyses, CHD was associated with dementia (sHR, 1.42; 95% CI 1.13-1.78), whereas peripheral artery disease and carotid artery stenosis were not. No significant interaction was observed by stroke subtype or dementia subtype. Lipid-adjustment and complete-case analyses yielded similar results.</p><p><strong>Conclusions: </strong>ECAVD was associated with a higher risk of all-cause dementia diagnosed more than 1 year after incident stroke. ECAVD may provide a practical indicator of cumulative systemic vascular burden and long-term cognitive risk, although vascular-bed count findings should be interpreted cautiously.</p>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13602991/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149035715","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Autoimmune Comorbidities as Modifiers of Phenotypic Heterogeneity in Facioscapulohumeral Dystrophy.","authors":"Jonathan Pini, Giulia Tammam, Andra Ezaru, Manuela Gambella, Benoît Sanson, Luisa Villa, Michele Cavalli, Mihai-Bogdan Ioncea, Angela Puma, Sabrina Sacconi","doi":"10.1002/acn3.70529","DOIUrl":"10.1002/acn3.70529","url":null,"abstract":"<p><strong>Objective: </strong>Facioscapulohumeral dystrophy type 1 (FSHD1) shows clinical heterogeneity that is only partly explained by D4Z4 repeat unit (RU) size. Although immune and inflammatory mechanisms may contribute to disease variability, the prevalence and clinical impact of autoimmune diseases in FSHD remain unclear. We investigated the spectrum of autoimmune comorbidities in FSHD1 and their relationship with D4Z4 repeat size, clinical phenotype, and disease severity.</p><p><strong>Methods: </strong>We retrospectively analyzed 299 genetically confirmed FSHD1 patients followed at a national neuromuscular reference center. Clinical severity was assessed using the FSHD score and Comprehensive Clinical Evaluation Form classification. Demographic, genetic, and clinical features were compared according to autoimmune disease status. Multivariable linear regression assessed the independent association between autoimmune disease and FSHD severity, adjusting for age, sex, disease duration, D4Z4 RU number, and clinical phenotype.</p><p><strong>Results: </strong>Eighty-two patients (27.4%) had at least one autoimmune disease, totaling 96 autoimmune conditions. Several autoimmune diseases were markedly overrepresented compared with published population estimates. Patients with autoimmune disease had larger D4Z4 repeat arrays (7.18 ± 1.82 vs. 6.53 ± 1.81 RU, p = 0.002) but higher FSHD severity scores (7.98 ± 3.65 vs. 6.56 ± 3.51, p = 0.003). Autoimmune comorbidities were enriched in patients carrying 7-10 D4Z4 RU. In multivariable analysis, autoimmune disease remained independently associated with greater FSHD severity (β = 2.12, 95% CI 1.47-2.76, p < 0.0001).</p><p><strong>Conclusions: </strong>Autoimmune diseases are frequent in FSHD1 and independently associated with greater severity despite larger D4Z4 repeat arrays, supporting immune-related mechanisms as potential modifiers of FSHD1 expression.</p>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2026-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13595050/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149014895","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Changes in Immune-Inflammation Status and Prognosis in Pregnancy-Related Cerebral Venous Thrombosis.","authors":"Xiaoming Zhang, Zilin Xu, Ran Duan, Chunxiao Lu, Da Zhou, Chen Zhou, Ran Meng, Xunming Ji","doi":"10.1002/acn3.70518","DOIUrl":"10.1002/acn3.70518","url":null,"abstract":"<p><strong>Objective: </strong>Distinguishing pathological changes from physiological adaptations in pregnancy-related cerebral venous thrombosis (CVT) is clinically challenging. This study aimed to characterize coagulation, immune-inflammation, and dehydration status in these patients and assess their prognostic value.</p><p><strong>Methods: </strong>In this retrospective study, 120 participants were categorized into four groups: CVT with pregnancy-puerperium, CVT without pregnancy-puerperium, pregnancy-puerperium without CVT, and healthy nonpregnant female controls (30 per group). Laboratory indices were compared across groups. Neurological severity and functional outcomes were assessed using the National Institutes of Health Stroke Scale (NIHSS) and the modified Rankin Scale (mRS) at admission, 7 days, and 3 months.</p><p><strong>Results: </strong>Compared with CVT without pregnancy-puerperium, CVT with pregnancy-puerperium presented earlier after symptom onset and exhibited more severe neurological manifestations. CVT with pregnancy-puerperium showed higher fibrinogen and D-dimer levels than CVT without pregnancy-puerperium and healthy nonpregnant controls, but these markers overlapped with healthy pregnancy-puerperium controls. Several inflammatory markers were elevated compared with non-pregnancy groups, while neutrophil count, NLR, SII, and CRP overlapped with healthy pregnancy-puerperium controls. Elevated urine specific gravity was more frequent in CVT with pregnancy-puerperium than in healthy pregnancy-puerperium controls. Within this group, D-dimer and inflammatory markers correlated positively with NIHSS and mRS scores at admission and 7 days. Plasma osmolality > 310 mOsm/(kg·H₂O) was associated with worse neurological outcomes at 7 days and 3 months.</p><p><strong>Interpretation: </strong>CVT during pregnancy-puerperium occurs against pregnancy-related hypercoagulability, immune-inflammatory activation, and fluid-balance changes, with overlap between pathological CVT-related changes and physiological pregnancy adaptations. D-dimer, inflammatory indices, and dehydration-related parameters may provide prognostic information for risk stratification.</p>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2026-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13584609/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148987338","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cerebrospinal Fluid Over Plasma Links Analytes to Cognitive Decline in Older Adults at Risk for Alzheimer's Disease.","authors":"Jagan A Pillai, Audrey Zhu, Chunxuan Ma, Ming Wang, Yifan Yang, Lynn M Bekris, Maria Khrestian, Frank P DiFilippo, Mikhail Nasrallah, Aaron Burberry, James B Leverenz, Yuan Hou, Feixiong Cheng, Stephen M Rao","doi":"10.1002/acn3.70532","DOIUrl":"10.1002/acn3.70532","url":null,"abstract":"<p><strong>Objective: </strong>To identify inflammatory analytes in cerebrospinal fluid (CSF) and plasma associated with cognitive decline in cognitively normal (CN) older adults at risk for Alzheimer's disease (AD).</p><p><strong>Methods: </strong>In a longitudinal study of 118 CN older adults (65-80 years, 54% APOE ε4, 26% preclinical AD), 1331 CSF and 1501 plasma analytes were quantified at baseline and 2-year follow-up using SomaLogic, with key inflammatory findings validated on the Luminex platform. Linear models (Limma), adjusted for age, sex, APOEε4, and amyloid-positive status, identified pathology-associated analytes. Co-expression network and multivariable modeling defined hub analytes and enriched pathways. To assess robustness, a targeted panel of 94 inflammation-related analytes was analyzed using best subsets regression to derive parsimonious models based on adjusted R² improvement (≥ 0.01) and ≥ 5 observations per predictor. Subgroup analyses by amyloid and APOE ε4 status were performed, and a two-stage elastic-net approach additionally validated analyte selection.</p><p><strong>Results: </strong>CSF proteomics revealed stronger APOEε4 and amyloid-associated analyte signatures than plasma. Forty-four CSF analytes were co-regulated by APOEε4 and amyloid-positive status, forming central network hubs (e.g., EFNB2, NPTN, UNC5D) enriched in axon guidance, synaptic signaling, and extracellular matrix pathways. In contrast, inflammatory analytes including eotaxin-1 and IL-17 pathway-related molecules were associated with longitudinal cognitive decline, with stronger effects observed in females. Eotaxin-1 demonstrated the most consistent predictive performance across analytic methods, subsets, and assay platforms, whereas network hub analytes were not predictive of cognitive outcomes.</p><p><strong>Interpretation: </strong>CSF reflects strong APOEε4/amyloid related proteomic network alterations linked to cognitive decline, while plasma provides weaker but complementary signals. CSF inflammatory signaling, particularly eotaxin-1, may serve as a correlate of longitudinal cognitive decline in cognitively normal older adults at risk for AD.</p>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2026-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13586066/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148987354","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Early Clinical and Cerebrospinal Fluid Predictors of 1-Year Recurrence in Autoimmune GFAP Astrocytopathy.","authors":"Qingting Hong, Yuping Xiao, Yuhan Wu, Ziyuan Huang, Weishan Liang, Li Xiao, Yaqing Shu, Tingting Lu, Yanyu Chang, Wei Qiu, Yuge Wang","doi":"10.1002/acn3.70535","DOIUrl":"10.1002/acn3.70535","url":null,"abstract":"<p><strong>Objective: </strong>Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is an inflammatory central nervous system disorder with variable outcomes. Relapse occurs in a subset of patients, but early predictors remain unclear. We aimed to identify admission-available features associated with 1-year recurrence and develop an interpretable risk stratification model.</p><p><strong>Methods: </strong>We retrospectively included 156 patients with CSF GFAP-IgG-positive GFAP-A between January 2021 and February 2025. Patients were classified by recurrence within 1 year. Early demographic, clinical, CSF, serological, neuroimaging, and treatment-related variables were compared between groups. Candidate predictors were screened using elastic net regression with repeated cross-validation, followed by multivariable logistic regression and internal validation.</p><p><strong>Results: </strong>Thirty-five patients (22.4%) relapsed within 1 year. Patients with and without recurrence within 1 year were broadly comparable in age, sex, baseline severity, neuroimaging findings, and treatment exposure. Patients with recurrence within 1 year had lower CSF white blood cell counts (42.0 [10.0-104.0] vs. 107.0 [34.0-200.0] × 10⁶/L; p = 0.002) and more frequent movement disorders (71.4% vs. 43.8%; p = 0.004). The final model included CSF white blood cell count, movement disorders, CSF chloride, and CSF GFAP-IgG titer category. Lower CSF white blood cell count, movement disorders, and higher GFAP-IgG titer category were associated with increased recurrence risk. The model showed acceptable optimism-corrected discrimination (AUC, 0.726), reasonable calibration, and potential clinical utility. Machine-learning models did not outperform logistic regression.</p><p><strong>Interpretation: </strong>In GFAP-A, 1-year recurrence was associated with distinct early clinical and CSF features rather than baseline severity or treatment exposure alone. This admission-based model may support recurrence risk stratification and follow-up planning.</p>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2026-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13583092/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148981709","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diffuse MRI Edema Predicts Relapse in Cerebral Amyloid Angiopathy-Related Inflammation.","authors":"G Abbas Kharal, Gowtham Yerneni, Kim J Griffin, Nicolas Thompson, Ken Uchino, Irene Katzan, Dolora Wisco, Abeer Farrag, Jayashree Sundarajan, Andrew Russman, M Shazam Hussain","doi":"10.1002/acn3.70525","DOIUrl":"10.1002/acn3.70525","url":null,"abstract":"<p><strong>Objective: </strong>To identify MRI predictors of relapse and quantify relapse and mortality risk in cerebral amyloid angiopathy-related inflammation (CAA-RI). A secondary objective was to assess the association between mycophenolate mofetil use and relapse risk.</p><p><strong>Methods: </strong>We performed a retrospective cohort study of 36 patients with CAA-RI treated at the Cleveland Clinic from January 2020 to December 2024. Relapse was defined as recurrent vasogenic edema on MRI after corticosteroid treatment. Edema was classified as focal (1 lobar region), multifocal (2-3 regions), or diffuse (≥ 4 regions). Cause-specific Cox models and Gray's test evaluated predictors of relapse and mortality, and the effect of mycophenolate on relapse.</p><p><strong>Results: </strong>Among 36 patients (mean age 71.6 ± 8.2 years; 66.7% female), 11 (30.6%) relapsed, 9 (25.0%) died without having relapse, and 16 (44.4%) had neither event after a median prednisone taper of 4.5 months. 31 patients had follow-up data for time-to-event analyses, with a median follow-up time of 18.7 months. Cumulative relapse rates were 22.4%, 26.3%, and 44.0% at 6, 12, and 24 months, respectively. Diffuse edema strongly predicted relapse (hazard ratio [HR] = 12.26; 95% confidence interval [CI] = 1.95-76.94; p = 0.007). Mycophenolate was used in 11 patients; relapse occurred in 18.2% versus 30.0% without mycophenolate treatment. Initiation of mycophenolate at steroid taper in high-relapse-risk patients was associated with a statistically nonsignificant reduction in relapse risk (HR = 0.36; 95% CI = 0.07-1.09, p = 0.231).</p><p><strong>Interpretation: </strong>Relapse in CAA-RI is common and extends beyond the early post-treatment phase. Diffuse edema on baseline MRI strongly predicts recurrence, enabling effective risk stratification. Mycophenolate shows promise as a steroid-sparing therapy for preventing relapse in high-risk patients and merits prospective evaluation.</p>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2026-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13562774/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148928477","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}