Victor Garcia, Louise Bicart-Sée, Isabelle Crassard, Nicolas Legris, Mathieu Zuber, Fernando Pico, Céline Guidoux, Michael Obadia, Naouel Boulenoir, Didier Smadja, Mikael Mazighi, Cecile Lavenu-Bombled, Elodie Baudry, Bertrand Lapergue, Guillaume Turc, Philippe Tuppin, Christian Denier
Background and purpose: We aimed to report the characteristics of cerebral venous thrombosis (CVT) in elderly people (aged ≥65 years).
Methods: This multicenter retrospective cohort included elderly patients hospitalized for a first CVT in nine Paris-Ile-de-France hospitals between 2011 and 2021. The estimated incidence was compared to CVT recorded by the French health insurance data system. Lariboisière Hospital's CVT registry allowed comparisons of our elderly cohort with individuals younger than 65 years.
Results: One hundred fourteen patients were included in this study (mean age = 74.2 years, range = 65-93, 61% female). The CVT annual incidence in Ile-de-France was 5.9-7.1 per million elderly individuals versus 8.5 per million nationwide. Headaches and focal deficits were the most common initial clinical features (50% and 51%, respectively), followed by seizures and confusion (40% and 27%). Treatment included anticoagulation (93%) and, rarely, endovascular procedure (2%) or craniectomy (1%). Compared with adult patients aged <65 years (younger adults), elderly patients presented fewer headaches (50% vs. 96%, p < 0.01) and intracranial hypertension (7% vs. 22%, p < 0.01) but more seizures and focal deficits (40% vs. 27% and 51% vs. 38%, respectively, p < 0.01). Underlying cancer, hemopathy, and locoregional infections were more frequent in elderly patients than among younger adults (p < 0.01). The prognosis of patients from our elderly cohort was poorer than that of younger adults; 8% died in the acute phase, and 73% had a favorable outcome at 1 year (vs. 1.7% and 87%, respectively, p < 0.01).
Conclusions: CVT in elderly patients has a specific clinical presentation, epidemiology, and risk factors such as cancer or hemopathy, justifying specialized management.
{"title":"Cerebral venous thrombosis in elderly patients.","authors":"Victor Garcia, Louise Bicart-Sée, Isabelle Crassard, Nicolas Legris, Mathieu Zuber, Fernando Pico, Céline Guidoux, Michael Obadia, Naouel Boulenoir, Didier Smadja, Mikael Mazighi, Cecile Lavenu-Bombled, Elodie Baudry, Bertrand Lapergue, Guillaume Turc, Philippe Tuppin, Christian Denier","doi":"10.1111/ene.16504","DOIUrl":"https://doi.org/10.1111/ene.16504","url":null,"abstract":"<p><strong>Background and purpose: </strong>We aimed to report the characteristics of cerebral venous thrombosis (CVT) in elderly people (aged ≥65 years).</p><p><strong>Methods: </strong>This multicenter retrospective cohort included elderly patients hospitalized for a first CVT in nine Paris-Ile-de-France hospitals between 2011 and 2021. The estimated incidence was compared to CVT recorded by the French health insurance data system. Lariboisière Hospital's CVT registry allowed comparisons of our elderly cohort with individuals younger than 65 years.</p><p><strong>Results: </strong>One hundred fourteen patients were included in this study (mean age = 74.2 years, range = 65-93, 61% female). The CVT annual incidence in Ile-de-France was 5.9-7.1 per million elderly individuals versus 8.5 per million nationwide. Headaches and focal deficits were the most common initial clinical features (50% and 51%, respectively), followed by seizures and confusion (40% and 27%). Treatment included anticoagulation (93%) and, rarely, endovascular procedure (2%) or craniectomy (1%). Compared with adult patients aged <65 years (younger adults), elderly patients presented fewer headaches (50% vs. 96%, p < 0.01) and intracranial hypertension (7% vs. 22%, p < 0.01) but more seizures and focal deficits (40% vs. 27% and 51% vs. 38%, respectively, p < 0.01). Underlying cancer, hemopathy, and locoregional infections were more frequent in elderly patients than among younger adults (p < 0.01). The prognosis of patients from our elderly cohort was poorer than that of younger adults; 8% died in the acute phase, and 73% had a favorable outcome at 1 year (vs. 1.7% and 87%, respectively, p < 0.01).</p><p><strong>Conclusions: </strong>CVT in elderly patients has a specific clinical presentation, epidemiology, and risk factors such as cancer or hemopathy, justifying specialized management.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142375313","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}
Davide Sparasci, Lenka Schilg-Hafer, Bettina Schreiner, Olivier Scheidegger, Anne-Kathrin Peyer, Agustina Maria Lascano, Alex Vicino, Bernhard Friedrich Décard, Pinelopi Tsouni, Andrea Monika Humm, Enea Pianezzi, Giulia Zezza, Thomas Hundsberger, Anelia Dietmann, Hans H Jung, Thierry Kuntzer, Einar Wilder-Smith, Gladys Martinetti-Lucchini, Orlando Petrini, Stefano Fontana, Peter Gowland, Christoph Niederhauser, Claudio Gobbi, Paolo Ripellino
Background and purpose: Infections and vaccinations have been identified as potential immunological triggers of neuralgic amyotrophy (NA), but the exact type and frequency of the preceding agents is unknown.
Methods: This was a multicentre, prospective, observational, matched case-control study. NA was diagnosed by neuromuscular experts according to validated clinical criteria and electrodiagnostic studies. Clinical data and biological samples of NA patients were collected within 90 days from disease onset between June 2018 and December 2023. All NA patients were asked about prior infection and vaccination in the month before disease onset. Serological tests for hepatitis E virus, human immunodeficiency virus, severe acute respiratory syndrome coronavirus 2, Epstein-Barr virus, cytomegalovirus, parvovirus B19, varicella-zoster virus, Borrelia burgdorferi, Mycoplasma pneumoniae and Bartonella henselae were performed in a central laboratory. Each case was matched with a healthy control for age, sex, place of residence and time of blood collection.
Results: Fifty-seven patients and corresponding controls were included. The mean age was 45 years for both groups. NA onset was preceded by a symptomatic infectious trigger confirmed by microbiological tests in 15/57 (26.3%) patients. Coronavirus disease 2019 vaccination was considered a potential trigger in 7/57 (12.3%) subjects. An acute viral infection was associated with a bilateral involvement of the brachial plexus (p = 0.003, Cramèr's V = 0.43).
Conclusions: Confirmed immune triggers (infection or vaccination) preceded disease onset in 22/57 (38.6%) NA cases. We suggest to test NA patients in the acute phase for intracellular antigens, especially in the case of concomitant bilateral involvement and hepatitis.
背景和目的:感染和疫苗接种被认为是神经性肌营养不良症(NA)的潜在免疫诱因,但前者的确切类型和频率尚不清楚:这是一项多中心、前瞻性、观察性、匹配病例对照研究。NA由神经肌肉专家根据有效的临床标准和电诊断学研究进行诊断。在2018年6月至2023年12月期间,收集了NA患者发病后90天内的临床数据和生物样本。所有NA患者均被问及发病前一个月的既往感染和疫苗接种情况。在中心实验室进行了戊型肝炎病毒、人类免疫缺陷病毒、严重急性呼吸系统综合征冠状病毒 2、爱泼斯坦-巴氏病毒、巨细胞病毒、B19 副病毒、水痘-带状疱疹病毒、布氏波氏杆菌、肺炎支原体和鸡巴顿氏菌的血清学检测。每个病例都与健康对照组进行了年龄、性别、居住地和采血时间的匹配:结果:共纳入 57 名患者和相应的对照组。两组患者的平均年龄均为 45 岁。15/57(26.3%)名患者在NA发病前曾有过经微生物检测证实的无症状感染诱因。7/57(12.3%)名受试者认为 2019 年接种的冠状病毒疾病是潜在的诱因。急性病毒感染与双侧臂丛神经受累有关(p = 0.003,Cramèr's V = 0.43):在 22/57 例(38.6%)NA 患者中,经证实的免疫诱因(感染或接种疫苗)发生在发病之前。我们建议对急性期的 NA 患者进行细胞内抗原检测,尤其是在同时伴有双侧受累和肝炎的情况下。
{"title":"Immune triggers preceding neuralgic amyotrophy.","authors":"Davide Sparasci, Lenka Schilg-Hafer, Bettina Schreiner, Olivier Scheidegger, Anne-Kathrin Peyer, Agustina Maria Lascano, Alex Vicino, Bernhard Friedrich Décard, Pinelopi Tsouni, Andrea Monika Humm, Enea Pianezzi, Giulia Zezza, Thomas Hundsberger, Anelia Dietmann, Hans H Jung, Thierry Kuntzer, Einar Wilder-Smith, Gladys Martinetti-Lucchini, Orlando Petrini, Stefano Fontana, Peter Gowland, Christoph Niederhauser, Claudio Gobbi, Paolo Ripellino","doi":"10.1111/ene.16462","DOIUrl":"https://doi.org/10.1111/ene.16462","url":null,"abstract":"<p><strong>Background and purpose: </strong>Infections and vaccinations have been identified as potential immunological triggers of neuralgic amyotrophy (NA), but the exact type and frequency of the preceding agents is unknown.</p><p><strong>Methods: </strong>This was a multicentre, prospective, observational, matched case-control study. NA was diagnosed by neuromuscular experts according to validated clinical criteria and electrodiagnostic studies. Clinical data and biological samples of NA patients were collected within 90 days from disease onset between June 2018 and December 2023. All NA patients were asked about prior infection and vaccination in the month before disease onset. Serological tests for hepatitis E virus, human immunodeficiency virus, severe acute respiratory syndrome coronavirus 2, Epstein-Barr virus, cytomegalovirus, parvovirus B19, varicella-zoster virus, Borrelia burgdorferi, Mycoplasma pneumoniae and Bartonella henselae were performed in a central laboratory. Each case was matched with a healthy control for age, sex, place of residence and time of blood collection.</p><p><strong>Results: </strong>Fifty-seven patients and corresponding controls were included. The mean age was 45 years for both groups. NA onset was preceded by a symptomatic infectious trigger confirmed by microbiological tests in 15/57 (26.3%) patients. Coronavirus disease 2019 vaccination was considered a potential trigger in 7/57 (12.3%) subjects. An acute viral infection was associated with a bilateral involvement of the brachial plexus (p = 0.003, Cramèr's V = 0.43).</p><p><strong>Conclusions: </strong>Confirmed immune triggers (infection or vaccination) preceded disease onset in 22/57 (38.6%) NA cases. We suggest to test NA patients in the acute phase for intracellular antigens, especially in the case of concomitant bilateral involvement and hepatitis.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142371281","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}
Alessandro P Burlina, Renzo Manara, Jessica Carretta, Chiara Cazzorla, Christian Loro, Vincenza Gragnaniello, Alberto B Burlina
Phenylketonuria, the most common inherited metabolic disease, results from a deficiency of phenylalanine hydroxylase enzyme activity that causes high blood phenylalanine levels. Most adults do not adhere to the gold standard therapy: lifelong treatment with a low-phenylalanine diet. Elevated and fluctuating phenylalanine levels in untreated adults can cause white matter abnormalities, neurological symptoms, and cognitive dysfunction (executive function). Pegvaliase, a derivative of the phenylalanine ammonia-lyase enzyme, metabolizes phenylalanine to trans-cinnamic acid and ammonia, and is approved by the US Food and Drug Administration and European Medicines Agency for subcutaneous administration in adults with phenylketonuria and blood phenylalanine concentrations > 600 μmol/L. In clinical trials, it reduced blood phenylalanine, even in patients consuming an unrestricted diet. We report longitudinal results on the first three such adults, in whom phenylalanine levels were quantified monthly, starting 1 year before pegvaliase administration and continuing through achievement of a pegvaliase response (defined as six consecutive monthly blood phenylalanine concentrations < 360 μmol/L while consuming an unrestricted diet). Brain magnetic resonance imaging (MRI) and neuropsychological assessments were performed before starting therapy and after response was achieved. Our results show that all three patients had significantly reduced white matter hyperintensities on brain MRI and improved executive function on neuropsychological assessment, especially on the Paced Auditory Serial Addition Test, which is known to be very sensitive to white matter functioning. To the best of our knowledge, this is the first report of concomitant improvements in cognitive performance and white matter damage after a pharmacological intervention to normalize phenylalanine levels in adults with phenylketonuria consuming an unrestricted diet.
{"title":"Effect of enzyme substitution therapy on brain magnetic resonance imaging and cognition in adults with phenylketonuria: A case series of three patients.","authors":"Alessandro P Burlina, Renzo Manara, Jessica Carretta, Chiara Cazzorla, Christian Loro, Vincenza Gragnaniello, Alberto B Burlina","doi":"10.1111/ene.16508","DOIUrl":"https://doi.org/10.1111/ene.16508","url":null,"abstract":"<p><p>Phenylketonuria, the most common inherited metabolic disease, results from a deficiency of phenylalanine hydroxylase enzyme activity that causes high blood phenylalanine levels. Most adults do not adhere to the gold standard therapy: lifelong treatment with a low-phenylalanine diet. Elevated and fluctuating phenylalanine levels in untreated adults can cause white matter abnormalities, neurological symptoms, and cognitive dysfunction (executive function). Pegvaliase, a derivative of the phenylalanine ammonia-lyase enzyme, metabolizes phenylalanine to trans-cinnamic acid and ammonia, and is approved by the US Food and Drug Administration and European Medicines Agency for subcutaneous administration in adults with phenylketonuria and blood phenylalanine concentrations > 600 μmol/L. In clinical trials, it reduced blood phenylalanine, even in patients consuming an unrestricted diet. We report longitudinal results on the first three such adults, in whom phenylalanine levels were quantified monthly, starting 1 year before pegvaliase administration and continuing through achievement of a pegvaliase response (defined as six consecutive monthly blood phenylalanine concentrations < 360 μmol/L while consuming an unrestricted diet). Brain magnetic resonance imaging (MRI) and neuropsychological assessments were performed before starting therapy and after response was achieved. Our results show that all three patients had significantly reduced white matter hyperintensities on brain MRI and improved executive function on neuropsychological assessment, especially on the Paced Auditory Serial Addition Test, which is known to be very sensitive to white matter functioning. To the best of our knowledge, this is the first report of concomitant improvements in cognitive performance and white matter damage after a pharmacological intervention to normalize phenylalanine levels in adults with phenylketonuria consuming an unrestricted diet.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142371280","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}
Miriam H Terkelsen, Alex Iranzo, Mónica Serradell, Andreas M Baun, Morten G Stokholm, Erik Hvid Danielsen, Karen Østergaard, Marit Otto, Kristina B Svendsen, Mette Møller, Erik L Johnsen, Alicia Garrido, Dolores Vilas, Joan Santamaria, Arne Møller, Carles Gaig, David J Brooks, Per Borghammer, Eduardo Tolosa, Nicola Pavese
Background and purpose: Most patients with isolated rapid eye movement sleep behaviour disorder (iRBD) progress to a parkinsonian alpha-synucleinopathy. However, time to phenoconversion shows great variation. The aim of this study was to investigate whether cholinergic and dopaminergic dysfunction in iRBD patients was associated with impending phenoconversion.
Methods: Twenty-one polysomnography-confirmed iRBD patients underwent baseline 11C-donepezil and 6-Fluoro-(18F)-l-3,4-dihydroxyphenylalanine (18F-DOPA) positron emission tomography (PET). Potential phenoconversion was monitored for up to 8 years. PET images were analysed according to patients' diagnoses after 3 and 8 years using linear regression. Time-to-event analysis was made with Cox regression, dividing patients into low and high tracer uptake groups.
Results: Follow-up was accomplished in 17 patients. Eight patients progressed to either Parkinson's disease (n = 4) or dementia with Lewy bodies (n = 4), while nine remained non-phenoconverters. Compared with non-phenoconverters, 8-year phenoconverters had lower mean 11C-donepezil uptake in the parietal (p = 0.032) and frontal cortex (p = 0.042), whereas mean 11C-donepezil uptake in 3-year phenoconverters was lower in the parietal cortex (p = 0.005), frontal cortex (p = 0.025), thalamus (p = 0.043) and putamen (p = 0.049). Phenoconverters within 3 years and 8 years had lower 18F-DOPA uptake in the putamen (p < 0.001). iRBD patients with low parietal 11C-donepezil uptake had a 13.46 (95% confidence interval 1.42;127.21) times higher rate of phenoconversion compared with those with higher uptake (p = 0.023). iRBD patients with low 18F-DOPA uptake in the most affected putamen were all phenoconverters with higher rate of phenoconversion (p = 0.0002).
Conclusions: These findings suggest that cortical cholinergic dysfunction, particularly within the parietal cortex, could be a biomarker candidate for predicting short-term phenoconversion in iRBD patients. This study aligns with previous reports suggesting dopaminergic dysfunction is associated with forthcoming phenoconversion.
{"title":"Cholinergic dysfunction in isolated rapid eye movement sleep behaviour disorder links to impending phenoconversion.","authors":"Miriam H Terkelsen, Alex Iranzo, Mónica Serradell, Andreas M Baun, Morten G Stokholm, Erik Hvid Danielsen, Karen Østergaard, Marit Otto, Kristina B Svendsen, Mette Møller, Erik L Johnsen, Alicia Garrido, Dolores Vilas, Joan Santamaria, Arne Møller, Carles Gaig, David J Brooks, Per Borghammer, Eduardo Tolosa, Nicola Pavese","doi":"10.1111/ene.16503","DOIUrl":"https://doi.org/10.1111/ene.16503","url":null,"abstract":"<p><strong>Background and purpose: </strong>Most patients with isolated rapid eye movement sleep behaviour disorder (iRBD) progress to a parkinsonian alpha-synucleinopathy. However, time to phenoconversion shows great variation. The aim of this study was to investigate whether cholinergic and dopaminergic dysfunction in iRBD patients was associated with impending phenoconversion.</p><p><strong>Methods: </strong>Twenty-one polysomnography-confirmed iRBD patients underwent baseline <sup>11</sup>C-donepezil and 6-Fluoro-(<sup>18</sup>F)-l-3,4-dihydroxyphenylalanine (<sup>18</sup>F-DOPA) positron emission tomography (PET). Potential phenoconversion was monitored for up to 8 years. PET images were analysed according to patients' diagnoses after 3 and 8 years using linear regression. Time-to-event analysis was made with Cox regression, dividing patients into low and high tracer uptake groups.</p><p><strong>Results: </strong>Follow-up was accomplished in 17 patients. Eight patients progressed to either Parkinson's disease (n = 4) or dementia with Lewy bodies (n = 4), while nine remained non-phenoconverters. Compared with non-phenoconverters, 8-year phenoconverters had lower mean <sup>11</sup>C-donepezil uptake in the parietal (p = 0.032) and frontal cortex (p = 0.042), whereas mean <sup>11</sup>C-donepezil uptake in 3-year phenoconverters was lower in the parietal cortex (p = 0.005), frontal cortex (p = 0.025), thalamus (p = 0.043) and putamen (p = 0.049). Phenoconverters within 3 years and 8 years had lower <sup>18</sup>F-DOPA uptake in the putamen (p < 0.001). iRBD patients with low parietal <sup>11</sup>C-donepezil uptake had a 13.46 (95% confidence interval 1.42;127.21) times higher rate of phenoconversion compared with those with higher uptake (p = 0.023). iRBD patients with low <sup>18</sup>F-DOPA uptake in the most affected putamen were all phenoconverters with higher rate of phenoconversion (p = 0.0002).</p><p><strong>Conclusions: </strong>These findings suggest that cortical cholinergic dysfunction, particularly within the parietal cortex, could be a biomarker candidate for predicting short-term phenoconversion in iRBD patients. This study aligns with previous reports suggesting dopaminergic dysfunction is associated with forthcoming phenoconversion.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142364835","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}
Huarong Liu, Bo Li, Ting Lu, Chong Chen, Xi Xiong, Xing Li, Rengui Yang
Background and purpose: Elevated serum lipoprotein(a) (Lp[a]) levels have been linked to an increased incidence of stroke. This systematic review and meta-analysis aimed to evaluate the impact of serum Lp(a) on the functional outcomes of patients with ischemic stroke (IS).
Methods: We conducted a comprehensive search of the MEDLINE, Web of Science, Embase, Wanfang, and China National Knowledge Infrastructure databases to identify relevant cohort studies. A random effects model was utilized to synthesize the data, accounting for study heterogeneity.
Results: The analysis included 11 cohort studies comprising 11,958 patients with IS. Pooled results indicated that high baseline Lp(a) levels were associated with an increased risk of poor functional outcomes during follow-up (odds ratio [OR] = 2.13, 95% confidence interval = 1.67-2.71, p < 0.001, I2 = 62%). Subgroup analyses revealed that the relationship between high Lp(a) levels and the risk of poor functional outcomes was more pronounced at discharge (OR = 3.25), 3 months (OR = 2.02), and 6 months (OR = 2.11) poststroke, compared to 12 months (OR = 1.25, p for subgroup difference < 0.001). Furthermore, the association was attenuated yet remained significant in studies adjusting for low-density lipoprotein cholesterol (LDL-C) compared to those that did not adjust for LDL-C (OR = 1.69 vs. 2.63, p for subgroup difference = 0.03).
Conclusions: High serum Lp(a) levels at baseline are significantly associated with poor functional outcomes in patients with IS.
{"title":"Impact of elevated lipoprotein(a) levels on the functional outcomes of ischemic stroke patients: A systematic review and meta-analysis.","authors":"Huarong Liu, Bo Li, Ting Lu, Chong Chen, Xi Xiong, Xing Li, Rengui Yang","doi":"10.1111/ene.16501","DOIUrl":"10.1111/ene.16501","url":null,"abstract":"<p><strong>Background and purpose: </strong>Elevated serum lipoprotein(a) (Lp[a]) levels have been linked to an increased incidence of stroke. This systematic review and meta-analysis aimed to evaluate the impact of serum Lp(a) on the functional outcomes of patients with ischemic stroke (IS).</p><p><strong>Methods: </strong>We conducted a comprehensive search of the MEDLINE, Web of Science, Embase, Wanfang, and China National Knowledge Infrastructure databases to identify relevant cohort studies. A random effects model was utilized to synthesize the data, accounting for study heterogeneity.</p><p><strong>Results: </strong>The analysis included 11 cohort studies comprising 11,958 patients with IS. Pooled results indicated that high baseline Lp(a) levels were associated with an increased risk of poor functional outcomes during follow-up (odds ratio [OR] = 2.13, 95% confidence interval = 1.67-2.71, p < 0.001, I<sup>2</sup> = 62%). Subgroup analyses revealed that the relationship between high Lp(a) levels and the risk of poor functional outcomes was more pronounced at discharge (OR = 3.25), 3 months (OR = 2.02), and 6 months (OR = 2.11) poststroke, compared to 12 months (OR = 1.25, p for subgroup difference < 0.001). Furthermore, the association was attenuated yet remained significant in studies adjusting for low-density lipoprotein cholesterol (LDL-C) compared to those that did not adjust for LDL-C (OR = 1.69 vs. 2.63, p for subgroup difference = 0.03).</p><p><strong>Conclusions: </strong>High serum Lp(a) levels at baseline are significantly associated with poor functional outcomes in patients with IS.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142344101","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}
David García-Azorín, Laura Santana-López, Ana Ordax-Díez, José Eugenio Lozano-Alonso, Diego Macias Saint-Gerons, Yésica González-Osorio, Silvia Rojo-Rello, José M Eiros, Javier Sánchez-Martínez, Álvaro Sierra-Mencía, Andrea Recio-García, Ángel Luis Guerrero-Peral, Ivan Sanz-Muñoz
{"title":"Improved comprehension of influenza-related headaches: Perspectives and suggestions for incidence and prevalence of headache in influenza-Response.","authors":"David García-Azorín, Laura Santana-López, Ana Ordax-Díez, José Eugenio Lozano-Alonso, Diego Macias Saint-Gerons, Yésica González-Osorio, Silvia Rojo-Rello, José M Eiros, Javier Sánchez-Martínez, Álvaro Sierra-Mencía, Andrea Recio-García, Ángel Luis Guerrero-Peral, Ivan Sanz-Muñoz","doi":"10.1111/ene.16478","DOIUrl":"https://doi.org/10.1111/ene.16478","url":null,"abstract":"","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142344102","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}
Romana Konvalinkova, Martin Srp, Kristyna Doleckova, Vaclav Capek, Ota Gal, Martina Hoskovcova, Radim Kliment, Jan Muzik, Evzen Ruzicka, Jiri Klempir
Background and purpose: Dysfunction of the airway defence system in Huntington's disease (HD) is a significant but often overlooked problem. Although expiratory muscle strength training (EMST) is frequently utilized in cough effectiveness treatment, its specific impact in HD patients has not yet been explored. This study investigated the effects of EMST on voluntary peak cough flow (vPCF) in HD patients and evaluated the retention of potential gains post-intervention.
Methods: In this prospective case-controlled trial, 29 HD patients completed an 8-week wait-to-start period, which served to identify the natural development of expiratory muscle strength and vPCF. This was followed by 8 weeks of EMST training and an additional 8 weeks of follow-up. The study's outcome parameters, vPCF and maximum expiratory pressure (MEP), were measured against those of age- and sex-matched healthy controls.
Results: Huntington's disease patients had significantly lower MEP (p < 0.001) and vPCF (p = 0.012) compared to healthy controls at baseline. Following the EMST, significant improvements in MEP (d = 1.39, p < 0.001) and vPCF (d = 0.77, p = 0.001) were observed, with HD patients reaching the cough performance levels of healthy subjects. However, these gains diminished during the follow-up, with a significant decline in vPCF (d = -0.451, p = 0.03) and in MEP (d = -0.71; p = 0.002).
Conclusions: Expiratory muscle strength training improves expiratory muscle strength and voluntary cough effectiveness in HD patients, but an ongoing maintenance programme is necessary to sustain the improvements.
背景和目的:亨廷顿氏病(Huntington's disease,HD)患者的气道防御系统功能障碍是一个重要问题,但往往被忽视。尽管呼气肌力训练(EMST)经常被用于咳嗽有效性治疗,但其对 HD 患者的具体影响尚未得到探讨。本研究调查了呼气肌力训练对 HD 患者自主咳嗽峰值流量(vPCF)的影响,并评估了干预后潜在收益的保持情况:在这项前瞻性病例对照试验中,29 名血液透析患者完成了为期 8 周的等待开始期,以确定呼气肌力和自主咳嗽流量的自然发展情况。随后进行为期8周的EMST训练和另外8周的随访。研究的结果参数,即呼气肌力和最大呼气压力(MEP),与年龄和性别匹配的健康对照组进行了对比测量:结果发现:亨廷顿氏病患者的最大呼气压力明显降低(p 结论:亨廷顿氏病患者的最大呼气压力明显降低:呼气肌力训练可改善 HD 患者的呼气肌力和自主咳嗽效果,但要保持这种改善效果,还需要持续的维护计划。
{"title":"The impact of expiratory muscle strength training on voluntary cough effectiveness in Huntington's disease.","authors":"Romana Konvalinkova, Martin Srp, Kristyna Doleckova, Vaclav Capek, Ota Gal, Martina Hoskovcova, Radim Kliment, Jan Muzik, Evzen Ruzicka, Jiri Klempir","doi":"10.1111/ene.16500","DOIUrl":"https://doi.org/10.1111/ene.16500","url":null,"abstract":"<p><strong>Background and purpose: </strong>Dysfunction of the airway defence system in Huntington's disease (HD) is a significant but often overlooked problem. Although expiratory muscle strength training (EMST) is frequently utilized in cough effectiveness treatment, its specific impact in HD patients has not yet been explored. This study investigated the effects of EMST on voluntary peak cough flow (vPCF) in HD patients and evaluated the retention of potential gains post-intervention.</p><p><strong>Methods: </strong>In this prospective case-controlled trial, 29 HD patients completed an 8-week wait-to-start period, which served to identify the natural development of expiratory muscle strength and vPCF. This was followed by 8 weeks of EMST training and an additional 8 weeks of follow-up. The study's outcome parameters, vPCF and maximum expiratory pressure (MEP), were measured against those of age- and sex-matched healthy controls.</p><p><strong>Results: </strong>Huntington's disease patients had significantly lower MEP (p < 0.001) and vPCF (p = 0.012) compared to healthy controls at baseline. Following the EMST, significant improvements in MEP (d = 1.39, p < 0.001) and vPCF (d = 0.77, p = 0.001) were observed, with HD patients reaching the cough performance levels of healthy subjects. However, these gains diminished during the follow-up, with a significant decline in vPCF (d = -0.451, p = 0.03) and in MEP (d = -0.71; p = 0.002).</p><p><strong>Conclusions: </strong>Expiratory muscle strength training improves expiratory muscle strength and voluntary cough effectiveness in HD patients, but an ongoing maintenance programme is necessary to sustain the improvements.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142344107","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}
{"title":"Conclusion.","authors":"Paul Boon, Elena Moro","doi":"10.1111/ene.16474","DOIUrl":"https://doi.org/10.1111/ene.16474","url":null,"abstract":"","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142344099","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}
E Vichayanrat, C Hentzen, S Simeoni, M Pakzad, V Iodice, Jalesh N Panicker
Background and purpose: Pure autonomic failure (PAF) presents primarily as cardiovascular autonomic failure and may phenoconvert to other neurodegenerative disorders. However, the involvement of other autonomic functions has been poorly evaluated. This study aims to characterize genitourinary and bowel dysfunction and explore their relationship with cardiovascular autonomic dysfunction.
Methods: Pure autonomic failure patients underwent cardiovascular autonomic testing and an assessment of pelvic autonomic dysfunction using urinary, sexual symptoms questionnaires and a bladder diary. Demographic, clinical features and related medical comorbidities were assessed.
Results: Twenty-five patients (10 males) with PAF were included (mean age 71 ± 8 years; disease duration 13 ± 8 years). 96% (24/25) reported lower urinary tract symptoms, of which overactive bladder symptoms were most commonly reported (n = 23; 92%; median overactive subscore 8, interquartile range [IQR] 3-11), followed by voiding difficulties (n = 19; 76%; median low stream subscore 2, IQR 1-3) using the Urinary Symptom Profile; however, only four (16%) required clean intermittent self-catheterization. Sexual dysfunction was common (n = 21; 84%) using the Arizona Sexual Experience Scale. Mild faecal incontinence and constipation were reported. 86% (19/22) had nocturnal polyuria (NP) and the median NP index was 47% (IQR 38%-51%; normal range <33%). 77% (10/13) had voiding dysfunction and 31% (4/13) had post-void residual urine >100 mL. There were no significant correlations between the need for catheterization and the degree of NP with age, disease duration and cardiovascular autonomic parameters (p > 0.05).
Conclusions: Nocturnal polyuria, genitourinary and bowel symptoms are commonly seen in PAF. The pathophysiology of NP in PAF is most likely multifactorial and may occur independent of cardiovascular autonomic failure.
{"title":"Pelvic autonomic dysfunction is common in patients with pure autonomic failure.","authors":"E Vichayanrat, C Hentzen, S Simeoni, M Pakzad, V Iodice, Jalesh N Panicker","doi":"10.1111/ene.16486","DOIUrl":"https://doi.org/10.1111/ene.16486","url":null,"abstract":"<p><strong>Background and purpose: </strong>Pure autonomic failure (PAF) presents primarily as cardiovascular autonomic failure and may phenoconvert to other neurodegenerative disorders. However, the involvement of other autonomic functions has been poorly evaluated. This study aims to characterize genitourinary and bowel dysfunction and explore their relationship with cardiovascular autonomic dysfunction.</p><p><strong>Methods: </strong>Pure autonomic failure patients underwent cardiovascular autonomic testing and an assessment of pelvic autonomic dysfunction using urinary, sexual symptoms questionnaires and a bladder diary. Demographic, clinical features and related medical comorbidities were assessed.</p><p><strong>Results: </strong>Twenty-five patients (10 males) with PAF were included (mean age 71 ± 8 years; disease duration 13 ± 8 years). 96% (24/25) reported lower urinary tract symptoms, of which overactive bladder symptoms were most commonly reported (n = 23; 92%; median overactive subscore 8, interquartile range [IQR] 3-11), followed by voiding difficulties (n = 19; 76%; median low stream subscore 2, IQR 1-3) using the Urinary Symptom Profile; however, only four (16%) required clean intermittent self-catheterization. Sexual dysfunction was common (n = 21; 84%) using the Arizona Sexual Experience Scale. Mild faecal incontinence and constipation were reported. 86% (19/22) had nocturnal polyuria (NP) and the median NP index was 47% (IQR 38%-51%; normal range <33%). 77% (10/13) had voiding dysfunction and 31% (4/13) had post-void residual urine >100 mL. There were no significant correlations between the need for catheterization and the degree of NP with age, disease duration and cardiovascular autonomic parameters (p > 0.05).</p><p><strong>Conclusions: </strong>Nocturnal polyuria, genitourinary and bowel symptoms are commonly seen in PAF. The pathophysiology of NP in PAF is most likely multifactorial and may occur independent of cardiovascular autonomic failure.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142344104","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}
Mustafa Yildirim, Christian Salbach, Christoph Reich, Regina Pribe-Wolferts, Barbara Ruth Milles, Tobias Täger, Matthias Mueller-Hennessen, Markus Weiler, Benjamin Meder, Norbert Frey, Evangelos Giannitsis
Background and purpose: Sparse information is available on the correct interpretation of elevated high-sensitivity cardiac troponin (hs-cTn) in confirmed muscular dystrophies.
Methods: Serum concentrations of hs-cTn T (hs-cTnT) and hs-cTn I (hs-cTnI) were determined in 35 stable outpatients with confirmed skeletal muscle dystrophies. We calculated sensitivities, specificities, and positive and negative predictive values of hs-cTnT and hs-cTnI for identification of cardiac involvement using a comprehensive definition that included diastolic left ventricular and right ventricular function, strain analysis using two-dimensional transthoracic echocardiogram and magnetic resonance imaging, myocardial biopsies, and consideration of a variety of triggers for cardiac injury, including arrhythmias, conduction disorders, and hypoxemia due to respiratory failure.
Results: Cardiac involvement was diagnosed in 34 of 35 cases. Specificities of hs-cTnT increased from 12.5% to 100% (p = 0.0006) applying the comprehensive definition compared to a definition based on electrocardiography and echocardiography alone. At the recommended 99th percentile upper limit of normal, sensitivities were significantly lower for hs-cTnI than for hs-cTnT (29.4% vs. 100%, p = 0.0164). Conversely, the specificities of hs-cTnT and hs-cTnI increased to 100% when using the comprehensive definition criteria for diagnosing cardiac involvement.
Conclusions: Elevated hs-cTnT but not hs-cTnI discriminates cardiac involvement in cases with confirmed skeletal muscle dystrophies with very high sensitivity and 100% specificity. Prior reports on worse performance may be explained by the use of less sensitive imaging methods or incomplete assessment of cardiac involvement.
{"title":"Improved diagnostic performance of high-sensitivity cardiac troponins in muscle dystrophies using comprehensive definition criteria for cardiac involvement: A longitudinal study on 35 patients.","authors":"Mustafa Yildirim, Christian Salbach, Christoph Reich, Regina Pribe-Wolferts, Barbara Ruth Milles, Tobias Täger, Matthias Mueller-Hennessen, Markus Weiler, Benjamin Meder, Norbert Frey, Evangelos Giannitsis","doi":"10.1111/ene.16498","DOIUrl":"https://doi.org/10.1111/ene.16498","url":null,"abstract":"<p><strong>Background and purpose: </strong>Sparse information is available on the correct interpretation of elevated high-sensitivity cardiac troponin (hs-cTn) in confirmed muscular dystrophies.</p><p><strong>Methods: </strong>Serum concentrations of hs-cTn T (hs-cTnT) and hs-cTn I (hs-cTnI) were determined in 35 stable outpatients with confirmed skeletal muscle dystrophies. We calculated sensitivities, specificities, and positive and negative predictive values of hs-cTnT and hs-cTnI for identification of cardiac involvement using a comprehensive definition that included diastolic left ventricular and right ventricular function, strain analysis using two-dimensional transthoracic echocardiogram and magnetic resonance imaging, myocardial biopsies, and consideration of a variety of triggers for cardiac injury, including arrhythmias, conduction disorders, and hypoxemia due to respiratory failure.</p><p><strong>Results: </strong>Cardiac involvement was diagnosed in 34 of 35 cases. Specificities of hs-cTnT increased from 12.5% to 100% (p = 0.0006) applying the comprehensive definition compared to a definition based on electrocardiography and echocardiography alone. At the recommended 99th percentile upper limit of normal, sensitivities were significantly lower for hs-cTnI than for hs-cTnT (29.4% vs. 100%, p = 0.0164). Conversely, the specificities of hs-cTnT and hs-cTnI increased to 100% when using the comprehensive definition criteria for diagnosing cardiac involvement.</p><p><strong>Conclusions: </strong>Elevated hs-cTnT but not hs-cTnI discriminates cardiac involvement in cases with confirmed skeletal muscle dystrophies with very high sensitivity and 100% specificity. Prior reports on worse performance may be explained by the use of less sensitive imaging methods or incomplete assessment of cardiac involvement.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.5,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142344103","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}