Neurology最新文献

{"title":"Microdystrophin Gene Therapy for Duchenne Muscular Dystrophy: The Ethics and Financial Toxicity of Exuberant Hope.","authors":"A Gordon Smith","doi":"10.1212/wnl.0000000000213776","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213776","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"74 1","pages":"e213776"},"PeriodicalIF":9.9,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143982458","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":"Use of Advanced Vessel Wall Imaging to Detect Vertebral Artery Dissection.","authors":"Xinyan Hu, Qianmei Jiang, Qian Jia, Jing Jing","doi":"10.1212/WNL.0000000000213542","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213542","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213542"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143811844","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":"Teaching Video NeuroImage: String Hallucinations in Parkinson Disease.","authors":"Jacky Ganguly, Shubhrangshu Banerjee, Purba Basu, Soumava Mukherjee, Nilam Singh, Hrishikesh Kumar","doi":"10.1212/WNL.0000000000213611","DOIUrl":"10.1212/WNL.0000000000213611","url":null,"abstract":"<p><strong>Statement of the clinical problem addressed by the case: </strong>String hallucination, a complex multimodal hallucination in patients with advanced Parkinson disease (PD).</p><p><strong>Brief description of case presentation: </strong>Two older patients with advanced PD and dementia presented with a peculiar type of hallucination, in the form of seeing and feeling threads on the surfaces or coming out from the fingers. They continued manipulating these imaginary threads before putting them aside. Quetiapine was helpful in both cases.</p><p><strong>Summary of the key teaching point in the case: </strong>Apart from the commonly described passage (a brief hallucination of a person, animal, or object passing sideways, within the periphery of the visual field) and presence (sensation of someone or something being present nearby, in the absence of seeing it) hallucinations, a multimodal (visual and tactile) hallucination can be seen in patients with advanced PD, mostly with dementia. The patients described here did not have any fluctuating cognition or other well-formed visual hallucinations as seen in dementia with Lewy bodies (DLB). An atypical antipsychotic like quetiapine is helpful to control such complex forms of hallucinations.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213611"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143811790","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":"Comparison of Inpatient and Emergency Department Costs to Research Funding for Functional Neurologic Disorder: An Economic Analysis.","authors":"Christopher D Stephen, Vicki Fung, David L Perez, Alberto J Espay","doi":"10.1212/WNL.0000000000213632","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213632","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213632"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143991269","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":"Efficacy and Safety of Sodium Oxybate in Adults With Idiopathic Hypersomnia: A Randomized Controlled Trial.","authors":"Yves Dauvilliers,Sofiene Chenini,Ophélie Thobois,Anna Laura Rassu,Claire Denis,Lily Guiraud,Isabelle Jaussent,Lucie Barateau","doi":"10.1212/wnl.0000000000213690","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213690","url":null,"abstract":"BACKGROUND AND OBJECTIVESIdiopathic hypersomnia (IH) is a rare central disorder of hypersomnolence characterized by excessive daytime sleepiness, prolonged nighttime sleep, and sleep inertia. Low-sodium oxybate is the sole Food and Drug Administration-approved treatment for IH. Objective measures of nighttime sleep and daytime sleepiness are lacking with oxybates in IH. We aimed to evaluate efficacy and safety of sodium oxybate (SXB) in IH.METHODSThis phase 3, double-blind, parallel-group, placebo-controlled trial was conducted at the National Reference Center for Hypersomnia in Montpellier-France. Eligible participants aged 18-60 years with IH with an Epworth Sleepiness Scale (ESS) score ≥14 were randomly assigned to receive SXB or placebo (1:1). After a 2-week screening without any drugs and without exposure to oxybate, patients started a 6-week individual twice-nightly up-titration scheme from 4.5 g to a maximum of 9 g. Treatment was administered at stable dose for 2 weeks, followed by a 2-week taper period. The primary endpoint was the between-group difference in ESS scores at week 8, identified by a covariance analysis, including baseline ESS scores. The same methodology was applied for secondary endpoints including Idiopathic Hypersomnia Severity Scale (IHSS) score and sleep latency on the Maintenance of Wakefulness Test (MWT). Safety was examined as a secondary endpoint.RESULTSAmong the 48 patients screened, 45 were randomized (36 women, 29.0 ± 7.5 years, 22 assigned to SXB, 23 to placebo) and 40 (19 receiving SXB, 21 placebo) completed the study. In the intention-to-treat analysis, the mean ESS score was significantly reduced in the SXB group compared with placebo, after adjusting for the baseline score (least squared [LS] mean difference: -6.86, 95% CI [-9.73 to -4.00]), p < 0.0001). Significant differences between SXB and placebo groups at week 8 were observed for the IHSS score (LS mean difference: -11.61; 95% CI [-16.63 to -6.59], p < 0.0001) and MWT latency (14.75; 95% CI [9.98-19.52], p < 0.0001). Treatment-emergent adverse events (nausea, headache, and dizziness) were reported in 81.8% patients with SXB and 26.1% with placebo.DISCUSSIONSXB resulted in a clinically meaningful improvement in adults with IH, reducing excessive sleepiness on ESS, improving wakefulness on MWT, and decreasing IH severity on IHSS after 8 weeks. The safety profile was consistent with previous reports on SXB.TRIAL REGISTRATION INFORMATIONClinicalTrials.gov NCT03597555, EudraCT number 2017-004122-15.CLASSIFICATION OF EVIDENCEThis article provides Class I evidence that sodium oxybate at a dose of 4.5-9 g per night compared with placebo reduces excessive sleepiness, improves wakefulness, and decreases disease severity with expected side effects in patients with idiopathic hypersomnia.","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"17 1","pages":"e213690"},"PeriodicalIF":9.9,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143945469","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":"Incidence Trends and Risk of Recurrent Stroke of Cervical Artery Dissections in the United States Between 2005 and 2019.","authors":"Liqi Shu, Lukas Strelecky, Adam de Havenon, Thanh N Nguyen, Nils Henninger, Zafer Keser, Muhib Khan, James Ernest Siegler, Mary Penckofer, Setareh Salehi Omran, Thalia S Field, Lily Zhou, Han Xiao, Austin Jacobson, Eric D Goldstein, Christoph Stretz, Farhan Khan, Elizabeth Perelstein, Karen Furie, Shadi Yaghi","doi":"10.1212/WNL.0000000000213548","DOIUrl":"10.1212/WNL.0000000000213548","url":null,"abstract":"<p><strong>Background and objectives: </strong>Cervical artery dissection (CeAD) is a common cause of acute ischemic stroke (AIS), especially in patients younger than 55 years, but data regarding trends and subsequent AIS risk after CeAD remain scarce. We aimed to determine national trends in CeAD admissions and examine post-CeAD risk of ischemic stroke.</p><p><strong>Methods: </strong>We used the National Inpatient Sample (2005-2019), National Readmission Database (2015-2019), and State Inpatient Database for New York (2011-2017) and Florida (2011-2019). Adult patients with spontaneous CeAD were identified using <i>International Classification of Diseases, Ninth Revision, Clinical Modification</i> and <i>International Classification of Diseases, Tenth Revision, Clinical Modification</i> codes. Survey-weighted annual CeAD cases were combined with US census data to estimate annual incidence. National estimates were verified with state-level data, which allows for the removal of duplicate admissions for a single patient through a unique patient identifier. Joinpoint regression was used to quantify the average annual percent change (AAPC) of CeAD incidence. AIS readmission risk after CeAD without concurrent AIS was assessed with death as a competing risk using Fine and Gray competing risk methodology.</p><p><strong>Results: </strong>From 2005 to 2019, we identified 125,102 patients (46.09% female, mean age 51.4 years) with spontaneous CeAD. CeAD incidence increased from 10.7 cases per million population in 2005 to 45.6 cases per million population in 2019, revealing an AAPC of 10.21% (95% CI 9.67%-10.76%). This substantial increase in CeAD admissions was particularly high in the older, Black, and Hispanic populations. Statewide data corroborated this upward trend with an AAPC of 8.47% (95% CI 7.97%-9.48%). Among patients with CeAD without AIS, vertebral artery dissection was the sole major predictor of subsequent ischemic stroke risk within 90 days (adjusted subdistributed hazard ratio 1.77, 95% CI 1.18-2.64, <i>p</i> = 0.006). Interaction and subgroup analyses were performed and demonstrated similar results.</p><p><strong>Discussion: </strong>There was an almost 5-fold increase in CeAD hospitalizations and an upward incidence trend from 2005 to 2019, particularly in racial minorities, which may be attributed to increased imaging and awareness of CeAD. Our study also revealed a small but significant risk of AIS in patients with vertebral artery dissection without concurrent ischemic stroke. These findings underscore the importance of studying acute treatment and secondary prevention strategies in patients with CeAD.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213548"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11966527/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143772770","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":"AAN Position: Opioids.","authors":"Umer Najib, Eric M Cheng, Rashmi B Halker Singh, Neishay Ayub, Sarah E Nelson, Patrick J Bushard, Justin T Jordan, Jason J Sico, Madeline Turbes, Wayne E Anderson","doi":"10.1212/WNL.0000000000213544","DOIUrl":"10.1212/WNL.0000000000213544","url":null,"abstract":"<p><p>This position statement serves to establish the American Academy of Neurology (AAN)'s stance on neurology care team's needs for safe prescribing of opioids and research for nonopioid pain alternatives so that the AAN can continue to advocate effectively for its members. While considerable improvements have been made in prescribing practices to address patient pain, opioids remain the most misused prescription medication in the United States. Neurology care teams often care for patients with chronic, complex conditions that include pain disorders such as migraine, peripheral neuropathy, traumatic brain injury, multiple sclerosis, and low back pain and are the 14th most frequent group to prescribe opioids. The AAN is dedicated to upstream approaches to prevent opioid misuse through research aimed at finding alternative therapies for chronic pain, reducing opioid addiction and misuse, and using tools such as electronic prescribing of controlled substances and state prescription monitoring programs to improve the safety of opioid prescribing when opioid treatment is necessary.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213544"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143803840","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":"Rapidly Progressive Clostridial Gas Gangrene of the Brain With Vascular Rupture Due to Occult Bowel Malignancy-Associated Bacteremia.","authors":"Niroshan Jeyakumar,Matthew Georgiades,Andrew Martin,Samuel Kim","doi":"10.1212/wnl.0000000000213680","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213680","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"25 1","pages":"e213680"},"PeriodicalIF":9.9,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143945468","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":"One-Year Radiologic Progression in Sporadic and Hereditary Cerebral Amyloid Angiopathy.","authors":"Maaike C van der Plas, Emma A Koemans, Manon R Schipper, Sabine Voigt, Ingeborg Rasing, Reinier G J van der Zwet, Kanishk Kaushik, Rosemarie van Dort, Sanne Schriemer, Thijs W van Harten, Erik van Zwet, Ellis S van Etten, Matthias J P van Osch, Gisela M Terwindt, Marianne van Walderveen, Marieke J H Wermer","doi":"10.1212/WNL.0000000000213546","DOIUrl":"10.1212/WNL.0000000000213546","url":null,"abstract":"<p><strong>Background and objectives: </strong>Knowledge on the short-term progression of cerebral amyloid angiopathy (CAA) is important for clinical practice and the design of clinical treatment trials. We investigated the 1-year progression of CAA-related MRI markers in sporadic (sCAA) and Dutch-type hereditary (D-CAA).</p><p><strong>Methods: </strong>Participants were included from 2 prospective cohort studies. 3T-MRI was performed at baseline and after 1 year. We assessed macrobleeds, cerebral microbleeds (CMBs), cortical superficial siderosis (cSS), convexity subarachnoid hemorrhages (cSAHs), white matter hyperintensities (WMH), enlarged centrum semiovale perivascular spaces (CSO-EPVS), and visually stimulated blood oxygenation level-dependent (BOLD) fMRI parameters. Progression was defined as increase in number of macrobleeds or CMBs, new focus or extension of cSS, increase in CSO-EPVS category, or volume increase of >10% of WMH. Multivariable regression analyses were performed to determine factors associated with progression and the association between events related to parenchymal injury (cSAH, macrobleeds) and radiologic progression.</p><p><strong>Results: </strong>We included 98 participants (47% women): 55 with sCAA (mean age 70 years), 28 with symptomatic D-CAA (mean age 59 years), and 15 with presymptomatic D-CAA (mean age 45 years). Progression of >1 MRI markers was seen in all 83 (100%) participants with sCAA and symptomatic D-CAA and in 9 (60%) with presymptomatic D-CAA. The number of CMBs showed the largest progression in sCAA (98%; median increase 24) and symptomatic D-CAA (100%; median increase 58). WMH volume (>10% increase in 70%; mean increase 1.2 mL) was most progressive in presymptomatic D-CAA. A decrease in the upslope of the visually evoked BOLD response was observed for most patients. Symptomatic D-CAA status was associated with more overall progression (adjusted odds ratio [aOR] 9.7; 95% CI 1.7-54.2), CMB (adjusted relative risk [aRR] 2.47; 95% CI 1.5-4.1), and WMH volume progression (β 2.52; 95% CI 0.3-4.8). Baseline CMB count (aRR 1.002; 95% CI 1.001-1.002) was associated with CMB progression and cSS presence at baseline (aOR 8.16; 95% CI 2.6-25.4) with cSS progression. cSS progression was also associated with cSAH and macrobleeds (aOR 21,029; 95% CI 2.042-216.537).</p><p><strong>Discussion: </strong>CAA is a radiologically progressive disease even in the short-term. After 1 year, all symptomatic and most of the presymptomatic participants showed progression of at least 1 MRI-marker. CMBs and WMH volume (in symptomatic CAA) and WMH volume (in presymptomatic CAA) are the most promising markers to track short-term progression in future trials.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213546"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11995281/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143811681","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":"Association Between Collateral Status, Blood Pressure During Thrombectomy, and Clinical Outcomes in Patients With Basilar Artery Occlusion.","authors":"Cong Luo, Thanh N Nguyen, Rui Li, Chunrong Tao, Xiaozhong Jing, Pengfei Xu, Li Wang, Anmo Wang, Feiyang Gao, Ming Cai, Keyi Zhang, Min Chen, Xia Jiang, Nan Shen, Mohamad Abdalkader, Patrik Michel, Jeffrey L Saver, Raul G Nogueira, Xinfeng Liu, Wei Hu","doi":"10.1212/WNL.0000000000213504","DOIUrl":"10.1212/WNL.0000000000213504","url":null,"abstract":"<p><strong>Background and objectives: </strong>We investigated the relationship between intraprocedural blood pressure (BP) and clinical outcomes in patients with basilar artery occlusion (BAO) undergoing endovascular treatment (EVT), exploring whether it is modifiable by collateral status.</p><p><strong>Methods: </strong>Patient data from the Endovascular Treatment for Acute Basilar Artery Occlusion (ATTENTION) randomized trial were analyzed for those with BAO who received EVT. Intraprocedural BP data were extracted, with collateral status assessed using the Basilar Artery on CT Angiography (BATMAN) score (BATMAN score ≥7 favorable collateral status, <7 unfavorable). Associations between BP parameters and outcomes were assessed using multivariable logistic regression and restricted cubic splines. The effect modification was assessed using an interaction term between BP parameters and collateral status. The primary outcome was a favorable outcome defined by a modified Rankin Scale (mRS) score of 0-3 at 90 days.</p><p><strong>Results: </strong>There were 212 patients included (median age 68 years, 32.1% female). Restricted cubic spline analysis showed that the SDs of systolic BP (SBP) and mean arterial pressure (MAP) had J-shaped relationships with favorable outcome (<i>p</i> for nonlinearity = 0.004 and <0.001, respectively), with inflection points at 12 and 8 mm Hg, respectively. Multivariable logistic regression showed that MAP of 80-110 mm Hg (adjusted odds ratio [aOR] 3.00, 95% CI 1.46-6.35) and MAP SD <8 mm Hg (aOR 2.28, 95% CI 1.24-4.25) were associated with favorable outcome. Significant interactions with collateral status were observed for MAP SD <8 mm Hg, SBP SD <12 mm Hg, MAP drop >20%, and minimum MAP and SBP (all <i>p</i>_interaction < 0.05). After Holm-Bonferroni correction, only the interaction between collateral status and MAP <80 mm Hg remained significant (corrected <i>p</i>_interaction = 0.036). In patients with unfavorable collateral status, MAP <80 mm Hg was associated with decreased probability of favorable outcome (aOR 0.04, 95% CI 0.00-0.21) while this association was not observed in patients with favorable collaterals.</p><p><strong>Discussion: </strong>For patients with BAO undergoing EVT, intraprocedural MAP between 80 and 110 mm Hg was associated with favorable outcome while MAP <80 mm Hg was associated with a lower probability of favorable outcome, especially in patients with unfavorable collateral status.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213504"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143784436","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}