Annals of Clinical and Translational Neurology最新文献

{"title":"Priming and task-specific training for arm weakness post stroke: A randomized controlled trial","authors":"Erin C. King,&nbsp;Jacob M. Schauer,&nbsp;Shyam Prabhakaran,&nbsp;Alexandra Wax,&nbsp;Sebastian Urday,&nbsp;Sangeetha Madhavan,&nbsp;Daniel M. Corcos,&nbsp;Mary Ellen Stoykov","doi":"10.1002/acn3.52271","DOIUrl":"10.1002/acn3.52271","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>The objective of this work was to evaluate if task-specific training (TST) preceded by bilateral upper limb motor priming (BUMP) reduces upper limb impairment more than TST preceded by control priming ([CP], sham electrical stimulation) in people with chronic stroke.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In this single-blind, randomized controlled trial, 76 adults with moderate to severe upper limb hemiparesis ≥6 months post-stroke were stratified by baseline impairment and randomized to receive either BUMP or CP prior to receiving the same TST protocol. Participants completed 30 h of treatment in 15 days over 6 weeks. The primary outcome was change in Fugl-Meyer upper extremity (FMUE) from baseline to 8-week follow-up. We also report clinically meaningful response rates defined as a change in FMUE score of 6 points or greater.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In response to treatment, both groups improved to a significant extent at follow-up, exceeding the FMUE minimum clinically important difference. Those in BUMP and CP saw a mean change of 5.68 (SE 0.76, <i>p</i> &lt; 0.001) and 5.87 (SE 0.76, <i>p</i> &lt; 0.001) respectively. There was no significant difference between treatment arms (mean difference of −0.20 (95% CI = [−2.37, 1.97], SE = 1.08, <i>p</i> = 0.86)). A response of ≥6 points was observed in 46% in BUMP and 50% in CP.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>There was no beneficial effect of BUMP. The magnitude of change seen in both groups reflects the largest improvement achieved with just 22.5 h of TST in this population, matching or out-performing more invasive, time-intensive, and costly interventions proposed in recent years.</p>\u0000 </section>\u0000 </div>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":"12 1","pages":"192-202"},"PeriodicalIF":4.4,"publicationDate":"2024-12-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11752083/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142833196","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":"Retinal thinning differentiates treatment effects in relapsing multiple sclerosis below the clinical threshold","authors":"Gabriel Bsteh,&nbsp;Harald Hegen,&nbsp;Nik Krajnc,&nbsp;Fabian Föttinger,&nbsp;Patrick Altmann,&nbsp;Michael Auer,&nbsp;Klaus Berek,&nbsp;Barbara Kornek,&nbsp;Fritz Leutmezer,&nbsp;Stefan Macher,&nbsp;Tobias Monschein,&nbsp;Markus Ponleitner,&nbsp;Paulus Rommer,&nbsp;Christiane Schmied,&nbsp;Karin Zebenholzer,&nbsp;Gudrun Zulehner,&nbsp;Tobias Zrzavy,&nbsp;Florian Deisenhammer,&nbsp;Franziska Di Pauli,&nbsp;Berthold Pemp,&nbsp;Thomas Berger","doi":"10.1002/acn3.52279","DOIUrl":"10.1002/acn3.52279","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To investigate retinal layer thinning as a biomarker of disease-modifying treatment (DMT) effects in relapsing multiple sclerosis (RMS).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>From an ongoing prospective observational study, we included patients with RMS, who (i) had an optical coherence tomography (OCT) scan within 6 to 12 months after DMT start (rebaseline) and ≥1 follow-up OCT ≥12 months after rebaseline and (ii) adhered to DMT during follow-up. Differences between DMT in thinning of peripapillary-retinal-nerve-fiber-layer (pRNFL) and macular ganglion cell-plus-inner plexiform-layer (GCIPL) were analyzed using mixed-effects linear regression. Eyes suffering optic neuritis during follow-up were excluded.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We included 291 RMS patients (mean age 30.8 years [SD 7.9], 72.9% female, median disease duration 9 months [range 6–94], median rebaseline-to-last-follow-up-interval 32 months [12–82]).</p>\u0000 \u0000 <p>Mean annualized rates of retinal layer thinning (%/year) in reference to DMF (<i>n</i> = 84, GCIPL 0.28, pRNFL 0.53) were similar under TERI (<i>n</i> = 18, GCIPL 0.34, pRNFL 0.59), GLAT (<i>n</i> = 24, GCIPL 0.32, pRNFL 0.56), and IFNb (<i>n</i> = 13, GCIPL 0.33, pRNFL 0.60) were slightly lower under S1PM (<i>n</i> = 27, GCIPL 0.19, pRNFL 0.42) and CLA (<i>n</i> = 23, GCIPL 0.20, pRNFL 0.42), and were significantly lower under NTZ (<i>n</i> = 47, GCIPL 0.09, pRNFL 0.24; both <i>p</i> &lt; 0.001) and antiCD20 (<i>n</i> = 55, GCIPL 0.10, pRNFL 0.23; both <i>p</i> &lt; 0.001). In patients achieving NEDA-2, observed thinning rates were lower overall, but still significantly lower under NTZ and antiCD20.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Applying a rebaselining concept, retinal layer thinning differentiates DMT effects even in clinically stable patients and, thus, might be a useful biomarker to monitor DMT efficacy on subclinical neuroaxonal degeneration—at least on a group level.</p>\u0000 </section>\u0000 </div>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":"12 2","pages":"345-354"},"PeriodicalIF":4.4,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/acn3.52279","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142833265","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":"Skull defect – Frontotemporal dementia sagging brain syndrome","authors":"Wouter I. Schievink,&nbsp;Marcel M. Maya,&nbsp;Robin Babadjouni,&nbsp;Angelique Sao-Mai S. Tay,&nbsp;Rachelle B. Taché","doi":"10.1002/acn3.52277","DOIUrl":"10.1002/acn3.52277","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Frontotemporal dementia (FTD) sagging brain syndrome is a disabling condition. An underlying spinal Cerebrospinal fluid leak can be identified in only a minority of patients and the success rate of non-directed treatments is low. Some of these patients have a remote history of craniectomy/cranioplasty and we report a positive response to custom implant cranioplasty revision many years after their initial cranioplasty.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We reviewed medical records and imaging studies of 61 consecutive patients with FTD sagging brain syndrome. A SIH Disability Assessment Score (SIHDAS) questionnaire was completed to assess the severity of the symptoms before and after custom implant cranioplasty. Pre- and post-operative brain MRI was obtained to assess degree of brain sagging.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Eight (13.1%) of the 61 patients had a history of craniectomy/cranioplasty 1.5–13.5 years prior to onset of symptoms of FTD sagging brain syndrome. The mean age of the one woman and seven men at the time of presentation to our medical center was 50 years (range, 26–68 years). None had sinking scalp flap syndrome. Prior treatments included epidural blood patching and dural reduction surgery. Custom cranial implant surgery was performed in four patients and resulted in prompt and remarkable improvement of symptoms in three patients (SIHDAS: very severe disability to no or mild disability) and mild improvement in one patient. Brain MRI showed improvement of brain sagging.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>A disproportionate number of patients with FTD sagging brain syndrome have a remote history of supratentorial craniectomy/cranioplasty and revision cranioplasty should be considered.</p>\u0000 </section>\u0000 </div>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":"12 1","pages":"226-234"},"PeriodicalIF":4.4,"publicationDate":"2024-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11752090/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142826840","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":"Argatroban plus alteplase in posterior versus anterior circulation stroke","authors":"Yu Cui,&nbsp;Hui-Sheng Chen","doi":"10.1002/acn3.52280","DOIUrl":"10.1002/acn3.52280","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>ARAIS trial failed to demonstrate benefit of argatroban as an adjunct to alteplase for stroke. Given differences between anterior circulation stroke (ACS) and posterior circulation stroke (PCS), we performed prespecified secondary analysis to investigate whether benefit of argatroban was different between ACS and PCS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In secondary analysis of ARAIS, patients with definite stroke territories based on responsible vessel examination were classified into ACS and PCS. The primary outcome was a 90-day excellent functional outcome (modified Rankin Scale score of 0 to 1). The efficacy was compared between argatroban plus alteplase and alteplase alone.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>This study included 356 patients from the full analysis set of ARAIS trial: 283 in the ACS group and 73 in the PCS group. Compared with alteplase alone, a higher likelihood of 90-day excellent functional outcome was associated with argatroban plus alteplase in PCS group (78.1% versus 61.0%; adjusted RD, 14.4%; 95% CI, 1.6% to 27.2%; <i>p</i> = 0.03), but similar in ACS group (61.7% versus 62.7%; adjusted RD, −2.4%; 95% CI, −10.1% to 5.2%; <i>p</i> = 0.54). After controlling unbalanced sample size bias by propensity score matching, significant interaction between efficacy and stroke territories was found (<i>p</i> = 0.01). The risk of symptomatic intracranial hemorrhage was higher following argatroban plus alteplase than alteplase alone in ACS group (<i>p</i> = 0.02).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Argatroban plus alteplase, compared with alteplase alone, was associated with improved functional outcomes in PCS. This study first demonstrated better benefits of argatroban plus alteplase in PCS, which deserves to be confirmed.</p>\u0000 </section>\u0000 </div>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":"12 2","pages":"355-365"},"PeriodicalIF":4.4,"publicationDate":"2024-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/acn3.52280","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142826820","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":"Prospective observational study of FKRP-related limb-girdle muscular dystrophy R9: A GRASP consortium study","authors":"Lindsay N. Alfano,&nbsp;Meredith K. James,&nbsp;Kristine Grosfjeld Petersen,&nbsp;Karen Rudolf,&nbsp;John Vissing,&nbsp;Renee Augsburger,&nbsp;Tahseen Mozaffar,&nbsp;Aileen Jones,&nbsp;Amanda Butler,&nbsp;Katie M. Laubscher,&nbsp;Shelley R. H. Mockler,&nbsp;Katherine D. Mathews,&nbsp;Megan A. Iammarino,&nbsp;Natalie F. Reash,&nbsp;Lindsay Pietruszewski,&nbsp;Linda P. Lowes,&nbsp;Talia Strahler,&nbsp;Matthew Wicklund,&nbsp;Stephanie Hunn,&nbsp;Conrad C. Weihl,&nbsp;Sandhya Sasidharan,&nbsp;Melissa Currence,&nbsp;Jeffrey M. Statland,&nbsp;Nikia Stinson,&nbsp;Megan Holzer,&nbsp;Doris G. Leung,&nbsp;Donovan J. Lott,&nbsp;Peter B. Kang,&nbsp;Scott Holsten,&nbsp;Urvi Desai,&nbsp;Nicholas E. Johnson,&nbsp;the GRASP-LGMD Consortium","doi":"10.1002/acn3.52276","DOIUrl":"10.1002/acn3.52276","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Limb-girdle muscular dystrophy R9 (LGMDR9, formerly known as LGMD2I), caused by variants in the fukutin-related protein (FKRP) gene leads to progressive muscle weakness of the shoulder and pelvic limb-girdles and loss of motor function over time. Clinical management and future trial design are improved by determining which standardized clinical outcome assessments (COA) of function are most appropriate to capture disease presentation and progression, informing endpoint selection and enrollment criteria. The purpose of our study was to evaluate the cross-sectional validity and reliability of clinical outcome assessments in patients with FKRP-related LGMDR9 participating in the Genetic Resolution and Assessments Solving Phenotypes in LGMD (GRASP) natural history study.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Enrolled patients completed a battery of COA on two consecutive days, including the North Star Assessment for limb girdle-type dystrophies (NSAD), the 100-m timed test (100 m), and the Performance of Upper Limb 2.0 (PUL).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 101 patients with FKRP-related LGMDR9 completed COA evaluations. All functional COA were highly and significantly correlated even across constructs, except for the 9-hole peg test. Similarly, all tests demonstrated excellent test–retest reliability across 2-day visits. The NSAD and PUL demonstrate robust psychometrics with good targeting, ordered response thresholds, fit and stability, and limited dependency of items across the scales.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This study has determined the suitability of several functional COA, cross-sectionally, in LGMDR9 to inform future trial design and clinical care.</p>\u0000 </section>\u0000 </div>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":"12 2","pages":"332-344"},"PeriodicalIF":4.4,"publicationDate":"2024-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/acn3.52276","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142826823","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":"Spatiotemporal spinal cord stimulation with real-time triggering exoskeleton restores walking capability: a case report","authors":"Penghao Liu,&nbsp;Yuanchen Cheng,&nbsp;Zhuofan Xu,&nbsp;Xiaoyu Li,&nbsp;Zan Chen,&nbsp;Wanru Duan","doi":"10.1002/acn3.52281","DOIUrl":"10.1002/acn3.52281","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Motor recovery is challenging for spinal cord injury (SCI), especially in low-level SCI.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A 16-year-old patient with complete SCI at T12 presented flaccid paralysis and inability to control defecation and was scored as ASIA A at admission. The patient underwent spinal cord stimulation (SCS) implantation at the T11-L1, followed by an innovative algorithm combining spatiotemporal SCS with real-time triggered exoskeleton training (EXS-SCS).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>After 1 month of treatment, she gained substantial improvement in her iliopsoas and quadriceps femoris muscle strength to grade 3–4 as well as percutaneous EMG, allowing for assisted standing and walking, and was reassessed as ASIA C.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>This case reveals the potential of SCS-EXS regimen in restoring walking capability of SCI patients.</p>\u0000 </section>\u0000 </div>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":"12 3","pages":"659-665"},"PeriodicalIF":4.4,"publicationDate":"2024-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/acn3.52281","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142826849","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":"Neuromuscular transmission deficits in patients with CMT and ClC-1 inhibition in CMT animal models","authors":"Thomas Skjærlund Grønnebæk,&nbsp;Helga Haahr-Lillevang,&nbsp;Martin Skov,&nbsp;Kristina Kelly,&nbsp;Nathan R. Kerr,&nbsp;Jose A. Viteri,&nbsp;Andrea Jaworek,&nbsp;Amy Bartlett,&nbsp;Jane Bold,&nbsp;John Hutchison,&nbsp;Jorge Quiroz,&nbsp;Hatice Tankisi,&nbsp;Thomas Holm Pedersen,&nbsp;Henning Andersen,&nbsp;William David Arnold","doi":"10.1002/acn3.52252","DOIUrl":"10.1002/acn3.52252","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Charcot–Marie Tooth (CMT) is a hereditary neuropathy characterized by muscle weakness and fatigue with no approved therapies. Preclinical studies implicate neuromuscular junction (NMJ) transmission deficits in muscle dysfunction in CMT. This study aimed to evaluate NMJ function in patients with CMT types 1 and 2, and to determine whether enhancing NMJ transmission can improve muscle function in preclinical CMT models.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>First, an observational study involving single fiber electromyography (SFEMG) and clinical testing in patients with CMT 1 and 2 and healthy controls (HC) was conducted. Next, preclinical studies examined muscle function, specifically nerve-stimulated muscle force after partially inhibiting ClC-1 chloride channels with the novel small molecule NMD670.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Twenty-one CMT patients (46.4 ± 14.4 years) and 10 HC (43.3 ± 12.7 years) were enrolled. SFEMG jitter (NMJ variability) was higher [median (range)] in the CMT patients [56 μs (35; 197 μs)] vs. HC [29 μs (19; 36 μs)], (<i>p</i> &lt; 0.05). Blocking (NMJ failure) was higher in the CMT patients (13.4% (0.0; 90.9%)) vs. HC (0.0% (0.0; 4.5%)), (<i>p</i> &lt; 0.05). In CMT, jitter and blocking correlated inversely with muscle strength, mobility, balance, and endurance. In CMT 1A and 2D mice, NMD670 increased both peak force and contractile endurance in vivo.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Our study suggests that NMJ dysfunction contributes to muscle dysfunction in patients with CMT 1 and 2. Furthermore, our preclinical data provide proof-of-mechanism for recovery of muscle function with ClC-1 inhibition in CMT mouse models. Collectively, these findings suggest that targeting NMJ dysfunction with ClC-1 inhibitors could enhance muscle function in CMT patients, warranting further clinical trials.</p>\u0000 </section>\u0000 </div>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":"12 2","pages":"320-331"},"PeriodicalIF":4.4,"publicationDate":"2024-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/acn3.52252","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142816743","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":"In vivo evidence for cell body loss in cortical lesions in people with multiple sclerosis","authors":"Eva A. Krijnen,&nbsp;Hansol Lee,&nbsp;Samantha Noteboom,&nbsp;Florence L. Chiang,&nbsp;Martijn D. Steenwijk,&nbsp;Menno M. Schoonheim,&nbsp;Eric C. Klawiter,&nbsp;Susie Y. Huang","doi":"10.1002/acn3.52237","DOIUrl":"10.1002/acn3.52237","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To quantify alterations in soma and neurite density imaging measures within and surrounding cortical lesions in people with multiple sclerosis using in vivo high-gradient diffusion MRI.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In this cross-sectional study, 41 people with multiple sclerosis and 34 age- and sex-matched healthy controls underwent 3 T high-gradient diffusion MRI. Cortical lesions were segmented on artificial intelligence-enabled double inversion recovery images. “Inner” and “outer” perilesional layers were segmented as two expanding shells of 2 mm surrounding a cortical lesion. Intracellular, intra-neurite, and extracellular signal fractions and apparent soma radius were estimated in (peri)lesional and normal-appearing cortex.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Cortical lesions were present in all people with multiple sclerosis with a median count of 8 [IQR 5–18] and total volume of 0.16 [0.09–0.46 mL]. People with multiple sclerosis (mean 0.27 ± 0.03) showed lower normalized cortical volumes compared to healthy controls (0.30 ± 0.02). Compared to healthy controls (mean 0.58 ± 0.028), normal-appearing cortex in multiple sclerosis (0.57 ± 0.034) showed lower intra-cellular signal fraction. Cortical lesions (0.49 ± 0.089) exhibited lower intra-cellular signal fractions compared to perilesional (“inner”: 0.55 ± 0.049, “outer”: 0.55 ± 0.039) and normal-appearing cortex, demonstrating a gradation of change. The soma radius varied significantly across cortices, becoming smaller when moving outward from cortical lesions (cortical lesions: 10.38 ± 0.209 μm, “inner” layer: 10.19 ± 0.140 μm, “outer” layer: 10.07 ± 0.149 μm, normal-appearing cortex: 9.99 ± 0.127 μm).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Cortical cell body loss in multiple sclerosis is most pronounced in cortical lesions and also present in normal-appearing cortex. Gradients of diffusion microstructural alterations moving outward from cortical lesions toward normal-appearing cortex highlight the potential of high-gradient diffusion MRI to identify both focal and diffuse cortical pathology.</p>\u0000 </section>\u0000 </div>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":"12 1","pages":"4-16"},"PeriodicalIF":4.4,"publicationDate":"2024-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11752088/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142823449","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":"Exercise as an abortive treatment for cluster headaches: Insights from a large patient registry","authors":"Mi-Kyoung Kang,&nbsp;Yooha Hong,&nbsp;Soo-Jin Cho","doi":"10.1002/acn3.52263","DOIUrl":"10.1002/acn3.52263","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>This study aimed to evaluate the potential of exercise as an abortive treatment for patients with cluster headache (CH).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A cross-sectional survey was conducted among CH patients at a single center in Korea. Demographics and headache characteristics were compared between those who found exercise effective and those who did not. We analyzed the type and intensity of exercise used. Headache improvement was measured as intensity reduction, with ≥50% improvement defined as a reduction in intensity by half or more. Exercise intensity was categorized as moderate (“possible to talk but hard to sing”) or high (“difficult to speak without pausing”). Case reports of patients exercising during CH attacks were also reviewed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 167 registered CH patients, 136 patients provided responses about exercise during CH attacks; 39.7% (54/136) attempted exercise as treatment. Improvement was reported by 42.6% (23/54), with ≥50% improvement in 29.6% (16/54). Patients who found exercise effective had lower Headache Impact Test-6 scores. Effective exercises included running (39.1%), squats (30.4%), and stair climbing (21.7%), with high-intensity exercise effective for 52.2% and moderate intensity for 43.5%. Among the 23 patients who benefited from exercise, 18 reported their most effective treatment, which were exercise alone in 50% (9/18), followed by exercise with triptans in 38.9% (7/18), and oxygen with or without triptans in 5.5% (1/18).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>About 40% of CH patients attempted exercise for acute CH management, with 30% experiencing over 50% improvement. While evidence is limited, exercise may help alleviate headaches and could be considered an adjunctive treatment for CH attacks.</p>\u0000 </section>\u0000 </div>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":"12 1","pages":"149-157"},"PeriodicalIF":4.4,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11752095/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142805560","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":"Efficacy and safety of combined low-dose rituximab regimen for chronic inflammatory demyelinating polyradiculoneuropathy","authors":"Ying Du,&nbsp;Qi Yan,&nbsp;Chuan Li,&nbsp;Wenping Zhu,&nbsp;Chao Zhao,&nbsp;Yunfeng Hao,&nbsp;Lin Li,&nbsp;Dan Yao,&nbsp;Xuan Zhou,&nbsp;Ying Li,&nbsp;Yuting Dang,&nbsp;Rong Zhang,&nbsp;Lin Han,&nbsp;Yuanyuan Wang,&nbsp;Tao Hou,&nbsp;Juan Li,&nbsp;Hailin Li,&nbsp;Panpan Jiang,&nbsp;Pei Wang,&nbsp;Fenying Chen,&nbsp;Tingge Zhu,&nbsp;Juntong Liu,&nbsp;Shuyu Liu,&nbsp;Lan Gao,&nbsp;Yingjun Zhao,&nbsp;Wei Zhang","doi":"10.1002/acn3.52270","DOIUrl":"10.1002/acn3.52270","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To determine the efficacy and safety of combined low-dose rituximab with conventional therapy for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) treatment.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Total 73 patients with CIDP were enrolled for the retrospective cohort study, and divided into conventional first-line therapy cohort (<i>n</i> = 40) and combined low-dose rituximab (100 mg per infusion) cohort (<i>n</i> = 33). The outcome measures include scores of I-RODS, mRS, INCAT, ONLS, TSS, and COMPASS 31 scale at baseline and regular four visits (4, 16, 28, and 52 weeks), as well as proportion of favorable response and outcome, corticosteroids dosage, and deterioration occurrence during follow-up.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Compared to conventional therapy cohort, combined rituximab cohort presented better improvements and higher proportion of favorable response in scales assessments at each visit, as well as significantly reduced corticosteroids dosage and deterioration occurrence during the follow-up. Analyses of subgroups showed better improvements in both typical CIDP and CIDP variants in combined rituximab cohort than those in conventional therapy cohort, but had no differences between each other. Early initiating combined rituximab regimen (&lt;10 weeks) showed better improvements than delayed initiation (≥10 weeks) at the first three visits within 28 weeks, while had no difference in favorable prognoses at the last visit of 52 weeks after once reinfusion. No rituximab correlated serious adverse events were reported in our patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Our simplified regimen of combined low-dose rituximab has been firstly demonstrated for the better efficacy and safety than conventional therapy in CIDP treatment.</p>\u0000 </section>\u0000 </div>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":"12 1","pages":"180-191"},"PeriodicalIF":4.4,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11752089/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142805558","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}