Neurology. Clinical practice最新文献

{"title":"Relationship Between Thrombolysis-to-Puncture Time and Outcomes of Endovascular Thrombectomy in Acute Ischemic Stroke.","authors":"Xu Tong, Baixue Jia, Gaoting Ma, Xuelei Zhang, Jens Fiehler, Fabian Flottmann, Matthias Bechstein, Gabriel Broocks, Uta Hanning, Helge C Kniep, Götz Thomalla, Milani Deb-Chatterji, Gerhard Schön, Yijun Zhang, Feng Gao, Ning Ma, Dapeng Mo, Zhongrong Miao, Lukas Meyer","doi":"10.1212/CPJ.0000000000200434","DOIUrl":"https://doi.org/10.1212/CPJ.0000000000200434","url":null,"abstract":"<p><strong>Background and objectives: </strong>Intravenous thrombolysis (IVT) followed by endovascular thrombectomy (EVT) improves functional outcomes in patients with acute ischemic stroke (AIS) caused by large vessel occlusion (LVO). There are limited data on the effect of thrombolysis-to-puncture time (TTP) on outcomes in patients with AIS undergoing IVT plus EVT.</p><p><strong>Methods: </strong>We selected 1,104 patients receiving IVT + EVT for anterior circulation LVO stroke from 2 prospective nationwide registries (259 cases from ANGEL-ACT in China: November 2017 to March 2019, 845 cases from German Stroke Registry-Endovascular Treatment in Germany: June 2015 to December 2019). Based on the TTP, eligible patients were divided into 4 groups (≤30 min, 31-50 min, 51-70 min, and >70 min). The radiologic and clinical outcomes (e.g., successful recanalization [modified Thrombolysis in Cerebral Infarction score of 2b-3] at final angiogram, modified Rankin Scale [mRS] score of 0-2 at 90 days, any intracranial hemorrhage [ICH], and symptomatic ICH within 24 hours) among the 4 groups were compared by χ² tests for trend and using multivariable logistic regression models.</p><p><strong>Results: </strong>In the 4 groups from ≤30 min to >70 min, 226, 282, 230, and 366 patients were included, respectively. An increased TTP was associated with a lower chance of successful recanalization (<i>p</i> = 0.016) and mRS score 0-2 (<i>p</i> = 0.002). Compared with the group of ≤30 min, the group of >70 min was less likely to achieve successful recanalization (adjusted odds ratio [OR] = 0.47, 95% CI 0.25-0.89) and the groups of 50-70 min and >70 min had a reduced probability of mRS score 0-2 (adjusted OR = 0.50, 95% CI 0.33-0.78; adjusted OR = 0.56, 95% CI 0.37-0.85). No significant differences were found for any ICH or symptomatic ICH among the 4 groups after adjustment with potential confounders.</p><p><strong>Discussion: </strong>Delay from thrombolysis to puncture should be minimized when considering bridging IVT before EVT for patients with AIS due to anterior circulation LVO. Further studies are warranted to verify and expand on these findings.</p><p><strong>Trial registration information: </strong>ClinicalTrials.gov, NCT03370939 and NCT03356392.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 4","pages":"e200434"},"PeriodicalIF":2.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12253961/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144626774","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":"Sleep Medicine Resource Utilization in Individuals With Parkinson Disease: A Population Study of Health Administrative Data.","authors":"Ryan Gotfrit, Robert Talarico, Priti Gros, Marta Kaminska, Tiago A Mestre, Tetyana Kendzerska","doi":"10.1212/CPJ.0000000000200511","DOIUrl":"https://doi.org/10.1212/CPJ.0000000000200511","url":null,"abstract":"<p><strong>Background and objectives: </strong>Individuals with Parkinson disease (PD) may face barriers in obstructive sleep apnea diagnosis/management due to sleep being lower priority and sleep disturbances being poorly recognized. Evidence on sleep-medicine service utilization in the PD population is lacking. We conducted a population-based study to identify discrepancies in sleep-medicine resource use (prevalence rates of polysomnograms [PSG] performed and positive airway pressure [PAP] initiated) over a 10-year period between the PD population and the matched non-PD population.</p><p><strong>Methods: </strong>We conducted a retrospective longitudinal population-based study using health administrative databases in Ontario from 2012 to 2021 in adults with PD to compare overall and annual prevalence rates of PSGs performed and PAP initiated to 1:1 randomly selected propensity score matched controls from the matched non-PD population based on simultaneous exact matching on age, sex, and calendar year and caliper matched propensity scores from a logistic regression model (based on sociodemographic variables and comorbidities) using validated health administrative definitions to identify PD cases and controls. We hypothesized that the PD population has lower rates of PSGs performed and PAP treatments initiated compared with the similar matched non-PD population. We used Poisson regression to estimate annual prevalence rate ratios to determine the relative change in prevalence over the study period between the groups.</p><p><strong>Results: </strong>Sixty-five thousand, one hundred sixty-seven patients with PD and 11,460,672 controls met our inclusion criteria. We successfully propensity score matched 64,879 PD cases to controls. From 2012 to 2021, there were a higher prevalence of any PSG performed in the PD population (8.2% vs 6.3%, [RR: 1.30, 95% CI: 1.25-1.35], <i>p</i> < 0.001) and no difference in the rates of any PAP initiated in the PD population vs controls (4.0% vs 4.1%, [RR: 0.93-1.03, 95% CI: 0.93-1.03], <i>p</i> = 0.46). For both groups, annual prevalence rates generally increased over time. There was no difference in the annual prevalence rate ratio of any PSG performed or any PAP initiated in the PD population vs controls (1.07 [95% CI: 1.06-1.07] vs 1.07 [95% CI: 1.07-1.08], <i>p</i> = 0.5; 1.10 [95% CI: 1.09-1.11] vs 1.11 [95% CI: 1.10-1.11], <i>p</i> = 0.18, respectively).</p><p><strong>Discussion: </strong>Sleep-medicine resource utilization in the PD population is at least similar to the matched non-PD population and follows the increase with time observed in the general population.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 4","pages":"e200511"},"PeriodicalIF":2.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12253960/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144626775","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":"Erratum: Psychosis in Neurocognitive Disorder Among Ethnoculturally Diverse Older Persons.","authors":"Estevana Isaac, Carolyn Wei Zhu, Monica Rivera Mindt, Albert L Siu, Alex Federman, Kristine Yaffe, Barbara G Vickrey, Jenifer Voeks, Parul Agarwal, Derrick Brooks, Omobolanle Ayo, Mary Sano","doi":"10.1212/CPJ.0000000000200509","DOIUrl":"10.1212/CPJ.0000000000200509","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1212/CPJ.0000000000200467.].</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 4","pages":"e200509"},"PeriodicalIF":2.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12245482/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144608917","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":"Invasive Neurostimulation During Pregnancy for Treatment of Epilepsy and Tourette Syndrome: Maternal and Fetal Outcomes.","authors":"Anhmai Vu, Aisha Abdulrazaq, Brian Lundstrom, Lauren M Jackson, Jeffrey W Britton, William O Tatum, Cornelia Drees, Elizabeth A Coon, Linda M Szymanski, Gregory A Worrell, Kelsey M Smith","doi":"10.1212/CPJ.0000000000200498","DOIUrl":"10.1212/CPJ.0000000000200498","url":null,"abstract":"<p><strong>Background and objectives: </strong>Invasive neurostimulation is rapidly becoming an established option for treatment of neurologic disorders, particularly those that are refractory to pharmacologic treatment. However, there is limited information on the use of neuromodulation during pregnancy. This study explores the safety and clinical outcomes of invasive neuromodulation-specifically vagus nerve stimulation (VNS), deep brain stimulation (DBS), and responsive neurostimulation (RNS)-in pregnant patients with epilepsy and movement disorders.</p><p><strong>Methods: </strong>Pregnant patients treated with VNS, DBS, or RNS were identified, and charts were reviewed to extract data on maternal epilepsy/movement disorder, treatment, and pregnancy.</p><p><strong>Results: </strong>A total of 14 patients (9 VNS, 3 DBS, 2 RNS) had 22 pregnancies. Neuromodulation indications included focal epilepsy (n = 6: 3 VNS, 2 RNS, 1 DBS), generalized epilepsy (n = 6: all VNS), and Tourette syndrome (n = 2: both DBS). The average age at implantation was 24.7 years for VNS, 29.6 years for DBS, and 28 years for RNS. Pregnancy complications included miscarriages (n = 4 pregnancies; 1 VNS, 2 DBS, 1 RNS), pre-eclampsia with fetal growth restriction (n = 3: 2 VNS, 1 DBS), and gestational diabetes (2 VNS). In addition, 10 pregnancies (8 VNS, 2 RNS) were complicated by seizure exacerbations. Delivery of eight of the pregnancies (5 VNS, 1 DBS, 2 RNS) was by cesarean section. There were no cases of maternal or neonatal mortality, and there were no major congenital malformations. Owing to exacerbated shortness of breath during the third trimester, 1 patient had her VNS turned off.</p><p><strong>Discussion: </strong>Pregnancy complications were consistent with previous reports of patients with neurologic disorders. Despite limitations in sample size and confounding factors related to medication use and neurologic diagnosis, our study suggests that implanted neuromodulation devices do not seem to pose a risk of neuromodulation-related teratogenicity. While these data are promising and may provide some reassurance for patient counseling regarding pregnancy, further studies with larger sample sizes are necessary.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 4","pages":"e200498"},"PeriodicalIF":3.2,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161771/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144302579","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":"Infection, Relapses, and Pseudo-Relapses in Individuals With Multiple Sclerosis.","authors":"Amber Salter, Samantha Lancia, Mudita Sharma, Gary R Cutter, Robert J Fox, Ruth Ann Marrie","doi":"10.1212/CPJ.0000000000200493","DOIUrl":"10.1212/CPJ.0000000000200493","url":null,"abstract":"<p><strong>Background and objectives: </strong>Infections are associated with an increased risk of relapse and pseudo-relapse in persons with multiple sclerosis (MS). However, the relationship with relapses and pseudo-relapses after SARS-CoV-2 infections (COVID) vs other infections in MS is poorly understood. Therefore, we compared the occurrence of relapse and pseudo-relapse after COVID and other infections with noninfected participants with MS.</p><p><strong>Methods: </strong>In spring 2023, we surveyed participants from the North American Research Committee on Multiple Sclerosis Registry regarding whether they had had a COVID infection, other infections, relapses, and pseudo-relapses. Recent infections, occurring in the 6 months before the survey, were used to categorize participants into groups: recent COVID, non-COVID infection (with no history of ever having COVID), COVID and non-COVID infections, or uninfected.</p><p><strong>Results: </strong>Of the 4,787 participants eligible for analysis, 2,927 participants were included, of whom 294 (10%) had a recent COVID infection; 853 (29.1%) had 1 recent infection other than COVID; 246 (8.4%) had a recent COVID and non-COVID infection; and 1,534 (52.4%) had no infection with COVID nor any infection within the past 6 months (uninfected). Compared with no infections, non-COVID infection was associated with a 39% increased likelihood of relapse (1.39, 95% CI [1.04-1.87]), whereas a recent COVID infection was associated with a decreased likelihood of relapse (0.45 [0.23, 0.87]), adjusting for covariates. All infection groups were associated with increased odds of pseudo-relapse compared with the uninfected group (non-COVID infections: 1.78 [1.44, 2.20]; COVID infection: 1.80 [1.32, 2.45]; COVID and non-COVID infection: 3.04 [2.24, 4.12]).</p><p><strong>Discussion: </strong>Because individuals with MS are at increased risk of infections, the association of infections with relapses and pseudo-relapses is clinically important. The high prevalence of acute worsening after infection, regardless of the type of infection, compared with those with no reported infection, needs to be considered in the management of persons with MS.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 4","pages":"e200493"},"PeriodicalIF":2.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12153504/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144285791","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":"\"Not Everything That Can Be Counted Counts, and Not Everything That Counts Can Be Counted\".","authors":"Adam C Webb","doi":"10.1212/CPJ.0000000000200514","DOIUrl":"10.1212/CPJ.0000000000200514","url":null,"abstract":"","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 4","pages":"e200514"},"PeriodicalIF":2.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12226005/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144575928","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":"Antiseizure Medication Prescription Patterns for Treatment of Post-Traumatic Epilepsy.","authors":"Matthew Pease, Arka N Mallela, Souvik Roy, Jorge Gonzalez-Martinez, David O Okonkwo, Flora M Hammond, Sergiu Abramovici, Jonathan Elmer, Wesley T Kerr, James Castellano","doi":"10.1212/CPJ.0000000000200466","DOIUrl":"https://doi.org/10.1212/CPJ.0000000000200466","url":null,"abstract":"<p><strong>Background and objective: </strong>Post-traumatic epilepsy (PTE) is common, occurring in upward of 1 in 3 patients with severe traumatic brain injury (TBI). There are limited data regarding initial prescription patterns and long-term course of antiseizure medications (ASMs).</p><p><strong>Methods: </strong>The goal of this study was to describe ASM prescription patterns and pharmacoresistance over time. We performed a secondary analysis of a prospective database of patients with severe TBI treated at a single center from 2002 through 2018. We included patients with PTE, defined as at least one seizure more than 7 days from injury, and at least six-month follow-up after PTE onset. ASMs were categorized as older (e.g., phenytoin) or newer (e.g., levetiracetam). We evaluated ASM prescription patterns and their association with epileptology referral and Glasgow Outcome Scale (GOS) score. We developed a logistic regression to predict pharmacoresistance.</p><p><strong>Results: </strong>Our cohort included 84 patients with PTE. ASM prescription for longer than 7 days after injury had only moderate correspondence with early post-traumatic seizures or PTE (Cohen Kappa 41%). At PTE onset, most (53%) were treated with newer ASM monotherapy, with 27% on older ASMs and 20% on multiple ASMs. Patients initially prescribed older ASM monotherapy were less likely to maintain their ASM monotherapy (e.g., medication switch/addition and self-wean) compared with those on newer ASMs (odds ratio [OR] 4.6; 95% confidence interval [CI] 1.3-16.7; <i>p</i> = 0.02). Only 23% of patients were referred to specialized epilepsy care despite 54% trialing 2 or more ASMs. Pharmacoresistance was associated with worse GOS outcomes (<i>p</i> = 0.04). Decompressive hemicraniectomy (OR 6.0; 95% CI 1.4-44; <i>p</i> = 0.03) and initial ASM polytherapy (OR 7.2; 95% CI 1.7-34; <i>p</i> < 0.01) predicted pharmacoresistance at 2 years.</p><p><strong>Discussion: </strong>We provide evidence suggesting that use of newer generation seizure medications is preferable when treating PTE. In addition, we identified early risk factors of pharmacoresistance, which was associated with poor long-term outcomes.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 4","pages":"e200466"},"PeriodicalIF":2.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12258814/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144643037","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":"Erratum: Herpes Zoster Infections With Multiple Sclerosis Disease-Modifying Therapies: A Real-World Pharmacovigilance Study.","authors":"Alexandra Balshi, Grace Leuenberger, John Dempsey, Nova Manning, Ursela Baber, Jacob A Sloane","doi":"10.1212/CPJ.0000000000200505","DOIUrl":"10.1212/CPJ.0000000000200505","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1212/CPJ.0000000000200462.].</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 4","pages":"e200505"},"PeriodicalIF":2.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12153501/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144285780","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":"Promoting Sleep for Neurology Inpatients: The Value of Routine Overnight Vital Signs.","authors":"Colin A Ellis, Patrick Z Liu, Alan Napole, Lydia Denison, Noor F Shaik, Grace Anya Venezia, Lovisa Ljungberg, Michael A Karamardian, Colleen Peachey, Michael Buckley, Charles J Bae, Laura Stein, Denise J Xu","doi":"10.1212/CPJ.0000000000200492","DOIUrl":"10.1212/CPJ.0000000000200492","url":null,"abstract":"<p><strong>Background and objectives: </strong>Sleep is a critical component of health and recovery. Awakening sleeping patients for routine vital sign monitoring overnight is a common inpatient practice, but its benefits are uncertain. This study asked 3 questions: (1) How often are routine overnight vital signs abnormal? (2) How often are those abnormal vital signs clinically actionable? (3) Are there patients who may not need routine overnight vital sign monitoring?</p><p><strong>Methods: </strong>We analyzed observational retrospective data extracted from electronic health records of patients admitted to inpatient neurology floors at our institution between 2017 and 2024. We defined routine vital signs as collected by nursing assistants at least 3 hours apart. The outcome measure was an urgent clinical action (stat orders, rapid responses, or transfers to a higher level of care) within 1 hour of vital sign events. We compared the rate of urgent clinical actions after abnormal vital signs with the base rate of those events after normal vital signs. Statistical analysis used generalized estimating equations to account for repeated measures.</p><p><strong>Results: </strong>We analyzed 102,184 routine vital sign events from 5,569 neurology admissions. In total, 9% of vital sign events were abnormal. The likelihood of urgent clinical actions increased after abnormal vital sign events, compared with after normal vital sign events during the day (4.3% vs 2.4%, odds ratio (OR) 1.8 [1.6-2.1]); at night (2.4% vs 0.9%, OR 2.8 [2.2-3.7]); during the night in low-risk patients with normal daytime vitals (2.0% vs 0.7%, OR 3.0 [1.8-4.9]); and at night in high-risk patients with abnormal daytime vitals (2.6% vs 1.1%, OR 2.5 [1.7-3.6]). The number needed to treat (NNT), i.e., the number of vital sign events needed to initiate 1 extra urgent clinical action above the base rate, was 1,856 [943; 4,598] in low-risk patients. The NNT for low-risk stroke patients was 1,570 [679; 4,707] and for general neurology patients was 2,231 [797; 5,423].</p><p><strong>Discussion: </strong>Urgent clinical actions attributable to abnormal routine vital signs were uncommon in neurology inpatients, especially overnight in patients with normal daytime vital signs. Determining the risks and benefits of routine overnight vital sign monitoring will help hospitals and health care providers move toward more patient-centered inpatient neurologic care.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 4","pages":"e200492"},"PeriodicalIF":3.2,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12253964/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144626772","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":"Diagnostic Journey and Health Care Burden of Patients With Creutzfeldt-Jakob Disease in the United States: A Real-World Evidence Study.","authors":"Emily Kutrieb, Montserrat Vera Llonch, Derek Weycker, Steven M Kymes, Duncan Brown, Anne V Smith, Robert S Pulido, Brian Appleby","doi":"10.1212/CPJ.0000000000200502","DOIUrl":"10.1212/CPJ.0000000000200502","url":null,"abstract":"<p><strong>Background and objectives: </strong>Evidence on the diagnostic journey and health care burden of patients with Creutzfeldt-Jakob disease (CJD) in the United States is limited. A real-world evidence study using a US health care claims database was undertaken to address this gap.</p><p><strong>Methods: </strong>A retrospective observational cohort study was conducted using data from the Merative MarketScan Research Databases (01/2012-12/2020). Study population comprised adults aged 18 years or older with evidence of CJD (initial diagnosis = index date), no evidence of selected neurologic conditions after the last CJD diagnosis, and health care coverage during the 12-month pre-index period; adults meeting selection criteria are referred herein as \"patients with CJD.\" Diagnostic journey was detailed based on evidence of symptoms and alternative neurologic conditions during the pre-index period as well as time to death (based on a proxy). Health care burden was summarized through levels of all-cause health care utilization and expenditures during the pre/post-index periods.</p><p><strong>Results: </strong>A total of 215 patients with CJD qualified for inclusion in the study population. The mean duration from first symptom to initial CJD diagnosis was 5.0 months, and 80% of patients had ≥3 symptoms, most commonly altered mental status (82%), gait/coordination disturbance (60%), and malaise/fatigue (44%). Most patients (63%) also had ≥1 alternative diagnosis, including cerebrovascular disease (49%), peripheral vertigo (11%), and Alzheimer disease (7%); the mean duration from first alternative diagnosis to initial CJD diagnosis was 2.4 months. The mean (median) time to death (proxy) from first symptom was 7.9 (6.6) months and from initial CJD diagnosis was 2.9 (1.1) months. During the 12-month pre-index period, mean (95% CI) cumulative health care expenditures were $35,493 ($28,914-$42,722); by the end of the post-index period, cumulative expenditures averaged $93,601 ($78,878-$109,776) per patient.</p><p><strong>Discussion: </strong>Study findings suggest that, in US clinical practice, patients with CJD present with one or more clinical symptoms affecting motor, cognitive, or other domains, and many alternative diagnoses are considered, which may prolong the diagnostic journey. Study findings also suggest that health care expenditures-especially proximate to the initial CJD diagnosis-are notably high. CJD should be considered in the differential diagnosis of adults with rapidly progressing dementia or motor disturbance.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 4","pages":"e200502"},"PeriodicalIF":3.2,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12204766/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144528932","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}