{"title":"Tracking myasthenia gravis severity over time: Insights from the French health insurance claims database.","authors":"Shahram Attarian, Jean-Philippe Camdessanché, Andoni Echaniz-Laguna, Mariana Ciumas, Cécile Blein, Benjamin Grenier, Guilhem Solé","doi":"10.1111/ene.16518","DOIUrl":"https://doi.org/10.1111/ene.16518","url":null,"abstract":"<p><strong>Background and purpose: </strong>Few data are available on the course of myasthenia gravis (MG) regarding disease severity and stability over time in real-world settings. This study used the French National Health Insurance Database (SNDS) to assess markers of disease severity in patients with MG longitudinally.</p><p><strong>Methods: </strong>All patients with MG-related claims in the SNDS between 2013 and 2020 were identified. Patients were followed for up to 8 years after the first claim. Intensive care unit (ICU) stays, treatment with intravenous immunoglobulin (IVIg) or plasma exchange (PE), and death were documented throughout the follow-up period. Standardized mortality rates were estimated, and mortality-related variables were identified using a Cox model.</p><p><strong>Results: </strong>In all, 14,459 individuals constituted the full study population, including 6354 incident patients. In the incident population, 2199 (34.6%) were admitted to ICUs at least once, principally during the first year after the index date (N = 1477; 23.3%). This proportion decreased progressively to reach 3.0% in the seventh year. A total of 2817 patients received IVIg and 432 PE, again principally in the first year. In the full study population, the standardized mortality rate was 1.08 (95% confidence interval [CI] 1.03-1.13), being lower in men (0.95, 95% CI 0.89-1.02) than in women (1.15, 95% CI 1.07-1.23) and in patients aged >65 years (1.06, 95% CI 1.01-1.11) than in younger patients (1.50, 95% CI 1.24-1.76). Male gender, older age and higher comorbidity were independently associated with mortality.</p><p><strong>Conclusions: </strong>A subgroup of patients with MG require ICU admission and rescue therapy with IVIg or PE, indicative of poor disease control. New therapies are needed to improve disease control and reduce disease burden.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":" ","pages":"e16518"},"PeriodicalIF":4.5,"publicationDate":"2024-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142567826","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}
Antonio Esposito, Fabrizia Falco, Giulia Scalia, Laura Gentile, Antonio Luca Spiezia, Giuseppe Corsini, Rosa Manganiello, Martina Eliano, Federica Lamagna, Marcello Moccia, Maria Petracca, Roberta Lanzillo, Vincenzo Brescia Morra, Antonio Carotenuto
{"title":"Association between CD20<sup>+</sup> T lymphocytes and neuropsychological findings in multiple sclerosis.","authors":"Antonio Esposito, Fabrizia Falco, Giulia Scalia, Laura Gentile, Antonio Luca Spiezia, Giuseppe Corsini, Rosa Manganiello, Martina Eliano, Federica Lamagna, Marcello Moccia, Maria Petracca, Roberta Lanzillo, Vincenzo Brescia Morra, Antonio Carotenuto","doi":"10.1111/ene.16536","DOIUrl":"https://doi.org/10.1111/ene.16536","url":null,"abstract":"<p><strong>Background and purpose: </strong>CD20<sup>+</sup> T lymphocytes are a subset of circulating T cells presenting the CD20<sup>+</sup> receptor, a molecular marker of B lineage. CD20<sup>+</sup> T lymphocytes are thought to play a pivotal role in multiple sclerosis (MS) pathology, especially at progressive stages. We aimed to investigate the correlation between CD20<sup>+</sup> T lymphocytes and neuropsychological features (i.e., cognition, depression, anxiety, fatigue, and sleep quality) in MS patients.</p><p><strong>Methods: </strong>We enrolled 90 MS patients. Each patient underwent cognitive assessment (Brief International Cognitive Assessment for Multiple Sclerosis) and psychometric assessment (modified Fatigue Impact Scale, Beck Anxiety Inventory, Beck Depression Inventory, Pittsburgh Sleep Quality Index). Cognitive status was defined through the cerebral functional score.</p><p><strong>Results: </strong>Forty-four of 90 patients were relapsing-remitting (49%) and 46 were progressive patients (51%). Seventy patients (18.9%) showed CD20<sup>+</sup> T lymphocytes in peripheral blood with a mean level of 0.38 ± 1.2%. Patients with CD20<sup>+</sup> T lymphocytes were more likely to be at progressive phases (76.5% vs. 23.5%, p = 0.02) and showed a higher Expanded Disability Status Scale score (median [range] = 6.0 [1.5-7.5] vs. 3.5 [1-7.5], p = 0.001). Moreover, patients with CD20<sup>+</sup> T lymphocytes showed worse cognitive functioning (p = 0.004), higher global fatigue symptoms (p = 0.02), higher cognitive fatigue (p = 0.01), higher psychosocial fatigue (p = 0.005), and a trend toward worse sleep quality (p = 0.06).</p><p><strong>Conclusions: </strong>The presence of CD20<sup>+</sup> T lymphocytes in the peripheral blood of MS patients was associated with worse neuropsychological functioning and progressive disease stages. Peripheral CD20<sup>+</sup> T lymphocytes could potentially serve as markers for both disease progression and development of fatigue in MS patients.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":" ","pages":"e16536"},"PeriodicalIF":4.5,"publicationDate":"2024-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142544517","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}
Hanna Jokinen, Hanna M Laakso, Anne Arola, Teemu I Paajanen, Jussi Virkkala, Teppo Särkämö, Tommi Makkonen, Iiris Kyläheiko, Heidi Heinonen, Johanna Pitkänen, Antti Korvenoja, Susanna Melkas
{"title":"Executive functions and processing speed in covert cerebral small vessel disease.","authors":"Hanna Jokinen, Hanna M Laakso, Anne Arola, Teemu I Paajanen, Jussi Virkkala, Teppo Särkämö, Tommi Makkonen, Iiris Kyläheiko, Heidi Heinonen, Johanna Pitkänen, Antti Korvenoja, Susanna Melkas","doi":"10.1111/ene.16533","DOIUrl":"https://doi.org/10.1111/ene.16533","url":null,"abstract":"<p><strong>Background and purpose: </strong>Executive dysfunction and slowed processing speed are central cognitive impairments in cerebral small vessel disease (cSVD). It is unclear whether the subcomponents of executive functions become equally affected and whether computerized tests are more sensitive in detecting early cognitive changes over traditional tests. The associations of specific executive abilities (cognitive flexibility, inhibitory control, working memory) and processing speed with white matter hyperintensities (WMHs) and Instrumental Activities of Daily Living (IADL) were examined.</p><p><strong>Methods: </strong>In the Helsinki Small Vessel Disease Study, 152 older individuals without stroke or dementia were assessed with brain magnetic resonance imaging and comprehensive neuropsychological evaluation. WMH volumes were obtained with automated segmentation. Executive functions and processing speed measures included established paper-and-pencil tests and the computer-based Flexible Attention Test (FAT), Simon task and Sustained Attention to Response Task.</p><p><strong>Results: </strong>White matter hyperintensity volume and IADL were associated with multiple cognitive measures across subdomains independently of demographic factors. The highest effect sizes were observed for FAT numbers and number-letter tasks (tablet modifications from the Trail Making Test), FAT visuospatial span, Simon task and semantic verbal fluency. Some of the widely used tests such as Stroop inhibition, phonemic fluency and digit span were not significantly associated with either WMHs or IADL.</p><p><strong>Conclusion: </strong>Processing speed and executive function subcomponents are broadly related to functional abilities and WMH severity in covert cSVD, but the strength of associations within subdomains is heavily dependent on the assessment method. Digital tests providing precise measures of reaction times and response accuracy seem to outperform many of the conventional paper-and-pencil tests.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":" ","pages":"e16533"},"PeriodicalIF":4.5,"publicationDate":"2024-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142544519","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 Berek, Anna Lindner, Philipp Kindl, Franziska Di Pauli, Alois J Schiefecker, Bettina Pfausler, Raimund Helbok, Florian Deisenhammer, Ronny Beer, Verena Rass, Harald Hegen
{"title":"Cerebrospinal fluid red blood cells and total protein are associated with clinical outcome in spontaneous subarachnoid hemorrhage.","authors":"Klaus Berek, Anna Lindner, Philipp Kindl, Franziska Di Pauli, Alois J Schiefecker, Bettina Pfausler, Raimund Helbok, Florian Deisenhammer, Ronny Beer, Verena Rass, Harald Hegen","doi":"10.1111/ene.16456","DOIUrl":"https://doi.org/10.1111/ene.16456","url":null,"abstract":"<p><strong>Background and purpose: </strong>Prognostication in patients with spontaneous subarachnoid hemorrhage (SAH) can be challenging. The aim of this study was to assess whether cerebrospinal fluid (CSF) red blood cell (RBC) count and total protein (TP) concentration are associated with SAH prognosis.</p><p><strong>Methods: </strong>Patients with SAH treated at the neurological intensive care unit (ICU) in Innsbruck were included in this real-world, observational study. Longitudinal CSF samples were collected as part of routine diagnostics. RBC count and CSF TP at the time of admission (RBC<sub>first</sub>, TP<sub>first</sub>), in Week 1 (RBC<sub>Days1-7</sub>, TP<sub>Days1-7</sub>), Week 2 (RBC<sub>Days8-14</sub>, TP<sub>Days8-14</sub>), and Week 3 or thereafter (RBC<sub>Day>14</sub>, TP<sub>Day>14</sub>), the highest detected value (RBC<sub>highest</sub>, TP<sub>highest</sub>), as well as the RBC count adjusted for disease duration (RBC<sub>adjusted</sub>) were assessed. Primary outcomes were good functional outcome after 3 months, defined as modified Rankin scale score ≤2 and ICU survival.</p><p><strong>Results: </strong>A total of 183 SAH patients with a female predominance (69%), a median (interquartile range [IQR]) age of 60 (50-70) years and median (IQR) Hunt and Hess score of 4 (3-5) were included. Multivariable analyses revealed that lower values of RBC<sub>first</sub>, RBC<sub>adjusted</sub>, RBC<sub>highest</sub>, TP<sub>first</sub> and TP<sub>highest</sub> were associated with good functional outcome and hospital survival. Lower TP concentrations in Weeks 1, 2 and 3 were associated with good functional outcome, and in Weeks 1 and 2 with ICU survival. Early RBC measurements (Week 1) were associated with good functional outcome and ICU survival.</p><p><strong>Conclusions: </strong>Low CSF RBC counts and TP concentrations were associated with good functional outcome and ICU survival in a real-world cohort of SAH patients requiring external ventricular drainage.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":" ","pages":"e16456"},"PeriodicalIF":4.5,"publicationDate":"2024-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142544518","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}
Giuseppe Scopelliti, Hilde Henon, Olivier Masheka-Cishesa, Julien Labreuche, Gregory Kuchcinski, Rabih Aboukais, Charlotte Cordonnier, Barbara Casolla
{"title":"Long-term cognitive outcomes after decompressive hemicraniectomy for right-hemisphere large middle cerebral artery ischemic stroke.","authors":"Giuseppe Scopelliti, Hilde Henon, Olivier Masheka-Cishesa, Julien Labreuche, Gregory Kuchcinski, Rabih Aboukais, Charlotte Cordonnier, Barbara Casolla","doi":"10.1111/ene.16492","DOIUrl":"https://doi.org/10.1111/ene.16492","url":null,"abstract":"<p><strong>Background and purpose: </strong>Decompressive hemicraniectomy (DH) improves survival and functional outcome in large middle cerebral artery (MCA) infarcts. However, long-term cognitive outcomes after DH remain underexplored. In a cohort of patients with large right-hemisphere MCA infarction undergoing DH, we assessed the rates of long-term cognitive impairment over 3-year follow-up.</p><p><strong>Methods: </strong>We prospectively evaluated consecutive patients included in the Lille Decompressive Surgery Database (May 2005-April 2022) undergoing DH according to existing guidelines for large hemisphere MCA infarction. We included patients with right-sided stroke and screened with the Mini-Mental State Examination (MMSE) in at least one of the prespecified follow-ups (3-month, 1-year, 3-year). Cognitive impairment was defined as an MMSE score < 24. We included only right-hemisphere strokes to avoid testing biases related to severe aphasia. We compared clinical and neuroimaging data in patients with and without cognitive impairment.</p><p><strong>Results: </strong>Three hundred four patients underwent DH during the study period. Among 3-month survivors, 95 had a right-hemisphere stroke and underwent at least one cognitive screening (median age = 51 years, 56.8% men). Forty-four patients (46.3%) exhibited cognitive impairment at least once during the 3-year follow-up. Baseline characteristics did not significantly differ between patients with and without cognitive impairment. Regarding long-term temporal trends, cognitive impairment was observed in 23 of 76 (30.3%), 25 of 80 (31.3%), and 19 of 66 (28.8%) patients at 3-month, 1-year, and 3-year follow-up, respectively, and it was associated with higher rates of functional disability (all p < 0.05).</p><p><strong>Conclusions: </strong>The persistently high rates of cognitive impairment after DH highlight the importance of cognitive monitoring to improve the long-term management of survivors.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":" ","pages":"e16492"},"PeriodicalIF":4.5,"publicationDate":"2024-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142544520","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 Ziser, Ruben P A van Eijk, Matthew C Kiernan, Allan McRae, Robert D Henderson, David Schultz, Merrilee Needham, Susan Mathers, Pam McCombe, Paul Talman, Steve Vucic
{"title":"Amyotrophic lateral sclerosis established as a multistep process across phenotypes.","authors":"Laura Ziser, Ruben P A van Eijk, Matthew C Kiernan, Allan McRae, Robert D Henderson, David Schultz, Merrilee Needham, Susan Mathers, Pam McCombe, Paul Talman, Steve Vucic","doi":"10.1111/ene.16532","DOIUrl":"https://doi.org/10.1111/ene.16532","url":null,"abstract":"<p><strong>Background and purpose: </strong>Given the accepted multistep process of disease causation in amyotrophic lateral sclerosis (ALS), the present study was undertaken to determine the number of steps required for disease onset across each of the ALS phenotypes.</p><p><strong>Methods: </strong>Clinical and demographic data were prospectively accumulated using the Australian Motor Neurone Disease Registry (2005-2016), and age-specific incidence rates were calculated. Poisson regression was utilized to assess the relationship between log age-specific incidence and log age of onset, with McFadden's R<sup>2</sup> used to assess the goodness of fit of the model.</p><p><strong>Results: </strong>In total, 2647 ALS patients were included, with mean disease-onset age being 62.2 ± 12.1 years. A linear relationship between log incidence and log age was established across ALS phenotypes, with variable slope estimates: bulbar 5.1 (95% confidence interval [CI] 4.6-5.6); cervical 2.7 (95% CI 2.3-3.0); lumbar 3.5 (95% CI 3.2-3.9); flail arm 4.7 (95% CI 3.9-5.5); flail leg 3.6 (95% CI 2.6-4.5); primary lateral sclerosis 2.7 (95% CI 1.8-3.7). Slope estimates were significantly higher in the bulbar compared to the cervical, lumbar and primary lateral sclerosis phenotypes. McFadden's R<sup>2</sup> values were >0.4 for all phenotypes indicating excellent model fit.</p><p><strong>Discussion: </strong>A multistep process has been established across all ALS phenotypes with variable slope estimates, suggesting that the number of steps to develop disease is different across clinical presentations. Identification of mechanisms underlying slope estimate variability could exert pathophysiological significance.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":" ","pages":"e16532"},"PeriodicalIF":4.5,"publicationDate":"2024-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142544516","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}
Benedikt Schoser, Jan B M Kuks, Marianne de Visser
{"title":"Professor Dr med. Stephan Zierz (1954-2024).","authors":"Benedikt Schoser, Jan B M Kuks, Marianne de Visser","doi":"10.1111/ene.16494","DOIUrl":"https://doi.org/10.1111/ene.16494","url":null,"abstract":"","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":" ","pages":"e16494"},"PeriodicalIF":4.5,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142521445","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}
Chloé Laurencin, Aurelia Poujois, Maxime Bonjour, Caroline Demily, Hélène Klinger, Emmanuel Roze, Victoire Leclert, Teodor Danaila, Carole Langlois-Jacques, Eduardo Couchonnal, France Woimant, Mickael Alexandre Obadia, Gwennaelle Perez, Michaela Pernon, Laurianne Blanchet, Emmanuel Broussolle, Marie Vidailhet, Behrouz Kassai, Elena Moro, Carine Karachi, Gustavo Polo, David Grabli, Aurélie Portefaix, Stéphane Thobois
{"title":"Deep brain stimulation for severe dystonia associated with Wilson disease: A prospective multicenter meta-analysis of an N-of-1 trial.","authors":"Chloé Laurencin, Aurelia Poujois, Maxime Bonjour, Caroline Demily, Hélène Klinger, Emmanuel Roze, Victoire Leclert, Teodor Danaila, Carole Langlois-Jacques, Eduardo Couchonnal, France Woimant, Mickael Alexandre Obadia, Gwennaelle Perez, Michaela Pernon, Laurianne Blanchet, Emmanuel Broussolle, Marie Vidailhet, Behrouz Kassai, Elena Moro, Carine Karachi, Gustavo Polo, David Grabli, Aurélie Portefaix, Stéphane Thobois","doi":"10.1111/ene.16524","DOIUrl":"https://doi.org/10.1111/ene.16524","url":null,"abstract":"<p><strong>Background and purpose: </strong>Disabling dystonia despite optimal medical treatment is common in Wilson disease (WD). No controlled study has evaluated the effect of deep brain stimulation (DBS) on dystonia related to WD. This study was undertaken to evaluate the efficacy of DBS on dystonia related to WD.</p><p><strong>Methods: </strong>A meta-analysis of an N-of-1 prospective, randomized, double-blind, multicenter DBS study was conducted at two French WD reference centers. Main inclusion criteria were patients with WD, stabilized for at least 6 months with significant disability due to dystonia despite optimized medical treatment. The subthalamic nucleus (STN) was targeted for bradykinetic patients with tonic dystonia, and the internal globus pallidus (GPi) was chosen for patients with hyperkinetic dystonia. Each patient underwent two periods of DBS \"on\" and two periods of DBS \"off,\" each lasting 4 months. The order of stimulation conditions was randomized. The primary outcome was the change in the Canadian Occupational Performance Measure Performance (COPM-P) and Satisfaction scores after each 4-month period. Secondary outcomes were changes in the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) severity and disability scores and Unified Wilson's Disease Rating Scale (UWDRS) scores.</p><p><strong>Results: </strong>Between 12 May 2016 and 7 October 2022, three patients were included. Two patients received bilateral GPi DBS, and one received bilateral STN DBS. There was no change of COPM-P (p = 0.956), BFMDRS, and UWDRS scores. No serious adverse events were reported.</p><p><strong>Conclusions: </strong>STN or GPi DBS are ineffective on dystonia related to WD.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":" ","pages":"e16524"},"PeriodicalIF":4.5,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142521443","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}
Diego Santos-García, Lydia López-Manzanares, Inés Muro, Pablo Lorenzo-Barreto, Elena Casas Peña, Rocío García-Ramos, Tamara Fernández Valle, Carlos Morata-Martínez, Raquel Baviera-Muñoz, Irene Martínez-Torres, María Álvarez-Sauco, Déborah Alonso-Modino, Inés Legarda, María Fuensanta Valero-García, José Andrés Suárez-Muñoz, Juan Carlos Martínez-Castrillo, Ana Belén Perona, Jose María Salom, Esther Cubo, Caridad Valero-Merino, Nuria López-Ariztegui, Pilar Sánchez Alonso, Sabela Novo Ponte, Elisa Gamo González, Raquel Martín García, Raúl Espinosa, Mar Carmona, Cici Esmerali Feliz, Pedro García Ruíz, Teresa Muñoz Ruíz, Beatriz Fernández Rodríguez, Marina Mata
{"title":"Effectiveness and safety of levodopa-entacapone-carbidopa infusion in Parkinson disease: A real-world data study.","authors":"Diego Santos-García, Lydia López-Manzanares, Inés Muro, Pablo Lorenzo-Barreto, Elena Casas Peña, Rocío García-Ramos, Tamara Fernández Valle, Carlos Morata-Martínez, Raquel Baviera-Muñoz, Irene Martínez-Torres, María Álvarez-Sauco, Déborah Alonso-Modino, Inés Legarda, María Fuensanta Valero-García, José Andrés Suárez-Muñoz, Juan Carlos Martínez-Castrillo, Ana Belén Perona, Jose María Salom, Esther Cubo, Caridad Valero-Merino, Nuria López-Ariztegui, Pilar Sánchez Alonso, Sabela Novo Ponte, Elisa Gamo González, Raquel Martín García, Raúl Espinosa, Mar Carmona, Cici Esmerali Feliz, Pedro García Ruíz, Teresa Muñoz Ruíz, Beatriz Fernández Rodríguez, Marina Mata","doi":"10.1111/ene.16535","DOIUrl":"https://doi.org/10.1111/ene.16535","url":null,"abstract":"<p><strong>Background and purpose: </strong>Levodopa-entacapone-carbidopa intestinal gel (LECIG) infusion is a recently developed device-aided therapy for advanced Parkinson disease (PD) patients. The aim of this study was to report real-world evidence about the effectiveness, tolerability, and safety of LECIG in PD patients.</p><p><strong>Methods: </strong>A multicenter observational retrospective study of the first patients who initiated LECIG in Spain was performed. All neurologists with an experience of at least two patients treated until 30 March 2024 were invited to participate. Data about effectiveness and safety from the medical records (V0, pre-LECIG; V1, initiation of LECIG; V2, post-LECIG follow-up) with a total of 246 variables were collected.</p><p><strong>Results: </strong>Seventy-three PD patients (61.6% males, 70.1 ± 9.1 years old) from 21 Spanish centers with a mean disease duration of 14.4 ± 6.3 years (range = 5-31) were included. Twenty-six patients (35.6%) were switched directly from levodopa-carbidopa intestinal gel. The mean exposure to LECIG was 177.3 ± 110.5 days (range = 7-476). The mean daily OFF time decreased from 5.2 ± 3 (pre-LECIG) to 1.9 ± 1.8 (post-LECIG; n = 66, p < 0.0001). Global improvement was observed in >85% of the patients. No significant change was detected in the levodopa equivalent daily dose from V0 to V2. Only 7% received 24-h infusion, and 24.7% required more than one cartridge per day at V2. Thirty-four patients (46.6%) had at least one adverse event related to LECIG and/or the device system. Five patients (6.8%) discontinued LECIG.</p><p><strong>Conclusions: </strong>LECIG was safe and effective in advanced PD patients.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":" ","pages":"e16535"},"PeriodicalIF":4.5,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142521444","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}
Belén Rodriguez, Lynn Pantano, Tobias Nef, René M Müri, Werner J Z'Graggen
{"title":"Visual symptoms in postural tachycardia syndrome: An investigation of position-dependent visual exploration.","authors":"Belén Rodriguez, Lynn Pantano, Tobias Nef, René M Müri, Werner J Z'Graggen","doi":"10.1111/ene.16507","DOIUrl":"https://doi.org/10.1111/ene.16507","url":null,"abstract":"<p><strong>Background and purpose: </strong>Patients with postural tachycardia syndrome report position-dependent visual symptoms. Despite their impact on daily life, these symptoms have remained largely unexplored in research. The aim of this study was to investigate the nature of visual symptoms in postural tachycardia syndrome and possible underlying pathophysiological mechanisms.</p><p><strong>Methods: </strong>Fifteen patients with postural tachycardia syndrome and 15 healthy controls were included in the study. Through a comprehensive array of measurements, including haemodynamics, subjective symptom assessments, eye movement tracking and pupil diameter analysis, participants were assessed during free image exploration in both supine and 60° head-up tilt positions.</p><p><strong>Results: </strong>During head-up tilt, patients showed a decreased number and duration of fixations, as well as a decreased number, peak velocity and amplitude of saccades compared to the supine position and the control group. This reduction in visual exploration occurred primarily in the peripheral field of view and coincided with the occurrence of subjective visual symptoms. No significant differences in the saccade main sequence were observed between the two groups in either body position.</p><p><strong>Conclusions: </strong>Patients with postural tachycardia syndrome have a reduced exploration of the peripheral field of view when in an upright body position, potentially leading to tunnel vision. Since the normality of the saccade main sequence in patients combined with the focus on the centre of the field of view and the lower saccade amplitudes points to an intact brainstem function, the decrease in peripheral visual exploration may be attributed to a position-dependent dysfunction of the frontal eye field.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":" ","pages":"e16507"},"PeriodicalIF":4.5,"publicationDate":"2024-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142497480","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}