Revue neurologique最新文献

{"title":"New treatment strategies in Myasthenia gravis","authors":"S. Attarian","doi":"10.1016/j.neurol.2024.09.006","DOIUrl":"10.1016/j.neurol.2024.09.006","url":null,"abstract":"<div><div>Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder characterized by muscle weakness and fatigue. The disease is primarily caused by antibodies targeting acetylcholine receptors (AChR) and muscle-specific kinase (MuSK) proteins at the neuromuscular junction. Traditional treatments for MG, such as acetylcholinesterase inhibitors, corticosteroids, and immunosuppressants, have shown efficacy but are often associated with significant long-term side effects and variable patient response rates. Notably, approximately 15% of patients exhibit inadequate responses to these standard therapies. Recent advancements in molecular therapies, including monoclonal antibodies, B cell-depleting agents, complement inhibitors, Fc receptor antagonists, and chimeric antigen receptor (CAR) T cell-based therapies, have introduced promising alternatives for MG treatment. These novel therapeutic approaches offer potential improvements in targeting specific immune pathways involved in MG pathogenesis. This review highlights the progress and challenges in developing and implementing these molecular therapies. It discusses their mechanisms, efficacy, and the potential for personalized medicine in managing MG. The integration of new molecular therapies into clinical practice could significantly transform the treatment landscape of MG, offering more effective and tailored therapeutic options for patients who do not respond adequately to traditional treatments. These innovations underscore the importance of ongoing research and clinical trials to optimize therapeutic strategies and improve the quality of life for individuals with MG.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"180 9","pages":"Pages 971-981"},"PeriodicalIF":2.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142392924","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"40 years of autoantibody research in paraneoplastic neurological syndromes","authors":"F. Graus","doi":"10.1016/j.neurol.2024.07.003","DOIUrl":"10.1016/j.neurol.2024.07.003","url":null,"abstract":"<div><div>Paraneoplastic neurologic syndromes (PNS) are a group of disorders that affect the central and the peripheral nervous system and frequently occur in patients with cancer which usually still is undiagnosed by the time the patient presents the first neurological manifestations. The discovery in the serum and cerebrospinal fluid of PNS patients of antibodies that target tumor antigens that also are normally expressed in the nervous system had a significant impact. First, the research on neuronal antibodies confirmed that most PNS are autoimmune disorders triggered by the underlying cancer supporting the use of immunotherapy to treat them; second, although the first antibodies described recognized intracellular neuronal antigens and therefore they were not pathogenic, these antibodies became robust biomarkers for the strict diagnosis of PNS; and third, the methodological approach used to characterize the first neuronal antibodies paved the way to the identification of antibodies against neuronal surface antigens that are pathogenic and responsible for some PNS and non-paraneoplastic encephalitis. Future studies should address several issues: (1) to improve the efficiency of commercial kits; (2) to provide strict criteria to select which neural antibodies should be used for the diagnosis of PNS; and (3) define in more detail the autoimmune mechanisms responsible for the brain injury in the PNS.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"180 9","pages":"Pages 848-861"},"PeriodicalIF":2.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142256857","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Postural balance and visual dependence in patients with demyelinating neuropathies differ between acquired and hereditary etiologies.","authors":"L Dupont, L Defebvre, J-B Davion, A Delval, C Tard","doi":"10.1016/j.neurol.2024.10.002","DOIUrl":"https://doi.org/10.1016/j.neurol.2024.10.002","url":null,"abstract":"<p><strong>Background: </strong>Demyelinating polyneuropathies affect posture and can be either hereditary, as in Charcot-Marie-Tooth type 1A (CMT1A), or autoimmune, as in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Clinical differentiation between these two neuropathies can be challenging and biomarkers are lacking. No comparative analysis of their balance profiles has been conducted.</p><p><strong>Methods: </strong>The postural balance of 23 patients with CIDP and 23 patients with CMT1A, matched for age, sex, and functional scores, were recorded using a force platform under various conditions. The effects of visual dependence were examined based on center of pressure velocity, 90% confidence ellipse area, and the Romberg quotient which represents the ratio between posturography with eyes closed and eyes open.</p><p><strong>Results: </strong>With eyes open, the two groups exhibited similar area and velocity. They increased their postural sway when visual input was eliminated. Nevertheless, the increase in postural sway was less pronounced in CMT1A patients than in patients with CIDP, who then had a higher Romberg quotient.</p><p><strong>Conclusion: </strong>Patients with CMT1A appear to have developed compensatory mechanisms over time resulting in reduced visual dependence. Further studies are necessary to explore other compensatory mechanisms of equilibrium that could be targeted by rehabilitation for patients with CIDP.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2024-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142506971","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Epidemiology of optic disc edema in 2021/2022: Results from a cohort of 197 patients.","authors":"R Attia, N Stolowy, R Fitoussi, K Mairot, T David","doi":"10.1016/j.neurol.2024.09.010","DOIUrl":"https://doi.org/10.1016/j.neurol.2024.09.010","url":null,"abstract":"<p><strong>Objective: </strong>The aim of our study was to determine the etiologies of optic disc edema between 2021 and 2022.</p><p><strong>Materials and methods: </strong>This was a multicentric study at the Timone and Nord university hospitals in Marseille. Patients were retrospectively followed in ophthalmology departments, with inclusion between January 2021 and December 2022. All patients presenting with newly diagnosed uni- or bilateral optic disc edema, both adults and children, were included. Their ophthalmological evaluation included a fundus examination and optical coherence tomography if feasible.</p><p><strong>Results: </strong>In total, 197 patients were included. Intracranial hypertension (IH) was the most frequent etiology (37.06%). The primary causes of IH were idiopathic (27/73), intracranial tumors (21/73), and cerebral venous thrombosis (12/73). The second etiology of optic disc edema was retinal vein occlusion in 19.9% of cases (39/197). Edema reactive to uveitis was found in 13.2% of cases (26/197). Finally, inflammatory (17/197) and ischemic (30/197) optic neuropathies were identified.</p><p><strong>Conclusion: </strong>This study updates the most frequent etiologies of optic disc edema in 2021 and 2022 to facilitate diagnostic hypotheses for de novo optic disc edema. It highlights the importance of a comprehensive and personalized evaluation in diagnosing optic disc edema, taking into account recent advances in imaging techniques and biomarkers.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2024-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142473656","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"French validation of the sexual complaints screener (SCS) for patients with multiple sclerosis.","authors":"S Bresch, H Joly, R Colamarino, I Bentellis, S Tur, S Fakir, C Burte, C Lebrun-Frenay","doi":"10.1016/j.neurol.2024.09.009","DOIUrl":"https://doi.org/10.1016/j.neurol.2024.09.009","url":null,"abstract":"<p><strong>Background: </strong>Sexual dysfunctions are frequent in the general population and even more frequent in individuals with chronic neurological disorders like multiple sclerosis (MS). Several studies indicate that patients would like healthcare workers to address sexual problems. Indeed, such complaints are not currently sufficiently detected and treated. In clinical practice, a screening tool is lacking to help French-speaking patients and healthcare professionals address this issue.</p><p><strong>Objective: </strong>The main aim of this study was to evaluate the reliability and validity of the French adaptation of the self-assessment Sexual Complaints Screener scale for Women (SCS-W) and Men (SCS-M).</p><p><strong>Method: </strong>A prospective study was conducted among patients with MS in two centers. The SCS-W was adapted into French (QPS-F: questionnaire de plaintes sexuelles - Femmes) and compared to the reference questionnaire Female Sexual Function Index-19 (FSFI-19). The SCS-M was adapted into French (QPS-H: questionnaire de plaintes sexuelles - Hommes) and compared to the reference International Index of Erectile Function-15 (IIEF-15).</p><p><strong>Results: </strong>Included were 101 women and 35 men with MS. Median age was 40.5 (range: 20-68) years. Based on the Cronbach alpha coefficient, the internal coherence of the QPS in French was 0.89 for women (QPS-F) and 0.71 for men (QPS-H), indicating high reliability. For QPS-F, the bivariate Pearson correlation coefficient indicated good convergence for desire and satisfaction, and average convergence for orgasm, pain, and arousal excitability. For QPS-H, the convergence was good for desire, pleasure, and ejaculation.</p><p><strong>Conclusion: </strong>The French versions of the SCS-W/M scales, namely QPS-F and QPS-H, are reliable and validated tools compared with the reference questionnaires, FSFI and IIEF-15, respectively. The QPS-F/H are useful tools for brief, simple, and accurate screening and assessment of sexual complaints. They provide supportive information for clinicians who are less familiar with the clinical significance of sexual complaints and hence can be helpful to achieve more adapted care. These scales are adapted, but not specific, to MS. They could be used in other pathologies and the general population.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2024-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142473657","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital myasthenic syndromes by Epsilon subunit mutations: Phenotypic profiles of 17 Algerian families.","authors":"M I Kediha, M Tazir, D Sternberg, B Eymard, L Ali Pacha","doi":"10.1016/j.neurol.2024.09.007","DOIUrl":"https://doi.org/10.1016/j.neurol.2024.09.007","url":null,"abstract":"<p><strong>Background: </strong>Congenital myasthenic syndromes (CMS) are a heterogeneous group of rare genetic disorders. The acetyl choline receptor contains five subunits, with a predominance of mutations affecting the epsilon subunit gene called cholinergic receptor nicotinic epsilon (CHRNE) gene.</p><p><strong>Objective: </strong>To study the clinical phenotype of 17 families with CHRNE gene mutations.</p><p><strong>Methods: </strong>We report a series of 17 families with 22 affected patients carrying different mutations encoding CHRNE proteins.</p><p><strong>Results: </strong>We studied their clinical and biological phenotypes, as well as their evolutionary profile and their response to the different therapies proposed. A phenotypic comparison was made between the families carrying the founding Maghrebian mutation and the other mutations found in this series.</p><p><strong>Conclusion: </strong>The CHRNE gene mutations are the most frequent ones in CMS. The phenotypes reported in this study are heterogeneous, and can depend on the causative mutation.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142392923","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The “zebra sign” after a lumbar puncture","authors":"","doi":"10.1016/j.neurol.2024.03.010","DOIUrl":"10.1016/j.neurol.2024.03.010","url":null,"abstract":"","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"180 8","pages":"Pages 831-833"},"PeriodicalIF":2.8,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140923007","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mindfulness in Parkinson's disease: A French national survey and a pilot intervention feasibility trial using the MBSR program (M-Park)","authors":"","doi":"10.1016/j.neurol.2024.03.013","DOIUrl":"10.1016/j.neurol.2024.03.013","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Background&lt;/h3&gt;&lt;div&gt;&lt;span&gt;Non-pharmacological complementary interventions, particularly mind-body practices, are of growing importance in the management of Parkinson's disease (PD). Among these, &lt;/span&gt;mindfulness&lt;span&gt;&lt;span&gt; meditation seems particularly effective, especially on anxiety and depression symptoms. However, current knowledge on mindfulness standardized programs in PD is still limited, particularly in France. Aiming at improving this knowledge we designed the M-PARK study in two phases. Phase 1 consisted in a French national survey to explore expectations, needs and initiatives for &lt;/span&gt;mindfulness meditation&lt;span&gt; for PD patients. Phase 2 was a clinical trial&lt;span&gt; with objectives to assess feasibility, acceptability and effects of a mindfulness (MBSR) program proposed to PD patients.&lt;/span&gt;&lt;/span&gt;&lt;/span&gt;&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methods&lt;/h3&gt;&lt;div&gt;&lt;span&gt;In phase 1, online questionnaires were addressed to members of a French PD patient's association (France Parkinson) and French MBSR&lt;span&gt; qualified instructors. In Phase 2, a clinical trial&lt;span&gt; involving 30 PD patients consisted of a standard MBSR program with two additional evaluation visits one month before and after the program. Data collection included a global clinical evaluation, assessment of depression and anxiety symptoms, sleep, pain and &lt;/span&gt;&lt;/span&gt;&lt;/span&gt;quality of life&lt;span&gt; and a face-to-face interview for qualitative assessment of the acceptability and lived experience during the program. Three MBSR programs were proposed to three groups of ten patients: two were online due to the pandemic situation, one proposed to patients with no or minor fluctuations (group 1) and one for patients with slight to moderate fluctuations (group 2), and the last one face-to-face for patients with no or minor fluctuations (group 3).&lt;/span&gt;&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Results&lt;/h3&gt;&lt;div&gt;French survey: 209 responses were collected for the questionnaire sent to the members of the association France Parkinson; and 68 for the questionnaire sent to the instructors. Two-thirds of patients surveyed had heard of mindfulness meditation (66%), but were unaware of what this approach really consisted and how it could really help them. Few instructors (29%) had had to deal with patients with PD in their current practice. Yet 90% of patients surveyed indicated they were in favor of introducing this type of approach into their care.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Clinical trial&lt;/h3&gt;&lt;div&gt;&lt;span&gt;The results indicated that the program is feasible and acceptable both online and face-to-face for patients with PD. Among the 30 patients enrolled, 25 completed the program. No unwanted effects related to mindfulness meditation practice were observed. The results showed a statistically significant reduction in anxiety symptoms, depressive symptoms, and improvement in quality of life. Furthermore, no statistically significant change was measured for pain or sleep quality. There was no striking difference in results observed be","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"180 8","pages":"Pages 777-790"},"PeriodicalIF":2.8,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140945982","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Herpes simplex virus-2 meningo-encephalitis hiding a criminal case of fatal methylmercury poisoning","authors":"","doi":"10.1016/j.neurol.2024.05.004","DOIUrl":"10.1016/j.neurol.2024.05.004","url":null,"abstract":"","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"180 8","pages":"Pages 833-835"},"PeriodicalIF":2.8,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141311598","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Discriminating factors in access to video-EEG for epilepsy surgery in a French tertiary epilepsy center","authors":"","doi":"10.1016/j.neurol.2024.04.003","DOIUrl":"10.1016/j.neurol.2024.04.003","url":null,"abstract":"<div><div><span>Equitable access to care and management is a priority for patients with epilepsy and may vary depending on each country's healthcare system. As this issue has not been specifically addressed in France, we conducted a retrospective study to identify discriminating factors in access to surgery at a French tertiary epilepsy center. Initially, we examined factors previously identified in other countries as influential in surgery access, including age at diagnosis, affected side, gender, years of education, socio-professional categories, and density of general practitioners in the residential area, in 293 consecutive French-native patients with refractory medial temporal lobe epilepsy and </span>hippocampal sclerosis<span> (MTLE-HS). Subsequently, we conducted a case-control study comparing patients born in France with 22 patients born abroad to specifically explore migratory status. The analysis revealed that the only three factors statistically influencing the delay between the onset of epilepsy and entry into video-EEG were early age at onset (associated with a longer delay), pensioner status (associated with a longer delay), and student status (associated with a shorter delay). Migratory status, gender, and socio-economic level (indirectly reflected by the level of education and socio-professional category) were not found to be discriminatory factors in access to video-EEG. Discrepancies between our study and foreign studies may be attributed to differences in healthcare systems and medical coverage among countries. Efforts in France to improve access to surgery should focus on enhancing communication among practitioners to promptly refer any MTLE-HS patient to an epilepsy surgery center, regardless of their age.</span></div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"180 8","pages":"Pages 770-776"},"PeriodicalIF":2.8,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141162065","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}