Pilar H García-Casanova, Pablo Pérez-Martínez, Teresa Sevilla, Rosalía Doménech, Montserrat León, Juan F Vázquez-Costa
Background and purpose: The purpose was to describe the risk of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, hospitalization for coronavirus disease 2019 (COVID-19) and related death and to assess the impact of the pandemic in the survival of amyotrophic lateral sclerosis (ALS) patients.
Methods: The risk of SARS-CoV-2 infection, hospitalization for COVID-19 and related death was assessed in ALS patients alive between March 2020 and July 2022. To evaluate its impact in the overall survival of ALS patients, the survival of patients who died before and during the pandemic was compared.
Results: Amongst 263 ALS patients alive during the pandemic, 62 got infected during the study period (infection rate 14.34 per 100 person-years). Most infections (68%) occurred during the sixth wave (November 2021 to January 2022) and most patients (67%) were vaccinated at the time of infection. The hospitalization rate due to COVID-19 was 4.16 per 100 person-years. The multivariable model confirmed non-invasive ventilation (NIV) use prior to infection as a risk factor for hospitalization (odds ratio [OR] = 7.96, p = 0.003) and COVID-19 vaccination as a protective factor (OR = 0.093, p = 0.025) independent of age, sex and gastrostomy. Within 30 days after infection, 7% of non-ventilated patients started NIV and five patients (8.06%) died, of whom four were previously ventilated. The median survival of ALS patients was similar before and during the pandemic and no effect was found in the Cox regression model (hazard ratio 1.02, p = 0.89).
Conclusions: This study shows a high risk of severe COVID-19 amongst ALS patients requiring NIV. Nevertheless, the pandemic showed no impact in the overall survival of ALS patients, probably due to a high vaccination rate and an adequate access to healthcare resources.
{"title":"Impact of SARS-CoV-2 infection and COVID-19 pandemic on the morbidity and mortality of amyotrophic lateral sclerosis patients in Valencia, Spain.","authors":"Pilar H García-Casanova, Pablo Pérez-Martínez, Teresa Sevilla, Rosalía Doménech, Montserrat León, Juan F Vázquez-Costa","doi":"10.1111/ene.16465","DOIUrl":"https://doi.org/10.1111/ene.16465","url":null,"abstract":"<p><strong>Background and purpose: </strong>The purpose was to describe the risk of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, hospitalization for coronavirus disease 2019 (COVID-19) and related death and to assess the impact of the pandemic in the survival of amyotrophic lateral sclerosis (ALS) patients.</p><p><strong>Methods: </strong>The risk of SARS-CoV-2 infection, hospitalization for COVID-19 and related death was assessed in ALS patients alive between March 2020 and July 2022. To evaluate its impact in the overall survival of ALS patients, the survival of patients who died before and during the pandemic was compared.</p><p><strong>Results: </strong>Amongst 263 ALS patients alive during the pandemic, 62 got infected during the study period (infection rate 14.34 per 100 person-years). Most infections (68%) occurred during the sixth wave (November 2021 to January 2022) and most patients (67%) were vaccinated at the time of infection. The hospitalization rate due to COVID-19 was 4.16 per 100 person-years. The multivariable model confirmed non-invasive ventilation (NIV) use prior to infection as a risk factor for hospitalization (odds ratio [OR] = 7.96, p = 0.003) and COVID-19 vaccination as a protective factor (OR = 0.093, p = 0.025) independent of age, sex and gastrostomy. Within 30 days after infection, 7% of non-ventilated patients started NIV and five patients (8.06%) died, of whom four were previously ventilated. The median survival of ALS patients was similar before and during the pandemic and no effect was found in the Cox regression model (hazard ratio 1.02, p = 0.89).</p><p><strong>Conclusions: </strong>This study shows a high risk of severe COVID-19 amongst ALS patients requiring NIV. Nevertheless, the pandemic showed no impact in the overall survival of ALS patients, probably due to a high vaccination rate and an adequate access to healthcare resources.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142139676","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Klaus V Toyka, Christian Krarup, Andreas Steck, Gérard Said, Zohar Argov, Jan van Gijn, José Ferro, Giancarlo Comi, Claudio L A Bassetti
Background and purpose: The European Academy of Neurology (EAN) was a merger from two parent societies: the European Neurological Association (ENS, founded in 1986) and the European Federation of Neurological Societies (EFNS, founded in 1987).
Methods: This article was written by nine former presidents, three of whom were also founders of the ENS, and is based on recollections and documents. It follows up on a review of the ENS history stored in the EAN archive.
Results: The first European society (ENS) was founded by eight individual European academic clinician-neuroscientists aiming at joining with other qualified European neuroscientists on an individual membership basis. After 1990 members were also invited from behind the former Iron Curtain. A principal goal was holding neurology meetings (700 participants in 1988 and over 3000 in 2010), promoting collaborative research projects with exchange of junior neuroscientists, and providing teaching and education independent from nationality. Health politics were not part of the agenda. The executive boards (4-year term) were staffed with academic scientists from all subspecialties of neurology. Numerous bursaries and fellowships were established for junior neurologists. The impact of ENS members on research activities of young investigators was appreciated by academia at large. After years of negotiations ENS and EFNS joint efforts resulted in forming the EAN covering all fields of neurology and neuroscience under one roof.
Conclusion: The basic principles of the ENS were successfully integrated into the new EAN in particular documented by the number of individual members rising to over 4000 in 2024.
{"title":"The history of the European Neurological Society (1986-2014)-10 years later.","authors":"Klaus V Toyka, Christian Krarup, Andreas Steck, Gérard Said, Zohar Argov, Jan van Gijn, José Ferro, Giancarlo Comi, Claudio L A Bassetti","doi":"10.1111/ene.16440","DOIUrl":"https://doi.org/10.1111/ene.16440","url":null,"abstract":"<p><strong>Background and purpose: </strong>The European Academy of Neurology (EAN) was a merger from two parent societies: the European Neurological Association (ENS, founded in 1986) and the European Federation of Neurological Societies (EFNS, founded in 1987).</p><p><strong>Methods: </strong>This article was written by nine former presidents, three of whom were also founders of the ENS, and is based on recollections and documents. It follows up on a review of the ENS history stored in the EAN archive.</p><p><strong>Results: </strong>The first European society (ENS) was founded by eight individual European academic clinician-neuroscientists aiming at joining with other qualified European neuroscientists on an individual membership basis. After 1990 members were also invited from behind the former Iron Curtain. A principal goal was holding neurology meetings (700 participants in 1988 and over 3000 in 2010), promoting collaborative research projects with exchange of junior neuroscientists, and providing teaching and education independent from nationality. Health politics were not part of the agenda. The executive boards (4-year term) were staffed with academic scientists from all subspecialties of neurology. Numerous bursaries and fellowships were established for junior neurologists. The impact of ENS members on research activities of young investigators was appreciated by academia at large. After years of negotiations ENS and EFNS joint efforts resulted in forming the EAN covering all fields of neurology and neuroscience under one roof.</p><p><strong>Conclusion: </strong>The basic principles of the ENS were successfully integrated into the new EAN in particular documented by the number of individual members rising to over 4000 in 2024.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142139679","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Koos P J van Dam, Luuk Wieske, Eileen W Stalman, Laura Y L Kummer, Anneke J van der Kooi, Joost Raaphorst, Diederik van de Beek, Jan J G M Verschuuren, Annabel M Ruiter, Esther Brusse, Pieter A van Doorn, Adája E Baars, W Ludo van der Pol, H Stephan Goedee, Anja Ten Brinke, S Marieke van Ham, Theo Rispens, Taco W Kuijpers, Filip Eftimov
Background and purpose: There are concerns for safety regarding SARS-CoV-2 vaccines for patients with autoimmune neuromuscular disease. We compared daily functioning using disease-specific patient-reported outcome measures (PROMs) before and after SARS-CoV-2 vaccinations.
Methods: In this substudy of a prospective observational cohort study (Target-to-B!), patients with myasthenia gravis (MG), chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), and idiopathic inflammatory myopathy (IIM) vaccinated against SARS-CoV-2 were included. Surveys of daily functioning (Myasthenia Gravis Activities of Daily Living, Inflammatory Rasch-Built Overall Disability Scale, Multifocal Motor Neuropathy Rasch-Built Overall Disability Scale, and Health Assessment Questionnaire-Disability Index) were sent before first vaccination and every 60 days thereafter for up to 12 months. Regression models were constructed to assess differences in PROM scores related to vaccination, compared to scores unrelated to vaccination. We also assessed the proportion of patients with deterioration of at least the minimal clinically important difference (MCID) between before first vaccination and 60 days thereafter.
Results: We included 325 patients (median age = 59 years, interquartile range = 47-67, 156 [48%] female sex), of whom 137 (42%) had MG, 79 (24%) had CIDP, 43 (13%) had MMN, and 66 (20%) had IIM. PROM scores related to vaccination did not differ from scores unrelated to vaccination. In paired PROMs, MCID for deterioration was observed in three of 49 (6%) MG patients, of whom none reported a treatment change. In CIDP, MCID for deterioration was observed in eight of 29 patients (28%), of whom two of eight (25%) reported a treatment change.
Conclusions: SARS-CoV-2 vaccination had no effect on daily functioning in patients with autoimmune neuromuscular diseases, confirming its safety in these patients.
{"title":"Patient-reported daily functioning after SARS-CoV-2 vaccinations in autoimmune neuromuscular diseases.","authors":"Koos P J van Dam, Luuk Wieske, Eileen W Stalman, Laura Y L Kummer, Anneke J van der Kooi, Joost Raaphorst, Diederik van de Beek, Jan J G M Verschuuren, Annabel M Ruiter, Esther Brusse, Pieter A van Doorn, Adája E Baars, W Ludo van der Pol, H Stephan Goedee, Anja Ten Brinke, S Marieke van Ham, Theo Rispens, Taco W Kuijpers, Filip Eftimov","doi":"10.1111/ene.16409","DOIUrl":"https://doi.org/10.1111/ene.16409","url":null,"abstract":"<p><strong>Background and purpose: </strong>There are concerns for safety regarding SARS-CoV-2 vaccines for patients with autoimmune neuromuscular disease. We compared daily functioning using disease-specific patient-reported outcome measures (PROMs) before and after SARS-CoV-2 vaccinations.</p><p><strong>Methods: </strong>In this substudy of a prospective observational cohort study (Target-to-B!), patients with myasthenia gravis (MG), chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), and idiopathic inflammatory myopathy (IIM) vaccinated against SARS-CoV-2 were included. Surveys of daily functioning (Myasthenia Gravis Activities of Daily Living, Inflammatory Rasch-Built Overall Disability Scale, Multifocal Motor Neuropathy Rasch-Built Overall Disability Scale, and Health Assessment Questionnaire-Disability Index) were sent before first vaccination and every 60 days thereafter for up to 12 months. Regression models were constructed to assess differences in PROM scores related to vaccination, compared to scores unrelated to vaccination. We also assessed the proportion of patients with deterioration of at least the minimal clinically important difference (MCID) between before first vaccination and 60 days thereafter.</p><p><strong>Results: </strong>We included 325 patients (median age = 59 years, interquartile range = 47-67, 156 [48%] female sex), of whom 137 (42%) had MG, 79 (24%) had CIDP, 43 (13%) had MMN, and 66 (20%) had IIM. PROM scores related to vaccination did not differ from scores unrelated to vaccination. In paired PROMs, MCID for deterioration was observed in three of 49 (6%) MG patients, of whom none reported a treatment change. In CIDP, MCID for deterioration was observed in eight of 29 patients (28%), of whom two of eight (25%) reported a treatment change.</p><p><strong>Conclusions: </strong>SARS-CoV-2 vaccination had no effect on daily functioning in patients with autoimmune neuromuscular diseases, confirming its safety in these patients.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142139677","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Wei Yu Chua, Jia Dong James Wang, Claire Kar Min Chan, Ling-Ling Chan, Eng-King Tan
Background and purpose: This study was undertaken to conduct a meta-analysis on the prevalence of aspiration pneumonia (AP) and hospital mortality in Parkinson disease (PD) as well as the risk of AP in PD patients compared to controls.
Methods: We searched MEDLINE and Embase from inception to 19 March 2024 to identify cross-sectional, cohort, and case-control studies comparing the frequency of AP and hospital mortality in PD patients. We computed risk ratios (RRs) with accompanying 95% confidence intervals (CIs) for each study and pooled the results using a random-effects meta-analysis.
Results: A total of 781 studies were initially screened, and 13 studies involving 541,785,587 patients were included. Patients with PD had >3 times higher risk of AP compared to controls (RR = 3.30, 95% CI = 1.82-6.00, p < 0.0001). This increased risk was similar in both cohort studies (RR = 3.01, 95% CI = 1.10-8.24, p = 0.03) and case-control studies (RR = 3.86, 95% CI = 3.84-3.87, p < 0.00001). The prevalence of AP in 12 studies was 2.74% (95% CI = 1.69-4.41), and hospital mortality was 10% in six studies (10.0%, 95% CI = 5.32-18.0). Prevalence of AP was higher in studies with smaller sample size (5.26%, 95% CI = 3.08-8.83 vs. 2.06%, 95% CI = 1.19-3.55, p = 0.02).
Conclusions: Our meta-analysis showed that patients with PD had >3 times higher risk of AP, with an average 2.74% prevalence and 10.0% hospital mortality. Early recognition and treatment of AP in PD patients will help reduce morbidity and mortality. A multidisciplinary holistic approach is needed to address the multifactorial causes of AP.
背景和目的:本研究旨在对帕金森病(PD)吸入性肺炎(AP)的发病率和住院死亡率以及帕金森病患者与对照组相比发生 AP 的风险进行荟萃分析:我们检索了从开始到 2024 年 3 月 19 日的 MEDLINE 和 Embase,以确定比较帕金森病患者吸入性肺炎发生率和住院死亡率的横断面、队列和病例对照研究。我们计算了每项研究的风险比(RRs)及95%置信区间(CIs),并采用随机效应荟萃分析对结果进行了汇总:初步筛选了781项研究,共纳入13项研究,涉及54178587名患者。与对照组相比,PD 患者罹患 AP 的风险要高出 3 倍以上(RR = 3.30,95% CI = 1.82-6.00,P我们的荟萃分析表明,帕金森病患者罹患 AP 的风险是对照组的 3 倍以上,平均发病率为 2.74%,住院死亡率为 10.0%。早期识别和治疗帕金森病患者的 AP 将有助于降低发病率和死亡率。需要采用多学科综合方法来解决导致 AP 的多因素原因。
{"title":"Risk of aspiration pneumonia and hospital mortality in Parkinson disease: A systematic review and meta-analysis.","authors":"Wei Yu Chua, Jia Dong James Wang, Claire Kar Min Chan, Ling-Ling Chan, Eng-King Tan","doi":"10.1111/ene.16449","DOIUrl":"https://doi.org/10.1111/ene.16449","url":null,"abstract":"<p><strong>Background and purpose: </strong>This study was undertaken to conduct a meta-analysis on the prevalence of aspiration pneumonia (AP) and hospital mortality in Parkinson disease (PD) as well as the risk of AP in PD patients compared to controls.</p><p><strong>Methods: </strong>We searched MEDLINE and Embase from inception to 19 March 2024 to identify cross-sectional, cohort, and case-control studies comparing the frequency of AP and hospital mortality in PD patients. We computed risk ratios (RRs) with accompanying 95% confidence intervals (CIs) for each study and pooled the results using a random-effects meta-analysis.</p><p><strong>Results: </strong>A total of 781 studies were initially screened, and 13 studies involving 541,785,587 patients were included. Patients with PD had >3 times higher risk of AP compared to controls (RR = 3.30, 95% CI = 1.82-6.00, p < 0.0001). This increased risk was similar in both cohort studies (RR = 3.01, 95% CI = 1.10-8.24, p = 0.03) and case-control studies (RR = 3.86, 95% CI = 3.84-3.87, p < 0.00001). The prevalence of AP in 12 studies was 2.74% (95% CI = 1.69-4.41), and hospital mortality was 10% in six studies (10.0%, 95% CI = 5.32-18.0). Prevalence of AP was higher in studies with smaller sample size (5.26%, 95% CI = 3.08-8.83 vs. 2.06%, 95% CI = 1.19-3.55, p = 0.02).</p><p><strong>Conclusions: </strong>Our meta-analysis showed that patients with PD had >3 times higher risk of AP, with an average 2.74% prevalence and 10.0% hospital mortality. Early recognition and treatment of AP in PD patients will help reduce morbidity and mortality. A multidisciplinary holistic approach is needed to address the multifactorial causes of AP.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142139678","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Charlotte Lens, Jelle Demeestere, Barbara Casolla, Hanne Christensen, Urs Fischer, Peter Kelly, Carlos Molina, Simona Sacco, Else Charlotte Sandset, Daniel Strbian, Götz Thomalla, Georgios Tsivgoulis, Kris Vanhaecht, Caroline Weltens, Ellen Coeckelberghs, Robin Lemmens
Background and purpose: Guidelines help physicians to provide optimal care for stroke patients, but implementation is challenging due to the quantity of recommendations. Therefore a practical overview related to applicability of recommendations can be of assistance.
Methods: A systematic review was performed on ischaemic stroke guidelines published in scientific journals, covering the whole acute care process for patients with ischaemic stroke. After data extraction, experts rated the recommendations on dimensions of applicability, that is, actionability, feasibility and validity, on a 9-point Likert scale. Agreement was defined as a score of ≥8 by ≥80% of the experts.
Results: Eighteen articles were identified and 48 recommendations were ultimately extracted. Papers were included only if they described the whole acute care process for patients with ischaemic stroke. Data extraction and analysis revealed variation in terms of both content and comprehensiveness of this description. Experts reached agreement on 34 of 48 (70.8%) recommendations in the dimension actionability, for 16 (33.3%) in feasibility and for 15 (31.3%) in validity. Agreement on all three dimensions was reached for seven (14.6%) recommendations: use of a stroke unit, exclusion of intracerebral haemorrhage as differential diagnosis, administration of intravenous thrombolysis, performance of electrocardiography/cardiac evaluation, non-invasive vascular examination, deep venous thrombosis prophylaxis and administration of statins if needed.
Discussion and conclusion: Substantial variation in agreement was revealed on the three dimensions of the applicability of recommendations. This overview can guide stroke physicians in improving the care process and removing barriers where implementation may be hampered by validity and feasibility.
{"title":"From guidelines to clinical practice in care for ischaemic stroke patients: A systematic review and expert opinion.","authors":"Charlotte Lens, Jelle Demeestere, Barbara Casolla, Hanne Christensen, Urs Fischer, Peter Kelly, Carlos Molina, Simona Sacco, Else Charlotte Sandset, Daniel Strbian, Götz Thomalla, Georgios Tsivgoulis, Kris Vanhaecht, Caroline Weltens, Ellen Coeckelberghs, Robin Lemmens","doi":"10.1111/ene.16417","DOIUrl":"https://doi.org/10.1111/ene.16417","url":null,"abstract":"<p><strong>Background and purpose: </strong>Guidelines help physicians to provide optimal care for stroke patients, but implementation is challenging due to the quantity of recommendations. Therefore a practical overview related to applicability of recommendations can be of assistance.</p><p><strong>Methods: </strong>A systematic review was performed on ischaemic stroke guidelines published in scientific journals, covering the whole acute care process for patients with ischaemic stroke. After data extraction, experts rated the recommendations on dimensions of applicability, that is, actionability, feasibility and validity, on a 9-point Likert scale. Agreement was defined as a score of ≥8 by ≥80% of the experts.</p><p><strong>Results: </strong>Eighteen articles were identified and 48 recommendations were ultimately extracted. Papers were included only if they described the whole acute care process for patients with ischaemic stroke. Data extraction and analysis revealed variation in terms of both content and comprehensiveness of this description. Experts reached agreement on 34 of 48 (70.8%) recommendations in the dimension actionability, for 16 (33.3%) in feasibility and for 15 (31.3%) in validity. Agreement on all three dimensions was reached for seven (14.6%) recommendations: use of a stroke unit, exclusion of intracerebral haemorrhage as differential diagnosis, administration of intravenous thrombolysis, performance of electrocardiography/cardiac evaluation, non-invasive vascular examination, deep venous thrombosis prophylaxis and administration of statins if needed.</p><p><strong>Discussion and conclusion: </strong>Substantial variation in agreement was revealed on the three dimensions of the applicability of recommendations. This overview can guide stroke physicians in improving the care process and removing barriers where implementation may be hampered by validity and feasibility.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142139675","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pietro Emiliano Doneddu, Chiara Gallo, Luca Gentile, Dario Cocito, Yuri Falzone, Vincenzo Di Stefano, Maurizio Inghilleri, Giuseppe Cosentino, Sabrina Matà, Anna Mazzeo, Massimiliano Filosto, Erdita Peci, Benedetta Sorrenti, Filippo Brighina, Federica Moret, Elisa Vegezzi, Martina Sperti, Barbara Risi, Eduardo Nobile-Orazio
Background and purpose: This study was undertaken to compare the sensitivity and specificity of the 2010 European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria for multifocal motor neuropathy (MMN) with those of the American Association of Electrodiagnostic Medicine (AAEM).
Methods: Sensitivity and specificity of the two sets of criteria were retrospectively evaluated in 53 patients with MMN and 280 controls with axonal peripheral neuropathy, inflammatory demyelinating polyneuropathy, or amyotrophic lateral sclerosis. Comparison of the utility of nerve conduction studies with different numbers of nerves examined was also assessed.
Results: The 2010 EFNS/PNS criteria had a sensitivity of 47% for definite MMN and 57% for probable/definite MMN, whereas the AAEM criteria had a sensitivity of 28% for definite MMN and 53% for probable/definite MMN. The sensitivity of the AAEM criteria was higher when utilizing area compared to amplitude reduction to define conduction block. Using supportive criteria, the sensitivity of the 2010 EFNS/PNS criteria for probable/definite MMN increased to 64%, and an additional 36% patients fulfilled the criteria (possible MMN). Specificity values for definite and probable/definite MMN were slightly higher with the AAEM criteria (100%) compared to the EFNS/PNS criteria (98.5% and 97%). Extended nerve conduction studies yielded slightly increased diagnostic sensitivity for both sets of criteria without significantly affecting specificity.
Conclusions: In our patient populations, the 2010 EFNS/PNS criteria demonstrated higher sensitivity but slightly lower specificity compared to the AAEM criteria. Extended nerve conduction studies are advised to achieve slightly higher sensitivity while maintaining very high specificity.
{"title":"Comparison of the diagnostic accuracy of the 2010 European Federation of Neurological Societies/Peripheral Nerve Society and American Association of Electrodiagnostic Medicine diagnostic criteria for multifocal motor neuropathy.","authors":"Pietro Emiliano Doneddu, Chiara Gallo, Luca Gentile, Dario Cocito, Yuri Falzone, Vincenzo Di Stefano, Maurizio Inghilleri, Giuseppe Cosentino, Sabrina Matà, Anna Mazzeo, Massimiliano Filosto, Erdita Peci, Benedetta Sorrenti, Filippo Brighina, Federica Moret, Elisa Vegezzi, Martina Sperti, Barbara Risi, Eduardo Nobile-Orazio","doi":"10.1111/ene.16444","DOIUrl":"https://doi.org/10.1111/ene.16444","url":null,"abstract":"<p><strong>Background and purpose: </strong>This study was undertaken to compare the sensitivity and specificity of the 2010 European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria for multifocal motor neuropathy (MMN) with those of the American Association of Electrodiagnostic Medicine (AAEM).</p><p><strong>Methods: </strong>Sensitivity and specificity of the two sets of criteria were retrospectively evaluated in 53 patients with MMN and 280 controls with axonal peripheral neuropathy, inflammatory demyelinating polyneuropathy, or amyotrophic lateral sclerosis. Comparison of the utility of nerve conduction studies with different numbers of nerves examined was also assessed.</p><p><strong>Results: </strong>The 2010 EFNS/PNS criteria had a sensitivity of 47% for definite MMN and 57% for probable/definite MMN, whereas the AAEM criteria had a sensitivity of 28% for definite MMN and 53% for probable/definite MMN. The sensitivity of the AAEM criteria was higher when utilizing area compared to amplitude reduction to define conduction block. Using supportive criteria, the sensitivity of the 2010 EFNS/PNS criteria for probable/definite MMN increased to 64%, and an additional 36% patients fulfilled the criteria (possible MMN). Specificity values for definite and probable/definite MMN were slightly higher with the AAEM criteria (100%) compared to the EFNS/PNS criteria (98.5% and 97%). Extended nerve conduction studies yielded slightly increased diagnostic sensitivity for both sets of criteria without significantly affecting specificity.</p><p><strong>Conclusions: </strong>In our patient populations, the 2010 EFNS/PNS criteria demonstrated higher sensitivity but slightly lower specificity compared to the AAEM criteria. Extended nerve conduction studies are advised to achieve slightly higher sensitivity while maintaining very high specificity.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142139674","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: Computerized cognitive tests may extend the reach of cognitive screening and monitoring to those with mobility issues or living in remote areas. Moreover, it could enable frequent and autonomous remote cognitive assessments in people with multiple sclerosis (pwMS) on account of its reduced economic and organizational costs. This may further improve our understanding of longitudinal trends and significantly improve the standard of care for pwMS living in remote areas or with mobility limitations. We aimed to evaluate the psychometric properties of an electronic Symbol-Digit Modalities Test (eSDMT) designed to allow pwMS to perform a rapid cognitive assessment independently from home using their own PC/laptop.
Methods: Sixty-two participants underwent a neuropsychological evaluation, and then performed the eSDMT in the clinic. Forty-two participants also repeated the eSDMT at home. We assessed concurrent validity (eSDMT vs. oral SDMT), test-retest reliability (in the clinic vs. at home), discriminant validity (pwMS with/without cognitive impairment), and other psychometric characteristics of the eSDMT (effect of age, sex, and education on test scores).
Results: We observed good-to-excellent concurrent validity (r ≥ 0.84, all p < 0.0001) and test-retest reliability (intraclass correlation coefficients [ICCs]>0.87, all p < 0.0001). Discriminant validity was excellent (area under the curves [AUCs] >0.84, all p < 0.0001). eSDMT scores were only slightly influenced by demographic characteristics (all R2 < 0.200).
Conclusions: We provided evidence which supports the use of our eSDMT as a feasible, valid, and reliable remote assessment of cognitive function in pwMS. Future studies will investigate long-term reliability and predictive power.
背景:计算机化认知测试可将认知筛查和监测的范围扩大到行动不便或居住在偏远地区的患者。此外,由于其经济和组织成本较低,还能对多发性硬化症患者(pwMS)进行频繁和自主的远程认知评估。这可能会进一步提高我们对纵向趋势的了解,并显著改善生活在偏远地区或行动不便的多发性硬化症患者的护理标准。我们的目的是评估电子符号-数字模型测试(eSDMT)的心理测量学特性,该测试旨在让残疾人在家中使用自己的个人电脑/笔记本电脑独立进行快速认知评估:62 名参与者接受了神经心理学评估,然后在诊所进行了 eSDMT 测试。42 名参与者还在家中重复了 eSDMT。我们评估了 eSDMT 的并发效度(eSDMT 与口服 SDMT 的比较)、重测信度(在诊所与在家中的比较)、判别效度(有/无认知障碍的 pwMS)以及 eSDMT 的其他心理测量特征(年龄、性别和教育程度对测试分数的影响):我们观察到了良好到出色的并发效度(r ≥ 0.84,所有 p 0.87,所有 p 0.84,所有 p 2 结论:我们提供的证据支持使用我们的 eSDMT 作为对 pwMS 认知功能进行可行、有效和可靠的远程评估。未来的研究将对其长期可靠性和预测能力进行调查。
{"title":"Development and validation of an electronic Symbol-Digit Modalities Test for remote monitoring of people with multiple sclerosis.","authors":"Michelangelo Dini, Giulia Gamberini, Marta Tacchini, Angela Boschetti, Alessandro Gradassi, Luca Chiveri, Mariaemma Rodegher, Giancarlo Comi, Letizia Leocani","doi":"10.1111/ene.16454","DOIUrl":"https://doi.org/10.1111/ene.16454","url":null,"abstract":"<p><strong>Background: </strong>Computerized cognitive tests may extend the reach of cognitive screening and monitoring to those with mobility issues or living in remote areas. Moreover, it could enable frequent and autonomous remote cognitive assessments in people with multiple sclerosis (pwMS) on account of its reduced economic and organizational costs. This may further improve our understanding of longitudinal trends and significantly improve the standard of care for pwMS living in remote areas or with mobility limitations. We aimed to evaluate the psychometric properties of an electronic Symbol-Digit Modalities Test (eSDMT) designed to allow pwMS to perform a rapid cognitive assessment independently from home using their own PC/laptop.</p><p><strong>Methods: </strong>Sixty-two participants underwent a neuropsychological evaluation, and then performed the eSDMT in the clinic. Forty-two participants also repeated the eSDMT at home. We assessed concurrent validity (eSDMT vs. oral SDMT), test-retest reliability (in the clinic vs. at home), discriminant validity (pwMS with/without cognitive impairment), and other psychometric characteristics of the eSDMT (effect of age, sex, and education on test scores).</p><p><strong>Results: </strong>We observed good-to-excellent concurrent validity (r ≥ 0.84, all p < 0.0001) and test-retest reliability (intraclass correlation coefficients [ICCs]>0.87, all p < 0.0001). Discriminant validity was excellent (area under the curves [AUCs] >0.84, all p < 0.0001). eSDMT scores were only slightly influenced by demographic characteristics (all R<sup>2</sup> < 0.200).</p><p><strong>Conclusions: </strong>We provided evidence which supports the use of our eSDMT as a feasible, valid, and reliable remote assessment of cognitive function in pwMS. Future studies will investigate long-term reliability and predictive power.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142132195","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Guoli Wang, Miriam Kessi, Xi Huang, Wen Zhang, Ciliu Zhang, Fang He, Jing Peng, Fei Yin, Lifen Yang
Background: We investigated the proper timing, efficacy and safety of tacrolimus for juvenile myasthenia gravis (JMG).
Methods: We conducted a retrospective cohort study for JMG patients treated with tacrolimus at Xiangya Hospital, Central South University, Changsha, China from 2010 to 2023. The clinical information of patients with a follow-up of more than 1 year was collected. Comparisons of clinical features between groups of patients who achieved therapeutic goal and those who did not achieve therapeutic goal as well as between groups of patients treated with tacrolimus within or after 1 year from JMG onset was carried out.
Results: Forty-three patients were enrolled, of whom 28 achieved therapeutic goal. Tacrolimus reduced glucocorticoids (GC) dosages for the 28 cases and 15 cases discontinued GC completely. Generalized myasthenia gravis (GMG) subtype had an association with a group of patients who achieved therapeutic goal (p = 0.001). Median duration from JMG onset to tacrolimus use was 10.50 months for those who achieved therapeutic goal and 36.00 months for those who did not achieve therapeutic goal (p = 0.010). The median Myasthenia Gravis Activities of Daily Living (MG-ADL) score improved significantly (p = 0.003). The initiation of tacrolimus within 1 year of JMG onset showed an association with achievement of therapeutic goal (p = 0.026). GMG subtype showed an association with a group of patients who received tacrolimus within 1 year (p = <0.001). Tacrolimus side effects were tolerable.
Conclusion: The provision of tacrolimus within 1 year of JMG onset is effective and safe.
{"title":"Treatment of juvenile myasthenia gravis with tacrolimus: A cohort study.","authors":"Guoli Wang, Miriam Kessi, Xi Huang, Wen Zhang, Ciliu Zhang, Fang He, Jing Peng, Fei Yin, Lifen Yang","doi":"10.1111/ene.16466","DOIUrl":"https://doi.org/10.1111/ene.16466","url":null,"abstract":"<p><strong>Background: </strong>We investigated the proper timing, efficacy and safety of tacrolimus for juvenile myasthenia gravis (JMG).</p><p><strong>Methods: </strong>We conducted a retrospective cohort study for JMG patients treated with tacrolimus at Xiangya Hospital, Central South University, Changsha, China from 2010 to 2023. The clinical information of patients with a follow-up of more than 1 year was collected. Comparisons of clinical features between groups of patients who achieved therapeutic goal and those who did not achieve therapeutic goal as well as between groups of patients treated with tacrolimus within or after 1 year from JMG onset was carried out.</p><p><strong>Results: </strong>Forty-three patients were enrolled, of whom 28 achieved therapeutic goal. Tacrolimus reduced glucocorticoids (GC) dosages for the 28 cases and 15 cases discontinued GC completely. Generalized myasthenia gravis (GMG) subtype had an association with a group of patients who achieved therapeutic goal (p = 0.001). Median duration from JMG onset to tacrolimus use was 10.50 months for those who achieved therapeutic goal and 36.00 months for those who did not achieve therapeutic goal (p = 0.010). The median Myasthenia Gravis Activities of Daily Living (MG-ADL) score improved significantly (p = 0.003). The initiation of tacrolimus within 1 year of JMG onset showed an association with achievement of therapeutic goal (p = 0.026). GMG subtype showed an association with a group of patients who received tacrolimus within 1 year (p = <0.001). Tacrolimus side effects were tolerable.</p><p><strong>Conclusion: </strong>The provision of tacrolimus within 1 year of JMG onset is effective and safe.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142125134","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Laura Zanandrea, Roberta Messina, Ilaria Cetta, Federica Genovese, Simone Guerrieri, Fabrizio Vernieri, Claudia Altamura, Sabina Cevoli, Valentina Favoni, Bruno Colombo, Massimo Filippi
Background and purpose: Fremanezumab, a monoclonal antibody targeting the calcitonin gene-related peptide for migraine prevention, is available in monthly (225 mg) and quarterly (675 mg) doses. Previous studies showed efficacy and safety for both regimens, but a real-life comparison is lacking. This study aimed to compare the effectiveness and safety of monthly and quarterly fremanezumab in a real-life setting.
Methods: This Italian, prospective, multicenter study enrolled 95 migraine patients. During a 3-month treatment period, patients received either monthly or quarterly fremanezumab (49 monthly, 46 quarterly). A 6-month treatment period involved 79 patients (43 monthly, 36 quarterly). Monthly headache (MHD) and migraine days (MMD), number of days (AMD) and pills (AMP) of acute medication intake, and Headache Impact Test (HIT-6), Migraine Disability Assessment (MIDAS) test, and Numeric Rating Scale (NRS) scores were recorded at baseline and after 3 and 6 months of treatment. Adverse events (AEs), responder rates, and medication overuse were also investigated.
Results: Both monthly and quarterly treatments led to significant reductions in MMD, MHD, AMP, AMD, HIT-6, MIDAS, and NRS scores after 3 and 6 months. The monthly regimen exhibited a slightly greater reduction in MMD and MHD after the first quarter, with no significant difference observed after 6 months. The most common AE was transient injection-site reaction, without between-group differences. Responder rates and resolution of medication overuse did not significantly differ between the groups.
Conclusions: Both monthly and quarterly regimens were effective and safe, with a tendency for an advantage of the monthly regimen only in the first quarter of treatment.
{"title":"Effectiveness and safety of monthly versus quarterly fremanezumab for migraine prevention: An Italian, multicenter, real-life study.","authors":"Laura Zanandrea, Roberta Messina, Ilaria Cetta, Federica Genovese, Simone Guerrieri, Fabrizio Vernieri, Claudia Altamura, Sabina Cevoli, Valentina Favoni, Bruno Colombo, Massimo Filippi","doi":"10.1111/ene.16410","DOIUrl":"https://doi.org/10.1111/ene.16410","url":null,"abstract":"<p><strong>Background and purpose: </strong>Fremanezumab, a monoclonal antibody targeting the calcitonin gene-related peptide for migraine prevention, is available in monthly (225 mg) and quarterly (675 mg) doses. Previous studies showed efficacy and safety for both regimens, but a real-life comparison is lacking. This study aimed to compare the effectiveness and safety of monthly and quarterly fremanezumab in a real-life setting.</p><p><strong>Methods: </strong>This Italian, prospective, multicenter study enrolled 95 migraine patients. During a 3-month treatment period, patients received either monthly or quarterly fremanezumab (49 monthly, 46 quarterly). A 6-month treatment period involved 79 patients (43 monthly, 36 quarterly). Monthly headache (MHD) and migraine days (MMD), number of days (AMD) and pills (AMP) of acute medication intake, and Headache Impact Test (HIT-6), Migraine Disability Assessment (MIDAS) test, and Numeric Rating Scale (NRS) scores were recorded at baseline and after 3 and 6 months of treatment. Adverse events (AEs), responder rates, and medication overuse were also investigated.</p><p><strong>Results: </strong>Both monthly and quarterly treatments led to significant reductions in MMD, MHD, AMP, AMD, HIT-6, MIDAS, and NRS scores after 3 and 6 months. The monthly regimen exhibited a slightly greater reduction in MMD and MHD after the first quarter, with no significant difference observed after 6 months. The most common AE was transient injection-site reaction, without between-group differences. Responder rates and resolution of medication overuse did not significantly differ between the groups.</p><p><strong>Conclusions: </strong>Both monthly and quarterly regimens were effective and safe, with a tendency for an advantage of the monthly regimen only in the first quarter of treatment.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142132196","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Céline Konecki, Bruno Francou, Kenneth Chappell, Lucie Augey, Guillemette Beaudonnet, Cécile Cauquil, Dalia Dimitri-Boulos, Adeline Not, Clovis Adam, Vianney Poinsignon, Céline Verstuyft, David Adams, Andoni Echaniz-Laguna, Céline Labeyrie
Background and purpose: Small-fiber neuropathy (SFN) affects only unmyelinated and thin myelinated fibers. It may be caused by amyloidogenic mutations of the transthyretin (TTR) gene, but not all TTR gene variants are pathogenic. The nonamyloidogenic c.76G>A (rs1800458) and c.337-18G>C (rs36204272) variants of TTR were recently reported to be associated with SFN. We investigated this putative association by analyzing TTR gene sequencing data retrospectively for two cohorts of patients, one with SFN and a control group.
Methods: In this retrospective single-center study, we analyzed the frequency of the c.76G>A and c.337-18G>C TTR gene variants in a cohort of patients meeting a strict definition of SFN, with or without dysautonomia, a control cohort of patients investigated for nonneurological conditions, and the gnomAD international database.
Results: We included 55 SFN patients in this study, 17 of whom had dysautonomia. The allelic frequencies of the two variants in our cohort of 55 SFN patients were 7.27% for c.76G>A TTR and 5.25% for c.337-18G>C. The frequencies of both variants were statistically similar in the 337 control patients and the gnomAD database.
Conclusions: The c.76G>A and c.337-18G>C TTR gene variants are not associated with SFN.
{"title":"Nonamyloidogenic TTR gene variants c.76G>A and c.337-18G>C are not associated with idiopathic small-fiber neuropathy.","authors":"Céline Konecki, Bruno Francou, Kenneth Chappell, Lucie Augey, Guillemette Beaudonnet, Cécile Cauquil, Dalia Dimitri-Boulos, Adeline Not, Clovis Adam, Vianney Poinsignon, Céline Verstuyft, David Adams, Andoni Echaniz-Laguna, Céline Labeyrie","doi":"10.1111/ene.16461","DOIUrl":"https://doi.org/10.1111/ene.16461","url":null,"abstract":"<p><strong>Background and purpose: </strong>Small-fiber neuropathy (SFN) affects only unmyelinated and thin myelinated fibers. It may be caused by amyloidogenic mutations of the transthyretin (TTR) gene, but not all TTR gene variants are pathogenic. The nonamyloidogenic c.76G>A (rs1800458) and c.337-18G>C (rs36204272) variants of TTR were recently reported to be associated with SFN. We investigated this putative association by analyzing TTR gene sequencing data retrospectively for two cohorts of patients, one with SFN and a control group.</p><p><strong>Methods: </strong>In this retrospective single-center study, we analyzed the frequency of the c.76G>A and c.337-18G>C TTR gene variants in a cohort of patients meeting a strict definition of SFN, with or without dysautonomia, a control cohort of patients investigated for nonneurological conditions, and the gnomAD international database.</p><p><strong>Results: </strong>We included 55 SFN patients in this study, 17 of whom had dysautonomia. The allelic frequencies of the two variants in our cohort of 55 SFN patients were 7.27% for c.76G>A TTR and 5.25% for c.337-18G>C. The frequencies of both variants were statistically similar in the 337 control patients and the gnomAD database.</p><p><strong>Conclusions: </strong>The c.76G>A and c.337-18G>C TTR gene variants are not associated with SFN.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142125133","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}