Neurology. Clinical practice最新文献

{"title":"Grounded in Evidence.","authors":"Lyell K Jones","doi":"10.1212/CPJ.0000000000200470","DOIUrl":"https://doi.org/10.1212/CPJ.0000000000200470","url":null,"abstract":"","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 3","pages":"e200470"},"PeriodicalIF":2.3,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11962046/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143780654","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Implementation of 2HELPS2B Seizure Risk Score: A Cost-Effective Approach to Seizure Detection in the Intensive Care Units.","authors":"Fazila Aseem, Emily Fink, Chuning Liu, John Whalen, Jessica Werdel, Parin Nanavati, Fei Zou, Angela Wabulya, Casey Olm-Shipman, Suzette Maria LaRoche, Clio Rubinos","doi":"10.1212/CPJ.0000000000200464","DOIUrl":"10.1212/CPJ.0000000000200464","url":null,"abstract":"<p><strong>Background and objectives: </strong>Continuous EEG (cEEG) has become a standard for monitoring critically ill patients, but it is resource-intensive with limited availability. The 2HELP2B seizure risk score can help stratify seizure risk and aid in clinical decision making to optimize duration of monitoring. This study aimed to incorporate the 2HELPS2B score to inform cEEG duration and provide cost-effective care without compromising seizure detection.</p><p><strong>Methods: </strong>We conducted a quality improvement study that targeted clinical workflow and seizure risk stratification in the intensive care units of a tertiary academic hospital. The study included adult patients who underwent cEEG between June 2020 and December 2022 (n = 552), after excluding patients undergoing cEEG for management of status epilepticus, spell characterization, intracranial pressure monitoring, and post-cardiac arrest (n = 129). We performed a retrospective chart review to establish baseline cEEG volume, seizure incidence, and monitoring duration. We then introduced the 2HELPS2B risk score through multidisciplinary education and used published recommendations to suggest optimal cEEG duration. After the intervention, we analyzed the impact of integrating the 2HELPS2B score on cEEG duration and seizure detection rates.</p><p><strong>Results: </strong>Of 552 patients, most were low risk (n = 311, 56.3%), followed by moderate risk (n = 189, 34.2%) and high risk (n = 52, 9.4%). Before the intervention, cEEG duration was similar for all risk groups. After implementation of the 2HELPSB score, there was a significant reduction in cEEG duration for low-risk and moderate-risk patients (low 36.3 vs 23.8 hours; <i>p</i> < 0.0001, moderate 36.5 vs 29.3 hours; <i>p</i> = 0.01) and no significant change for the high-risk group (41.3 vs 40.4 hours; <i>p</i> = 0.92). Seizure detection was low except for the high-risk group (1.3% vs 7.9% vs 39.1%). Reduction in cEEG duration after implementation of the 2HELPS2B score did not lead to a significant change in seizure detection (0.6% vs 9% vs 37.9%).</p><p><strong>Discussion: </strong>Most critically ill patients had low or moderate seizure risk and, accordingly, a low incidence of seizures detected during cEEG. Implementing the 2HELPS2B seizure risk score allowed customization of cEEG duration for individual patients, applying the practice of precision medicine. This approach successfully improved cEEG utilization without compromising seizure detection. In conclusion, implementing seizure risk stratification can provide cost-effective monitoring and improve cEEG access.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 3","pages":"e200464"},"PeriodicalIF":2.3,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11962049/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143780657","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevention of Cerebrovascular Emergencies in Spaceflight: A Review and a Proposal for Enhanced Medical Screening Guidelines.","authors":"Mark J Rosenberg, Brian F Saway, William J Tarver, James H Pavela, Jacob Hall, Sami Al Kasab, Guilherme Porto, Donna R Roberts","doi":"10.1212/CPJ.0000000000200445","DOIUrl":"10.1212/CPJ.0000000000200445","url":null,"abstract":"<p><strong>Purpose of review: </strong>A growing number of opportunities for paying customers to travel to space are becoming available. Preflight medical screening of these potential private astronauts will likely be performed by local physicians, with referral to specialists in aerospace medicine as required for more in-depth evaluation before flight qualification. Neurologic concerns contribute a portion of the potential medical risks for these private astronauts, especially with the participation of more diversified crews than traditional governmental astronauts. The objective of this article was to review the current knowledge base concerning cerebrovascular adaptation to spaceflight to inform risk factor assessment for flight-associated cerebrovascular emergencies by the neurologic community when performing initial medical screening of potential private astronauts.</p><p><strong>Recent findings: </strong>A review of published human spaceflight studies and medical guidelines regarding cerebrovascular risks for spaceflight was conducted. Most of the available literature describes cohorts of a small number of astronauts undergoing spaceflight missions of various flight profiles. While there are gaps in the literature, cerebrovascular adaptation to spaceflight occurs, which may alter the medical risk profile in susceptible individuals. The occurrence of an inflight cerebrovascular emergency could have devastating consequences; therefore, additional screening tests may be required, based on risk level and mission profile, in assessing the more diverse commercial spaceflight population expected over the next decade.</p><p><strong>Summary: </strong>With increasing interest in commercial space tourism among diverse participant populations, the stroke risk in microgravity/reduced gravity environments is unknown. Furthermore, stresses of rocket ascent/descent, abnormal fluid dynamics in microgravity, altered atmospheric conditions, and other unknown occupational hazards add additional complexity. Because inflight emergency management protocols have yet to be developed, the most effective tool to ensure spaceflight participant safety is comprehensive preflight preventative screenings. Determining neurologic risk factors is critical for developing evidence-based guidelines for preventative measures and treatment protocols in the future.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 3","pages":"e200445"},"PeriodicalIF":2.3,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11966524/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143780660","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Suicidal Ideation and Sleep Disturbances Among People With Huntington Disease: Evidence From the HDBOI Study.","authors":"Idaira Rodríguez Santana, Samuel A Frank, Tiago A Mestre, Astri Arnesen, Jamie L Hamilton, Hayley Hubberstey, Michaela Winkelmann, Elena Hernandez-Jimenez, Jeff Frimpter, Ricardo Dolmetsch, Talaha M Ali","doi":"10.1212/CPJ.0000000000200461","DOIUrl":"10.1212/CPJ.0000000000200461","url":null,"abstract":"<p><strong>Background and objectives: </strong>Suicidal ideation and sleep disturbances are more common among people with Huntington disease (PwHD) than otherwise healthy peers; however, the scope and magnitude of these challenges are not well understood. This study evaluated suicidal thoughts and sleep disturbances among PwHD in Europe and the United States using data from the Huntington's Disease Burden of Illness (HDBOI) study.</p><p><strong>Methods: </strong>The HDBOI study is a cross-sectional burden-of-illness study of PwHD in France, Germany, Italy, Spain, the United Kingdom, and the United States. Eligible participants were adults (18 years and older) with motor manifest Huntington disease (HD) ≥ 12 months before study recruitment. PwHD were categorized as having early-stage (ES), mid-stage (MS), or advanced-stage (AS) HD as reported by the treating physician. Data were collected by the physician, and a voluntary questionnaire was completed by the PwHD or a caregiver. All findings were analyzed descriptively. Differences were assessed using analysis of variance or χ² tests.</p><p><strong>Results: </strong>A total of 2,094 PwHD were included; 1,602 (77%) were from Europe and 492 (23%) were from the United States, with 846 (40%) with ES, 701 (33%) with MS, and 547 (26%) with AS HD. PwHD reported current (13%, n = 272) or previous (28%, n = 575) suicidal ideation, which was more common with advanced HD (ES, 11%; MS, 14%; AS, 15%; <i>p</i> < 0.05). Of 482 questionnaire respondents, 91% (n = 437) reported difficulty sleeping, which was more common with AS HD (<i>p</i> < 0.05; [<i>p</i> = 0.000]).</p><p><strong>Discussion: </strong>The HDBOI study showed a substantial burden of suicidal ideation and sleep disturbances among PwHD, which tended to worsen with disease severity.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 3","pages":"e200461"},"PeriodicalIF":2.3,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11962051/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143772928","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Quality Improvement Initiative to Implement Anxiety Screening for Children and Teens With Headache and Epilepsy.","authors":"Christina Murphy, Sara E Molisani, Amanda C Riisen, Carinna M Scotti-Degnan, Dina Karvounides, Stephanie Witzman, Michael C Kaufman, Alexander K Gonzalez, Mark Ramos, Christina L Szperka, Nicholas S Abend","doi":"10.1212/CPJ.0000000000200458","DOIUrl":"10.1212/CPJ.0000000000200458","url":null,"abstract":"<p><strong>Background and objectives: </strong>We conducted a quality improvement initiative to implement standardized screening for anxiety among adolescents with headache and/or epilepsy receiving outpatient neurology care at a quaternary health care system, consistent with recommendations from the American Academy of Neurology. Our SMART (Specific, Measurable, Achievable, Relevant, and Time-Based) aim was to screen ≥90% of established patients aged 12 years or older seen by a participating health care professional using a standardized anxiety screener by February 2024.</p><p><strong>Methods: </strong>This initiative was conducted in patients seen for follow-up by 17 participating neurology health care professionals. Health care professional opinions were assessed before and after implementation of the Generalized Anxiety Disorder-7 (GAD-7), administered as a previsit questionnaire distributed using the electronic health record. The integrated workflow included a best practice advisory (BPA) alert that permitted easy access to interventions and automatic population of education materials into the after-visit summary. After 12 months of use (March 2023 to February 2024), we assessed demographic and diagnostic information, GAD-7 completion rates, anxiety symptom severity, BPA utilization, and health care professional acceptance of the intervention.</p><p><strong>Results: </strong>The GAD-7 was completed for 64% of 3,671 encounters and by 71% of 2031 unique patients. The GAD-7 was more often completed for encounters if the patient was female, younger, or White or had a headache diagnosis. Among unique patients, anxiety symptoms were minimal in 50%, mild in 24%, moderate in 17%, and severe in 10%. Severe anxiety symptoms were more often present in female patients or those with a headache diagnosis. Among patients with severe anxiety symptoms, 66% had established behavioral health care plans and, for remaining patients, referrals were made to community behavioral health care professionals (11%), or pediatric psychologists (4%) or social workers (3%) within neurology. Clinicians indicated that the approach was easy to use and improved the quality of patient care.</p><p><strong>Discussion: </strong>We implemented standardized EHR-based screening for anxiety symptoms for pediatric neurology patients, most of whom had headache or epilepsy. Screening was feasible, and approximately one-quarter of patients had moderate or severe anxiety symptoms. Future work will focus on improving completion rates of previsit questionnaires including the GAD-7 and optimizing clinician actions based on the screening data.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 3","pages":"e200458"},"PeriodicalIF":2.3,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11962050/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143780716","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Benzodiazepine Initiation and the Risk of Falls or Fall-Related Injuries in Older Adults Following Acute Ischemic Stroke.","authors":"Shuo Sun, Victor Lomachinsky, Louisa H Smith, Joseph P Newhouse, M Brandon Westover, Deborah Lynne Blacker, Lee H Schwamm, Sebastien Haneuse, Lidia M V R Moura","doi":"10.1212/CPJ.0000000000200452","DOIUrl":"10.1212/CPJ.0000000000200452","url":null,"abstract":"<p><strong>Background and objectives: </strong>Benzodiazepine (BZD) use in older adults after acute ischemic stroke (AIS) is common. We aimed to assess the risk of falls or fall-related injuries (FRIs) in older adults after the use of BZDs during the acute poststroke recovery period.</p><p><strong>Methods: </strong>We emulated a hypothetical randomized trial of BZD use during the acute poststroke recovery period using linked data from the Get With the Guidelines Stroke Registry and Mass General Brigham's electronic health records. Our cohort included patients aged 65 years and older with an AIS admission between 2014 and 2021, no documented previous stroke, and no BZD prescriptions in the 3 months before admission. The potential for immortal time and confounding bias was addressed separately using inverse probability weighting.</p><p><strong>Results: </strong>We analyzed data from 495 patients who initiated inpatient BZDs within 3 days of admission and 2,564 who did not. After standardization, the estimate was 694 events per 1,000 (95% CI 676-709) for the BZD initiation strategy and 584 events per 1,000 (95% CI 575-595) for the noninitiation strategy. Subgroup analyses showed risk differences of 142 events per 1,000 (95% CI 111-165) and 85 events per 1,000 (95% CI 64-107) for patients aged 65-74 years and 75 years and older, respectively. Risk differences were 187 events per 1,000 (95% CI 159-206) for patients with minor (NIH Stroke Severity Scale score <math><mrow><mo>≤</mo></mrow> </math> 4) AIS and 32 events per 1,000 (95% CI 10-58) for those with moderate-to-severe AIS.</p><p><strong>Discussion: </strong>Initiating BZDs within 3 days of an AIS is associated with an elevated ten-day risk of falls or FRIs, particularly for patients aged 65-74 years and for those with mild stroke. This underscores the need for caution when initiating BZDs, especially among individuals likely to be ambulatory during the acute and subacute poststroke period.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 3","pages":"e200452"},"PeriodicalIF":2.3,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11936338/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143720854","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cenobamate Monotherapy for Focal Epilepsy: A Single-Center Retrospective Study.","authors":"Erkam Zengin, Ferhat Erol, Andrea Gil Guevara, Fred Alexander Lado","doi":"10.1212/CPJ.0000000000200460","DOIUrl":"10.1212/CPJ.0000000000200460","url":null,"abstract":"<p><strong>Background and objectives: </strong>Cenobamate is a novel antiseizure medication (ASM) approved by the Food and Drug Administration for use as adjunctive therapy in focal epilepsy. However, there are limited data on its use as a standalone monotherapy. The aim of our study was to investigate the use of cenobamate monotherapy and evaluate its clinical efficacy and safety in treating focal epilepsy.</p><p><strong>Methods: </strong>This single-center retrospective study was conducted on patients who were transitioned to cenobamate monotherapy for more than 6 months at a daily dosage of at least 150 mg. The cohort comprised patients transitioned from monotherapy and those previously on polytherapy with ASMs. Efficacy was based on the seizure freedom and seizure frequency reduction rates between pretreatment and post-treatment with cenobamate while safety was estimated by the reported adverse events.</p><p><strong>Results: </strong>A total of 527 patients were found to use cenobamate as part of their treatment regimen; 45 patients (9%) were transitioned to cenobamate monotherapy and met our predefined criteria. The median follow-up was 14.6 months. Before treatment with cenobamate, 56% were taking one ASM, 33% two ASMs, and 9% three ASMs. The median dose for cenobamate was 250 mg. The mean seizure frequency on cenobamate was reduced from 4.3 to 0.7 per month; the responder rate (50% reduction in seizure frequency) was achieved at 77%, and 55% of the patients remained seizure-free during the 12-month observation period.</p><p><strong>Discussion: </strong>Cenobamate monotherapy was found to significantly reduce seizure frequency and achieve high seizure freedom rates and was well tolerated in patients with focal epilepsy, highlighting its promise as an emerging alternative for patients with refractory focal epilepsy.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 3","pages":"e200460"},"PeriodicalIF":2.3,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11966522/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143795825","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessment of Seizure Action Plans in Pediatric Convulsive Status Epilepticus: Focus on Benzodiazepine-Responsive and Resistant Cases.","authors":"Coral M Stredny, Sahar Rostamian, Theodore Alexander Sheehan, Marina Gaínza-Lein, Shannon Carey, Samuel Lewis, Justice Clark, Bethany Bucciarelli, Madeline Chiujdea, Annalee Antonetty, Bo Zhang, Luisa Fernanda Atunes Ortega, Lillian Voke, Tobias Loddenkemper","doi":"10.1212/CPJ.0000000000200449","DOIUrl":"10.1212/CPJ.0000000000200449","url":null,"abstract":"<p><strong>Background and objectives: </strong>Optimal acute treatment is crucial in pediatric convulsive status epilepticus (CSE). However, data on the benefits of seizure action plans (SAPs) and treatment algorithm implementation in relation to process and outcome measures in CSE are scarce. Our study examines treatment algorithm adherence in benzodiazepine (BZD)-responsive and BZD-resistant groups and specifically focuses on the relationship with personalized SAPs.</p><p><strong>Methods: </strong>We performed a prospective observational cohort study evaluating patient care processes and outcomes in young patients with CSE lasting ≥5 minutes, requiring admission and an antiseizure medication(s) (ASM) for seizure termination from February 2016 to July 2018. Patients with infantile spasms, invasive EEG monitoring, unclear seizure duration, unclear ASM administration time, or seizure cluster were excluded. We used univariate statistics to analyze the data.</p><p><strong>Results: </strong>We enrolled 60 patients (median age 3.7 [1.9-7.0] years, 48% female), including 34 BZD-responsive and 26 BZD-resistant patients. Patients who had access to a personalized SAP, even if it was not adhered to, experienced a median (p25-p75) time to first ASM of 6 minutes (5-18; n = 34). By contrast, patients without access to a personalized SAP had a longer median (p25-p75) time to the first ASM of 15 minutes (8-27; n = 24; <i>p</i> < 0.05). The median (p25-p75) time to administer the first ASM was 6 (5-15; n = 33) minutes in BZD-responsive patients vs 15 (6-32; n = 25; <i>p</i> < 0.05) minutes in BZD-resistant patients. Treatment protocol implementation rates were lower in BZD-resistant vs BZD-responsive patients. The median (p25-p75) time to administer the first ASM was 5 minutes (4-5; n = 14) in patients who implemented a personalized SAP compared with 16 minutes (6-31; n = 20; <i>p</i> < 0.001) in patients without SAP implementation. The median (p25-p75) seizure duration in personalized SAP implementation and nonimplementation groups was 9 (6-16; n = 14) and 22 (11-64; n = 20; <i>p</i> < 0.01) minutes, respectively. The intubation rate was 14% for those who implemented the personalized SAP and 53% for those who did not (<i>p</i> < 0.05).</p><p><strong>Discussion: </strong>Seizure duration was shorter in patients with personalized SAP, and the time to administer ASM was faster. In addition, BZD-resistant patients were less likely to follow treatment protocols, and the time to first-line therapy was slower. SAP and algorithm implementation was associated with a lower intubation rate, indicating potential benefits, including improved process and patient outcome measures.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 3","pages":"e200449"},"PeriodicalIF":2.3,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11966521/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143795820","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Role of Central Sensitization Syndrome in Patients With Autonomic Symptoms.","authors":"Peter Novak, Sadie P Marciano, Aleandra Witte","doi":"10.1212/CPJ.0000000000200463","DOIUrl":"10.1212/CPJ.0000000000200463","url":null,"abstract":"<p><strong>Background and objectives: </strong>Idiosyncratic autonomic-like symptomatology, e.g., when objective autonomic tests cannot fully explain autonomic concerns, is poorly understood. We hypothesize that central sensitization plays a role in the autonomic symptoms-sings dichotomy.</p><p><strong>Methods: </strong>This retrospective case-control study was conducted at Brigham and Women's Faulkner Hospital Autonomic Laboratory between 2022 and 2023 and analyzed patients who completed autonomic testing that included surveys (Central Sensitization Inventory [assessing central sensitization syndrome {CSS}], Compass-31 [assessing autonomic symptoms], Neuropathy Total Symptom Score-6 [assessing sensory symptoms]) and autonomic (Valsalva maneuver, deep breathing, sudomotor evaluation, and head-up tilt), cerebrovascular (cerebral blood flow velocity [CBFv]), respiratory (capnography), and neuropathic (skin biopsies for assessment of small fiber neuropathy) testing.</p><p><strong>Results: </strong>In total, 555 patients were enrolled and 455 (78%) satisfied criteria for CSS. Patients with CSS were younger and more frequently female and had longer duration of symptoms, more comorbidities, and higher Compass-31 scores and NTSS-6 compared with non-CSS patients. Autonomic testing showed lower orthostatic end-tidal CO₂ (<i>p</i> = 0.002) and larger orthostatic decline in CBFv (<i>p</i> < 0.001) in the CSS group. There was no difference in the peripheral nervous system markers (sudomotor tests and skin biopsies). The frequency of moderate autonomic failure (AF) (91.4% vs 95%, <i>p</i> = 0.321) was similar between the groups, but the CSS group had lower AF score (4.21 ± 3.34 vs 5.23 ± 4.08, <i>p</i> < 0.021).</p><p><strong>Discussion: </strong>CSS is present in most patients with chronic autonomic concerns. Central sensitization amplifies autonomic symptoms presumably through perturbed interoceptive processing and can be an underlying mechanism driving idiosyncratic autonomic-like symptomatology. Patients with CSS had objective evidence of autonomic impairment; however, it was less severe than in non-CSS patients. Our study shows that CSS and AF coexist and both conditions need to be treated.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 3","pages":"e200463"},"PeriodicalIF":2.3,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11970932/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143795847","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Seizures in Cerebral Amyloid Angiopathy: A Systematic Review and Meta-Analysis.","authors":"Brin E Freund, Md Manjurul Islam Shourav, Anteneh M Feyissa, James F Meschia, Amen Yonas, Kevin M Barrett, William O Tatum, Michelle P Lin","doi":"10.1212/CPJ.0000000000200454","DOIUrl":"10.1212/CPJ.0000000000200454","url":null,"abstract":"<p><strong>Purpose of review: </strong>Cerebral amyloid angiopathy (CAA) is a disease of the cerebral vasculature that can result in microhemorrhages, as well as intraparenchymal and subarachnoid hemorrhage, superficial siderosis (SS), and/or secondary infarct/inflammation. CAA may be encountered as an isolated pathology or with Alzheimer disease and has been demonstrated to be associated with an increased risk of seizures. However, the overall rates of seizures and specific pathologies related to CAA and their subsequent risk of seizures have not been elucidated.</p><p><strong>Recent findings: </strong>Prior studies of CAA and seizures are predominantly case reports or small case series, and larger studies have focused primarily on smaller subgroups of patients with CAA. Only 2 prior studies assessed larger heterogeneous populations of patients with CAA. One study focused on long-term outcomes and evaluated the impact of seizures on cognitive and survival outcomes retrospectively, although it did not delineate the effects of acute and chronic seizure disorders (epilepsy) and did not find an association. Long-term prospective or retrospective studies on outcomes regarding seizures/epilepsy and CAA are therefore lacking.</p><p><strong>Summary: </strong>A total of 1,376 articles were identified, with 48 (34 case reports/series and 14 cohort studies) included in this review. Acute symptomatic seizures (ASyS) and epilepsy were poorly defined, and the overall prevalence of seizures in cohort studies was 22.8%, with significant heterogeneity (<i>I</i> ² = 77%; <i>p</i> < 0.01). Epilepsy was diagnosed in 34.4% and ASyS in 10.6% of patients in heterogeneous cohorts. Most of the studies assessed seizures in specific subgroups of CAA with variable prevalence, including CAA with related inflammation (CAA-ri): 56.9%; lobar intracranial hemorrhage (ICH): 17.1%; and cortical SAH (cSAH) or SS: 8.7%. In heterogeneous cohorts, SS (<i>p</i> < 0.001 and <i>p</i> = 0.03, respectively) and CAA-ri (<i>p</i> = 0.005 and <i>p</i> = 0.04, respectively) were significantly associated with epilepsy/seizures. In 1 study, cSAH (<i>p</i> = 0.03) and acute lobar ICH (<i>p</i> = 0.002) were associated with seizures, likely related to inclusion of ASyS. Status epilepticus (14/125) and drug resistance (6/89) were infrequent. Clinical pathologic entities associated with a risk of seizures include cSAH, CAA-ri, SS, and acute ICH.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 3","pages":"e200454"},"PeriodicalIF":2.3,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11952699/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753700","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}