eNeurologicalSci最新文献

{"title":"Myasthenia gravis: The evolving therapeutic landscape","authors":"Gil I. Wolfe,&nbsp;Jonathan E. Hanson,&nbsp;Nicholas J. Silvestri","doi":"10.1016/j.ensci.2024.100541","DOIUrl":"10.1016/j.ensci.2024.100541","url":null,"abstract":"<div><div>Pharmacological options in the management of generalized myasthenia gravis (gMG) have expanded rapidly in the last 7 years. There are now several complement inhibitors and neonatal Fc receptor antagonists on the market in many countries for patient management, following the successful completion of Phase 3 studies. In open-label extensions, these agents have proven to be effective over the longer term extending several years, with benefits such as reduction of corticosteroid requirements being observed. In the communication below, we will briefly summarize recent pharmacologic advancements in the management of gMG and outline how these agents are currently being used and may be used in the future.</div></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100541"},"PeriodicalIF":0.0,"publicationDate":"2024-11-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142699415","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Thalamic deep brain stimulation for postural tremor caused by hyperthermia-induced cerebellar dysfunction: A case report","authors":"Mitsuyoshi Tamura ,&nbsp;Shigeki Hirano ,&nbsp;Yoshihisa Kitayama ,&nbsp;Marie Morooka ,&nbsp;Tomoki Suichi ,&nbsp;Kazumoto Shibuya ,&nbsp;Yoshinori Higuchi ,&nbsp;Satoshi Kuwabara","doi":"10.1016/j.ensci.2024.100536","DOIUrl":"10.1016/j.ensci.2024.100536","url":null,"abstract":"<div><h3>Background</h3><div>The efficacy of deep brain stimulation (DBS) in treating tremor symptoms in cerebellar disorders remains unclear.</div></div><div><h3>Case presentation</h3><div>A 47-year-old woman presented with neck and arm tremor and ataxic speech/gait after four days of &gt;40 °C fever due to septic shock attributed to lithiasis-pyelonephritis. Left ventral intermediate nucleus thalamus DBS alleviated contralateral postural arm tremor, although the action tremor and terminal oscillation remained unchanged.</div></div><div><h3>Discussion</h3><div>To our knowledge, this is the first report of thalamic DBS for hyperthermia-induced cerebellar dysfunction. Patients with postural tremor resulting from cerebellar damage can benefit from thalamic DBS, leading to improved activities of daily living.</div></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100536"},"PeriodicalIF":0.0,"publicationDate":"2024-11-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142699416","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neurobehavioral features in medication-overuse headache","authors":"Franz Riederer ,&nbsp;Roberto Pirrotta ,&nbsp;Chantal Martin Soelch ,&nbsp;Andreas R. Gantenbein ,&nbsp;Adrian Scutelnic ,&nbsp;Antonia Klein ,&nbsp;Christoph J. Schankin ,&nbsp;Peter S. Sándor","doi":"10.1016/j.ensci.2024.100538","DOIUrl":"10.1016/j.ensci.2024.100538","url":null,"abstract":"<div><h3>Background</h3><div>Medication-overuse headache (MOH) has been related to the spectrum of dependence behavior and impaired orbitofrontal cortex function. Alexithymia is a trait comprising deficits in identifying self-emotions and perception. It was the aim of the study to investigate impulsivity and alexithymia, in patients with MOH and perform correlations with cerebral grey matter.</div></div><div><h3>Material and methods</h3><div>Patients with chronic migraine and MOH according to ICHD criteria from a tertiary headache clinic and healthy controls were investigated by a single psychiatrist, using clinical scales for self-control (BIS-11) and alexithymia (TAS-20) and screened for dependence based on DSM-IV criteria. Correlations of BIS-11 and TAS-20 with cerebral grey matter were analysed with the SPM based toolbox CAT12, using high resolution T1weighted MRI-Sequences acquired on a 3 T scanner.</div></div><div><h3>Results</h3><div>MRI data were available from 30 MOH patients (24 women) and 47 healthy controls (26 women). MOH patients had increased impulsivity (62.2 ± 11.1 vs. 55.7 ± 7.2; <em>p</em> = 0.007) and alexithymia (49.8 ± 14.8 vs. 38.0 ± 6.5; <em>p</em> &lt; 0.001). Analyzing only women, the results remained significant. Ninety percent of patients fulfilled DSM-IV criteria for substance dependence. There was a positive correlation between impulsivity and grey matter in the left middle orbital gyrus in healthy controls but not in patients (<em>p</em> &lt; 0.05, corrected). No correlations with alexithymia and cerebral grey matter were found.</div></div><div><h3>Conclusions</h3><div>The present study suggests a neurobehavioral basis for MOH, consisting of impaired impulse control, and self-perception along with features of substance dependence. Although decreased orbitofrontal cortex volume was confirmed in this MOH cohort, impulsivity and alexithymia were not correlated with this structural abnormality.</div></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100538"},"PeriodicalIF":0.0,"publicationDate":"2024-11-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142699413","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"World neurology updates: Other primary headache disorder – Treatment","authors":"Sina Marzoughi ,&nbsp;Peter J. Goadsby","doi":"10.1016/j.ensci.2024.100535","DOIUrl":"10.1016/j.ensci.2024.100535","url":null,"abstract":"","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100535"},"PeriodicalIF":0.0,"publicationDate":"2024-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142699414","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"De novo myasthenia gravis in a patient with malignant melanoma after concurrent SARS-CoV-2 vaccination and immune checkpoint inhibitor therapy: Case report and literature review","authors":"Mohadese Shahin ,&nbsp;Pedram Fadavi ,&nbsp;Mohammad Mostafa Ansari Ramandi ,&nbsp;Soroush Shahrokh ,&nbsp;Farzad Taghizadeh-Hesary","doi":"10.1016/j.ensci.2024.100534","DOIUrl":"10.1016/j.ensci.2024.100534","url":null,"abstract":"<div><div>In recent years, the advent and increasingly common use of immune checkpoint inhibitors (ICIs) in cancer treatment have been notable. While ICIs have shown relatively better toxicity profiles compared to traditional chemotherapy agents, they are linked to a unique range of toxicities known as immune-related adverse events (irAEs), stemming from immune system dysregulation. Following the coronavirus disease 2019 (COVID-19) pandemic, cancer patients were universally categorized as the highest priority subgroup for vaccination against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), despite being excluded from vaccine trials. The exclusion of cancer patients from vaccine trials has raised concerns within the scientific community about the potential for a hyperactive autoimmune response, which could lead to severe irAEs in patients receiving concurrent ICIs and anti-SARS-CoV-2 vaccines. Retrospective studies have indicated subtle safety concerns for mRNA vaccines in cancer patients who have undergone ICI treatment, with none of these studies encompassing inactivated anti-SARS-CoV-2 vaccines. Here, we present a case of a patient with malignant melanoma who developed fatal myasthenia gravis (MG) following concurrent vaccination with Sinopharm's inactivated COVID-19 vaccine (BBIBP-CorV) and initiation of pembrolizumab. Additionally, we examine current research on the relationship between anti-SARS-CoV-2 vaccination and irAEs in patients treated with ICIs and propose a potential mechanism responsible for the fatal MG in our patient.</div></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100534"},"PeriodicalIF":0.0,"publicationDate":"2024-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142657485","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of physical activity on quality of life in patients with epilepsy in a developing country: A cross-sectional, survey-based study","authors":"Daniel San-Juan ,&nbsp;Miguel Benjamín Cervera-Sánchez ,&nbsp;Miguel Angel Morales-Morales ,&nbsp;Emilio Israel Wong-Valenzuela ,&nbsp;Dara Lizeth Torres-Rodríguez ,&nbsp;Daniela Carolina Pimentel-Saona","doi":"10.1016/j.ensci.2024.100533","DOIUrl":"10.1016/j.ensci.2024.100533","url":null,"abstract":"<div><h3>Rationale</h3><div>Patients with epilepsy face different barriers based on ignorance and fear of themselves and others while exercising, this situation complicates attempts at exercise and promotes discrimination against patients. Physical activity has been associated with favorable results in the health of this population and can be used as a non-pharmacological treatment seeking to increase the health and improve living conditions of patients. Our study aims to explore how physical activity impacts quality-of-life perception in these patients.</div></div><div><h3>Methods</h3><div>We performed a cross-sectional, survey-based study conducted at the outpatient Epilepsy Clinic in the National Institute of Neurology and Neurosurgery “Manuel Velasco Suárez”. Patients between 17 and 65 years of age and with an established diagnosis of epilepsy were considered for participation. A 31-item questionnaire was administered to all participants face-to-face or by telephone. The questions included in the survey were selected from the Global Physical Activity Questionnaire and the Quality of Life in Epilepsy Inventory to assess the level of exercise and perceived quality of life of participants. Descriptive and inferential Mann-Whitney <em>U</em> test statistical analysis was done using IBM SPSS Statistics 23.0 software.</div></div><div><h3>Results</h3><div>Participants' mean age was 35.69 ± 11.93 years, 45 % were male and 55 % female. Significant differences were found in the domain of perceived quality of life in the self-reported physically active participants (<em>p</em> = 0.0256; 95 % IC: 1.00–15.22), with a mean score of 75.78 ± 16.64 in the active group vs. 67.66 ± 15.86 in the sedentary group. In those who complied with the exercise recommendations according to the WHO, there was significant evidence of improvement in the general quality of life (<em>p</em> = 0.0032; 95 % IC:4.9–23.9), with a mean score of 75.22 ± 15.92 in the active group vs. 60.76 ± 17.836 in the sedentary group.</div></div><div><h3>Conclusions</h3><div>Physical activity is associated with a significant impact on how patients with epilepsy perceive their quality of life. In addition, it improves memory and concentration in the tasks they perform in their daily lives.</div></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100533"},"PeriodicalIF":0.0,"publicationDate":"2024-11-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142657738","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"IIH, SIH and headache: Diagnosis and treatment update","authors":"Shuu-Jiun Wang","doi":"10.1016/j.ensci.2024.100532","DOIUrl":"10.1016/j.ensci.2024.100532","url":null,"abstract":"<div><div>Idiopathic intracranial hypertension (IIH) and spontaneous intracranial hypotension (SIH) are two major secondary headache disorders resulting from abnormal intracranial pressure (ICP). This short communication outlines the pathophysiology, diagnostic criteria, and management strategies for IIH and SIH. IIH predominantly affects obese women of childbearing age and presents with daily headaches, visual disturbances, and papilledema. It is characterized by elevated cerebrospinal fluid (CSF) pressure, with diagnosis supported by imaging and lumbar puncture. Treatment includes weight reduction, medications, and surgical interventions in refractory cases. SIH, conversely, is caused by spontaneous spinal CSF leaks and presents with acute orthostatic headaches. Diagnosis is supported by neuroimaging and lumbar puncture, revealing low CSF pressure. Treatment includes supported care, (targeted) epidural blood patch, with surgical repair considered in refractory cases. Advances in imaging and treatment have significantly improved outcomes for both conditions.</div></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100532"},"PeriodicalIF":0.0,"publicationDate":"2024-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142538905","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Double trouble: The intersection of neurobrucellosis and systemic lupus erythematosus in a 24-year-old female","authors":"Hashim Talib Hashim ,&nbsp;Aws Murad Frhood ,&nbsp;Ahmed Abdulhussain Shahatta ,&nbsp;Ahmed Dheyaa Al-Obaidi ,&nbsp;Arshed Shakir Kadim","doi":"10.1016/j.ensci.2024.100531","DOIUrl":"10.1016/j.ensci.2024.100531","url":null,"abstract":"<div><h3>Background</h3><div>Systemic lupus erythematosus (SLE) is a chronic autoimmune disease affecting multiple organs, while brucellosis is a zoonotic infection prevalent in endemic areas. Neurobrucellosis, a severe complication of brucellosis, can mimic or coexist with autoimmune conditions like SLE, complicating diagnosis and treatment. This case report highlights the diagnostic challenges and management strategies for such overlapping diseases.</div></div><div><h3>Case presentation</h3><div>In this case report, we present a 24-year-old female who initially presented with symptoms that were misleadingly attributed to a simple vaginal ulcer. Her clinical course evolved to include migratory polyarthralgia, nocturnal fevers, and significant weight loss, eventually culminating in severe headaches and dizziness that led to a diagnosis of meningitis. Comprehensive diagnostic work-up, including the presence of a malar rash, positive anti-dsDNA antibodies, and elevated inflammatory markers, pointed towards SLE. However, the identification of bacterial infection markers in the cerebrospinal fluid (CSF) and her history of animal contact in an endemic area led to the diagnosis of neurobrucellosis. The patient's remarkable response to doxycycline and rifampicin confirmed the infectious etiology, highlighting the complex interplay between these two diseases.</div></div><div><h3>Conclusion</h3><div>The intersection of SLE and neurobrucellosis in this patient underscores the importance of a multidisciplinary approach to diagnosis and treatment, ensuring that both the autoimmune and infectious aspects of the patient's condition are adequately addressed. This case contributes valuable insights into the management of such rare and complex presentations, emphasizing the need for vigilance and adaptability in clinical practice.</div></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100531"},"PeriodicalIF":0.0,"publicationDate":"2024-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142421598","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Levodopa / opicapone as a complement to STN-DBS in clinical practice. A retrospective single-centre analysis.","authors":"Moritz A. Loeffler ,&nbsp;Philipp Klocke ,&nbsp;Idil Cebi ,&nbsp;Alireza Gharabaghi ,&nbsp;Daniel Weiss","doi":"10.1016/j.ensci.2024.100530","DOIUrl":"10.1016/j.ensci.2024.100530","url":null,"abstract":"<div><h3>Objective</h3><div>Deep brain stimulation of the subthalamic nucleus (STN-DBS) is a well-established treatment option in Parkinson's disease with motor and non-motor fluctuations allowing for postoperative reduction of dopaminergic medication. However, evidence is scarce on optimal medication adjustments following STN-DBS implantation. Opicapone allows for long-lasting inhibition of the catechol-<em>O</em>-methyltransferase (COMT) thereby enabling more constant dopaminergic stimulation compared to levodopa alone. However, especially COMT inhibitors are regularly discontinued after STN-DBS surgery. In this single-centre retrospective analysis, we aimed to analyse the clinical phenotype of patients selected for opicapone treatment following STN-DBS implantation and to define clinical determinants of patients requiring more intense dopamine-stabilising strategies after STN-DBS implantation.</div></div><div><h3>Methods</h3><div>A patient cohort treated with STN-DBS + levodopa + opicapone (<em>n</em> = 16) was compared to an age-matched control cohort without opicapone treatment at baseline before and ≥ 5 months post-surgery. As main outcomes we assessed the MDS-UPDRS III and IV scores and reduction of the cumulative dopaminergic medication quantified by the levodopa equivalent dosages (LED).</div></div><div><h3>Results</h3><div>Whilst the MDS-UPDRS III (median [min – max]) in patients with STN-DBS as well as anatomical electrode positions did not differ significantly between the opicapone 20 [4–40] and control cohort 14 [1–44], the patients selected for opicapone treatment showed a significantly higher degree of dyskinesias already preoperatively as reflected by a UPDRS-IV A subscore of 2 [0–4] compared to controls 0 [0–4]. Postoperatively, the opicapone cohort showed stronger motor fluctuations MDS-UPDRS IV 6 [0–14] compared to the controls 0 [0−10], albeit without statistical significance. Moreover, the opicapone cohort showed significantly less reduction of dopaminergic medication (−36.4 % vs. -46.2 % in the control cohort) following STN-DBS implantation independent from the intake of dopamine agonists.</div></div><div><h3>Conclusion</h3><div>These results indicate a clinical phenotype characterised by more motor fluctuations requiring a more stable dopamine replacement therapy to address the patients' disease biology. In these cases, levodopa + COMT inhibition by opicapone represents a therapeutic approach but determination of the potential clinical benefit requires further prospective studies.</div></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100530"},"PeriodicalIF":0.0,"publicationDate":"2024-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142421599","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Internal carotid artery dissection in a patient with Parkinson's disease after COVID-19 infection","authors":"Takanobu Okubo ,&nbsp;Hidehiro Ishikawa ,&nbsp;Keita Matsuura ,&nbsp;Asako Tamura ,&nbsp;Koichi Miyashita ,&nbsp;Maki Umino ,&nbsp;Masayuki Maeda ,&nbsp;Akihiro Shindo","doi":"10.1016/j.ensci.2024.100529","DOIUrl":"10.1016/j.ensci.2024.100529","url":null,"abstract":"<div><h3>Background</h3><div>Internal carotid artery (ICA) dissection is a relatively rare cause of acute ischemic stroke. Stretching and compression of ICA due to sudden acceleration, deceleration, and rotational forces are risk factors for ICA dissection. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is believed to trigger an inflammatory response that exacerbates endothelial dysfunction and leads to arterial dissection. Although levodopa-induced cervical dyskinesia in Parkinson's disease often manifests as choreiform movement, dissection has not been reported in such patients.</div></div><div><h3>Case presentation</h3><div>A 51-year-old man with Parkinson's disease (PD) presented with gradually worsening neck pain and transient aphasia 1 week after mild coronavirus disease 2019 (COVID-19) infection. The patient already had neck pain due to cervical spondylosis and presented with levodopa-induced cervical dyskinesia. Magnetic resonance imaging revealed acute ischemic stroke in the left parietal lobe and an intramural hematoma with an area of stenosis in the left ICA. The patient was diagnosed with left ICA dissection.</div></div><div><h3>Conclusions</h3><div>COVID-19 infection can cause vessel wall vulnerability. Although patients with PD often have neck pain, ICA dissection should be considered a differential diagnosis if the patient has a recent history of COVID-19.</div></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100529"},"PeriodicalIF":0.0,"publicationDate":"2024-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142421597","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}