Neurology. Clinical practice最新文献

{"title":"Disproportionately Enlarged Subarachnoid-Space Hydrocephalus on MRI in Pathologically Confirmed Progressive Supranuclear Palsy.","authors":"Miki Kawazoe, Shunsuke Koga, Hiroaki Sekiya, Keith Anthony Josephs, Neill R Graff-Radford, Dennis W Dickson","doi":"10.1212/CPJ.0000000000200431","DOIUrl":"10.1212/CPJ.0000000000200431","url":null,"abstract":"<p><strong>Background and objective: </strong>Several studies have shown that idiopathic normal-pressure hydrocephalus (iNPH) can mimic other neurodegenerative disorders, particularly progressive supranuclear palsy (PSP). In this study, we investigated iNPH clinical and neuroimaging features in patients with autopsy-confirmed PSP or Lewy body disease (LBD) by assessing the normal pressure hydrocephalus (NPH) triad of symptoms and imaging features of disproportionately enlarged subarachnoid-space hydrocephalus (DESH) and Evans index (EI) on antemortem MRI scans.</p><p><strong>Methods: </strong>Among our study participants (N = 190), the mean (SD) age was 76.8 (9.2) years and 134 (70.5%) were male. The patients had been followed at Mayo Clinic and had autopsy diagnosis of either PSP or LBD. Patients were excluded if they had Alzheimer disease or a history of a disorder that could cause hydrocephalus, such as chronic meningitis or neoplasia. The study included 101 patients with PSP and 89 with LBD. The frequency of DESH and a high EI on brain MRI were analyzed in PSP and LBD with logistic regression analyses, adjusting for age, sex, and brain weight. The NPH triad of symptoms was assessed relative to imaging findings.</p><p><strong>Results: </strong>We found that DESH and high EI were similar between PSP and LBD. The mean age at death (PSP: 74.0 [8.2]; LBD: 80.0 [9.2]) and brain weight (PSP: 1,190 [123]; LBD: 1,300 [150]) were greater in LBD compared with PSP (<i>p</i> < 0.001 for each). The frequency of DESH was greater in LBD than PSP (13% vs 3%, <i>p</i> = 0.004), while a high EI was similar in PSP and LBD (36% vs 32%, <i>p</i> = 0.500). The adjusted odds ratios for DESH and high EI were similar between the 2 groups (DESH: adjusted ORs 0.3, 95% CI 0.06-1.25, <i>p</i> = 0.119; high EI: adjusted ORs 1.8, 95% CI 0.86-4.06, <i>p</i> = 0.120).</p><p><strong>Discussion: </strong>These findings suggest that DESH and high EI, often considered biomarkers for iNPH, may lack specificity and may be found in a subset of patients with PSP or LBD leading to unnecessary neurosurgery for iNPH.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 2","pages":"e200431"},"PeriodicalIF":2.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11850053/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143502871","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":"Revised Diagnostic Criteria for Huntington Disease: What's It Gonna Take?","authors":"Jane S Paulsen, Deven K Burks","doi":"10.1212/CPJ.0000000000200430","DOIUrl":"10.1212/CPJ.0000000000200430","url":null,"abstract":"","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 2","pages":"e200430"},"PeriodicalIF":2.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11781268/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143080416","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":"Understanding Patient Referral Practices and Perceptions of Mental Health Professionals Among Neurologists.","authors":"Margaret T Gopaul, Sirikanya Chiraroekmongkon, Hamada Hamid Altalib","doi":"10.1212/CPJ.0000000000200455","DOIUrl":"10.1212/CPJ.0000000000200455","url":null,"abstract":"<p><strong>Background and objectives: </strong>To examine neurologists' practices in managing mental health care. The study aims to identify gaps in coordination between neurology and mental health care and to assess neurologists' perspectives on integrating mental health services into neurology clinics.</p><p><strong>Methods: </strong>A mixed-methods approach was used, combining survey data and qualitative interviews of key informants. The survey assessed management strategies for psychiatric comorbidities through 3 hypothetical cases and questions regarding referral patterns, accessibility of mental health professionals, and perceived effectiveness of mental health care. Chi-square tests were conducted to compare responses by location (the United States vs international) and specialty (general neurology, subspecialist neurology, and neuropsychiatry).</p><p><strong>Results: </strong>Significant differences in follow-up plans (χ² = 11.025, <i>p</i> = 0.026) and referral frequency for functional neurologic disorders (χ² = 24.943, <i>p</i> < 0.001) were observed, with US physicians more likely to comanage patients with mental health professionals. US neurologists also report referring patients with psychiatric comorbidities more frequently (χ² = 8.424, <i>p</i> = 0.038). No significant difference in the accessibility of mental health professionals was reported by location (χ² = 1.364, <i>p</i> = 0.968) or specialty (χ² = 2.939, <i>p</i> = 0.568). Neurologists in the United States were more likely than neurologists from other countries to perceive mental health professionals to be highly effective (χ² = 8.590, <i>p</i> = 0.035).</p><p><strong>Discussion: </strong>Most neurologists favor integrating mental health services within neurology clinics, underscoring the need for better care coordination. Despite the global effort to integrate mental health in primary care, these findings suggest a need to consider alternative models in coordinating mental health care.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 2","pages":"e200455"},"PeriodicalIF":2.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11839230/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143468582","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":"National Trends in Stroke Presentation, Treatments, and Outcomes During the First 2 Years of the COVID-19 Pandemic.","authors":"Cyrus Ayubcha, Peter Smulowitz, James O'Malley, Lidia Moura, Lawrence Zaborski, J Michael McWilliams, Bruce E Landon","doi":"10.1212/CPJ.0000000000200436","DOIUrl":"10.1212/CPJ.0000000000200436","url":null,"abstract":"<p><strong>Background and objectives: </strong>Early presentation and acute treatment for patients presenting with ischemic stroke are associated with improved outcomes. The onset of the COVID-19 pandemic was associated with a large decrease in patients presenting with ischemic stroke, but it is unknown whether these changes persisted.</p><p><strong>Methods: </strong>This study analyzed emergency department (ED) stroke presentations (n = 158,060) to all nonfederal hospitals in the 50 states and Washington, D.C., from 2019 through 2021 using administrative claims data of traditional fee-for-service Medicare enrollees aged 66 years or older. Patients presenting with stroke were identified using the ICD-10 CM (I63.X). We examined the number of beneficiaries presenting with ischemic stroke to the ED, both overall and by demographic categories (race, age, sex, region, Medicaid eligibility, comorbidity status), admission rates conditional on presentation, use of neurovascular interventions, thirty-day mortality, intensive care unit and mechanical ventilation use, length of stay, and discharge destination.</p><p><strong>Results: </strong>With the onset of the pandemic in March 2020, there was a drop of 32.1% in ED stroke presentations compared with March 2019 levels, and by December 2021, the rate remained 17.7% lower than baseline levels in December 2019. Relative to the prepandemic period, there were decreases in the proportions of those dually eligible for Medicaid (-0.8%, <i>p</i> < 0.0001) or Black (-0.8%, <i>p</i> < 0.0001), as well as those with atrial fibrillation (-1.1%, <i>p</i> < 0.0001), hypertension (-0.7%, <i>p</i> < 0.0001), and chronic obstructive pulmonary disease (-1.8%, <i>p</i> < 0.0001). Admitted patients were more often discharged to home as opposed to postacute care settings (+3.5%, <i>p</i> < 0.0001). The percentage of patients receiving intravenous thrombolysis changed minimally while those receiving intracranial mechanical thrombectomy (+17.8%, <i>p</i> < 0.0001) and carotid interventions (+6.9%, <i>p</i> < 0.0001) increased from baseline throughout the pandemic. Adjusted thirty-day mortality or referral to hospice increased (+1.81%, <i>p</i> < 0.0001) with larger increases seen among Black beneficiaries and those dually eligible for Medicaid.</p><p><strong>Discussion: </strong>After an initial sharp decline, stroke presentations remained substantially lower than at baseline through the end of 2021, especially among racial minority and those dually eligible for Medicaid. The observed increased mortality rates for those presenting with stroke may have resulted from later time of presentation after the onset of symptoms or preferential presentation of more vs less severe strokes.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 2","pages":"e200436"},"PeriodicalIF":2.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737637/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143008877","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":"Consensus Recommendations for the Management of Neurosarcoidosis: A Delphi Survey of Experts Across the United States.","authors":"Giovanna Sophia Manzano, James Eaton, Michael Levy, Justin R Abbatemarco, Allen J Aksamit, Pria Anand, Denis T Balaban, Paula Barreras, Robert P Baughman, Shamik Bhattacharyya, Roberto Bomprezzi, Tracey A Cho, Bart Chwalisz, Stacey Lynn Clardy, David B Clifford, Eoin P Flanagan, Jeffrey M Gelfand, George Kyle Harrold, Spencer K Hutto, Siddharama Pawate, Noellie Rivera Torres, Lama Abdel-Wahed, Steven Richard Dunham, Rajesh Kumar Gupta, Brandon Moss, Carlos A Pardo, Rohini D Samudralwar, Nagagopal Venna, Aram Zabeti, Ilya Kister","doi":"10.1212/CPJ.0000000000200429","DOIUrl":"10.1212/CPJ.0000000000200429","url":null,"abstract":"<p><strong>Background and objectives: </strong>Neurosarcoidosis poses a diagnostic and management challenge due to its rarity, phenotypic variability, and lack of randomized controlled studies to guide treatment selection. Recommendations for management based on expert opinion are useful in clinical practice and provide a framework for designing prospective studies.</p><p><strong>Methods: </strong>In this Delphi survey study, specialists with experience in managing patients with neurosarcoidosis were invited to anonymously complete 2 surveys about key elements of evaluation, diagnosis, treatment, monitoring, and long-term management of neurosarcoidosis. Expert consensus recommendations were adopted if >80% threshold of agreement was reached.</p><p><strong>Results: </strong>Of the 41 invited expert clinicians across the United States, 32 (78%) participated in the study. All round 1 respondents self-identified as neuroimmunologists (except for 1 pulmonologist). Consensus was reached regarding the need to consider neurosarcoidosis phenotype and severity to guide the choice of initial immunosuppression in both the acute (relapse) and maintenance phases. Experts endorsed the use of TNF-α inhibitors as first-line agents in selected phenotypes with poor prognosis. Neuroimaging was recommended to complement clinical surveillance for treatment response.</p><p><strong>Discussion: </strong>There was agreement on several key issues, most importantly on the need to consider neurosarcoidosis phenotype and severity when deciding initial treatment. No consensus was achieved on the dosing and duration of specific immunosuppressants, nor regarding the management of the peripheral nervous system manifestation of neurosarcoidosis. These topics warrant further investigation.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 2","pages":"e200429"},"PeriodicalIF":2.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737638/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143008874","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":"Disparate Use of Diagnostic Modalities for Patients With Limited English Proficiency and Neurologic Disorders.","authors":"Altaf Saadi, Redwan Bin Abdul Baten, Margarita Alegría, David Himmelstein, Steffie Woolhandler","doi":"10.1212/CPJ.0000000000200417","DOIUrl":"10.1212/CPJ.0000000000200417","url":null,"abstract":"<p><strong>Background and objectives: </strong>Limited English proficiency (LEP) impairs health access-including outpatient specialty care-and quality care, i.e., inappropriate use of diagnostic tests. At least in some cases, studies have suggested that clinicians may substitute testing for time-consuming clinical evaluation involving medical interpreters. This study (1) examines disparities in receipt of diagnostic testing among patients with LEP and neurologic illness, in both the ambulatory and emergency department (ED) settings, including (2) whether better patient-provider communication is associated with reduced testing disparities and (3) how testing disparities vary according to insurance.</p><p><strong>Methods: </strong>We analyzed nationally representative data from the 2003-2018 Medical Expenditure Panel Survey and identified adults with neurologic illness using diagnostic codes. To assess the association between LEP status and diagnostic testing (CT/MRI, laboratory tests, and any diagnostic tests), we estimated logistic regression models that included year-fixed effects. We constructed separate models for ambulatory and ED settings, including models with a patient-provider communication measure to see how that influenced the LEP-diagnostic testing association. Finally, we conducted stratified analyses by sources of health insurance.</p><p><strong>Results: </strong>LEP status was associated with greater receipt of laboratory tests (OR = 1.46, <i>p</i> < 0.05) but less CT/MRI in the ambulatory setting (0.86, <i>p</i> < 0.05), patterns that persisted in analyses stratified by insurance status. Factoring in patient-provider communication attenuated but did not eliminate these disparities, with attenuation most notable in rates of CT/MRI. We found fewer testing disparities for patients with LEP in the ED than in ambulatory settings.</p><p><strong>Discussion: </strong>In this nationwide study of patients with neurologic illness, we observed both greater and less use of diagnostic tests for patients with LEP and neurologic illness. The greater use of laboratory tests may reflect the overuse of easily obtainable tests for patients with LEP. Conversely, the less use of CT/MRI may be due to time and transportation challenges in scheduling follow-up visits, alongside other barriers to patient follow-up. The population of patients with LEP is growing, making it critical to study not only disparities in their care but also nuances and determinants of these disparities beyond patient-provider communication and across clinical settings.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 2","pages":"e200417"},"PeriodicalIF":2.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11727602/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142984294","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":"A Real-World Prospective Multiple Sclerosis Pregnancy Registry in the United States: PREG-MS.","authors":"Maria K Houtchens, Maria Claudia Manieri, Tatenda Dawn Mahlanza, Jeta Pol-Patil, Eric C Klawiter, Andrew J Solomon, Ellen Lathi, Joshua Katz, Carolina Ionete, Idanis Berrios Morales, Christopher Severson, Jonathan Zurawski, James M Stankiewicz, Ann Cabot, Adele Dessa Sadovnick","doi":"10.1212/CPJ.0000000000200425","DOIUrl":"10.1212/CPJ.0000000000200425","url":null,"abstract":"<p><strong>Background and objectives: </strong>Multiple sclerosis (MS) affects more than 1 million people in the United States, including reproductive-age women. There has been a paucity of prospective, pregnancy registries based on MS disease rather than medication exposures. A prospective MS pregnancy registry (PREG-MS) was established in 2017 as a prospective, single-cohort, real-world MS pregnancy registry in New England States of the United States, with goals to evaluate (1) course of MS and disease-modifying therapies (DMT) use during conception attempts and in the peripartum period, (2) pregnancy outcomes in women with MS (WwMS), and (3) longer-term developmental outcomes in offspring of WwMS.</p><p><strong>Methods: </strong>Between 2017 and 2020, PREG-MS recruited from 11 preselected academic and community MS centers and followed WwMS and their children from conception attempts and any pregnancy trimester, up to 3 years of postpartum. Comprehensive neurologic, obstetric, and pediatric development information was collected through telephone interviews and medical records.</p><p><strong>Results: </strong>One hundred forty-six patients were enrolled between 2017 and 2020; there were 122 pregnancies from 135 participants, and 105 infants were born on study. 24.6% pregnancies were unplanned; 14.1% had an infertility diagnosis. Assisted reproductive technologies were used by 12.6%. 54% of pregnancies were designated as \"high-risk\", and ∼40% had peripartum obstetrical complications with 17% adverse pregnancy outcomes. Mean baseline Expanded Disability Status Scale was 1.09 ± 0.84. ∼85% were treated with DMTs up to the time of conception. 19.7% had 1 or more relapses within prepregnancy year, correlating with increased duration of conception attempts (<i>p</i> < 0.0001). 12% had intrapartum, and 24.5% had postpartum relapses. Any fertility treatments predicted intrapartum relapses independent of DMT status (OR 5.18, 95% CI 1.58-17.02, <i>p</i> = 0.007). 33.6% were exposed to DMTs in pregnancy. Intrapartum relapses (<i>p</i> = 0.008) and high-risk pregnancy (<i>p</i> = 0.036) were associated with postpartum exacerbations.</p><p><strong>Discussion: </strong>Our real-world, prospective, nondisabled MS pregnancy cohort had a sizable proportion of participants with clinical disease activity in the prepartum and intrapartum period, despite high-DMT utilization prepartum. A greater-than-expected number of participants were considered to have high-risk pregnancies and reported peripartum complications. The use of any fertility treatments was independently predictive of intrapartum relapses, supporting hormonal-immune interactions as disease modulators in MS. Larger prospective, longitudinal registries are needed to confirm our findings.</p><p><strong>Trial registration information: </strong>Clinical trial registration number: NCT03368157.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 2","pages":"e200425"},"PeriodicalIF":2.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11727603/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142984287","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":"Improving the Clinical Diagnostic Criteria for Genetically Confirmed Adult-Onset Huntington Disease: Considering Nonmotor Presentations.","authors":"Ciaran M Considine, Clare M Eddy, Samuel A Frank, Sandra K Kostyk, Mayke Oosterloo, Annie Killoran, Erin Furr Stimming, Matthias Dose, Travis Cruickshank, Thomas D Bird, Louise Vetter, Astri Arnesen, James Valvano, Herwig W Lange, Daniel O Claassen","doi":"10.1212/CPJ.0000000000200427","DOIUrl":"10.1212/CPJ.0000000000200427","url":null,"abstract":"<p><strong>Background: </strong>Huntington disease (HD) is a genetic neurodegenerative disorder. Given the focus on motor manifestations, nonmotor symptoms are frequently underappreciated in clinical evaluations, despite frequently contributing to primary functional impairment.</p><p><strong>Recent findings: </strong>A diagnosis of motor-onset as the definition of manifest symptoms misrepresents the complex nature of HD presentation. Despite recent attempt to integrate nonmotor diagnostic criteria, practical guidelines are necessary to inform clinical diagnosis. We propose an HD diagnostic framework and staging system that prioritizes genetic testing, integrates motor and nonmotor symptom considerations in the determination of clinical disease onset and severity, and acknowledges the secondary role of clinically indicated diagnostic assessments, incorporating the broad symptom profiles observed in clinical practice.</p><p><strong>Implications for practice: </strong>The proposed diagnostic criteria more accurately reflect the presentation of HD and provide greater opportunities for health care professionals to provide appropriate clinical care guidelines for adults with gene-expanded HD.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 2","pages":"e200427"},"PeriodicalIF":2.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11741291/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143008876","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: Patient- and Caregiver-Reported Impact of Symptoms in Alzheimer Disease, Mild Cognitive Impairment, and Dementia.","authors":"Jamison Seabury, Jennifer Weinstein, Anika Varma, Spencer James Rosero, Charlotte Engebrecht, Abigail Arky, Christine Zizzi, Nuran Dilek, Abigail Mathewson, Susan Salem-Spencer, Elizabeth J Santos, Chad Rydel Heatwole","doi":"10.1212/CPJ.0000000000200444","DOIUrl":"10.1212/CPJ.0000000000200444","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1212/CPJ.0000000000200418.].</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 2","pages":"e200444"},"PeriodicalIF":2.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11839228/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143458703","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":"ALS Motor Observational Telemedicine Objective Rasch-Built Assessment: A Quantitative Scale for the Era of Teleneurology.","authors":"Christina N Fournier, Mark Levine, Karon Simmons, Rocío Carolina García-Santibáñez, Anne Rowland, Colin C Quinn, Doreen T Ho, Richard S Bedlack, Jonathan D Glass","doi":"10.1212/CPJ.0000000000200432","DOIUrl":"10.1212/CPJ.0000000000200432","url":null,"abstract":"<p><strong>Background and objectives: </strong>Telemedicine has become a mainstay of ALS clinical care, but there is currently no standardized approach for assessing and tracking changes to the neurologic examination in this format. The goal of this study was to create a standardized telemedicine-based motor examination scale to objectively and reliably track ALS progression and use Rasch methodology to validate the scale and improve its psychometric properties.</p><p><strong>Methods: </strong>A draft telemedicine examination scale with 25 items assessing movement in the bulbar muscles, neck, trunk, and extremities was created by an ALS expert panel, incorporating input from patient advisors. This prospective, observational study was approved by the Emory IRB, and participants provided informed consent. Adults with a diagnosis of ALS who were able to undergo a video telemedicine evaluation by an Emory clinician were eligible for participation. Rasch analyses were performed to determine the final item responses and optimize the scoring structure. Test-retest reliability was assessed in a subset of participants through 2 separate examinations by 2 different examiners within a 7-day period. Construct validity was assessed by calculating correlations with simultaneously administered Rasch-built Overall ALS Disability Scale (ROADS) and revised ALS Functional Rating Scale (ALSFRS-R).</p><p><strong>Results: </strong>The ALS Motor Observational Telemedicine Objective Rasch-built assessment was administered to a total of 258 PALS representing the full spectrum of a typical ALS clinic population. After performing Rasch analyses, 3 items were removed and item response categories were consolidated for 8 items. The final 22-item ALS MOTOR scale conformed to Rasch model criteria. The inter-rater reliability was 95%. The ALS MOTOR had a 0.78 (95% CI 0.72-0.83) correlation with ALSFRS-R and 0.81 (95% CI 0.76-0.85) correlation with ROADS.</p><p><strong>Discussion: </strong>The ALS MOTOR is a novel, accessible tool for remotely and objectively tracking ALS progression for both clinical care and research studies. Use of Rasch methodology for scale validation allowed for optimization of scale psychometric properties, which is particularly important when using the sum score as an overall outcome measure. Longitudinal and external validation studies are ongoing.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":"15 2","pages":"e200432"},"PeriodicalIF":2.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11772018/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143059625","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}