eNeurologicalSci最新文献

{"title":"World neurology updates: Other primary headache disorder – Treatment","authors":"Sina Marzoughi , 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":"2024-12-01","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":"2024-12-01","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":"Dynamic aphasia as an early sign of corticobasal degeneration: Clinico-radio-pathological correlation","authors":"Masanori Kurihara ,&nbsp;Akira Arakawa ,&nbsp;Aya Midori Tokumaru ,&nbsp;Tomoyasu Matsubara ,&nbsp;Hiroto Eguchi ,&nbsp;Yasushi Shimo ,&nbsp;Masato Hasegawa ,&nbsp;Kazutomi Kanemaru ,&nbsp;Katsuhiko Takeda ,&nbsp;Atsushi Iwata ,&nbsp;Shigeo Murayama ,&nbsp;Yuko Saito","doi":"10.1016/j.ensci.2024.100526","DOIUrl":"10.1016/j.ensci.2024.100526","url":null,"abstract":"<div><p>A 72-year-old man presented with a 6-month history of decreased voluntary speech. Sparse speech and decreased word fluency were observed. Articulation, naming, comprehension, and repetition were preserved. Agrammatism and paraphasia were not observed. These characteristics matched those reported as dynamic aphasia. Other findings were mild behavioral symptoms, recent memory impairment, and right hemiparkinsonism. The patient‘s voluntary speech continued to reduce and behavioral symptoms progressed. Brain MRI including voxel-based morphometric analysis showed left-dominant white matter volume reduction in the frontal lobe including those between the left supplementary motor area (SMA)/preSMA and the frontal operculum, likely involving the frontal aslant tract (FAT). The patient became completely mute after two years from disease onset and died of aspiration pneumonia. The neuropathological diagnosis was corticobasal degeneration (CBD). This case suggests that dynamic aphasia may be the initial sign of CBD and that early involvement of left FAT may be responsible for this feature.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100526"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405650224000339/pdfft?md5=470e69884011f9416ef222cc17438085&pid=1-s2.0-S2405650224000339-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142164087","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":"2024-12-01","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":"Evaluation of the gold cost criteria as a diagnostic criteria of amyotrophic lateral sclerosis","authors":"Ramez M. Odat ,&nbsp;Omar Alomari ,&nbsp;Anas Elgenidy","doi":"10.1016/j.ensci.2024.100524","DOIUrl":"10.1016/j.ensci.2024.100524","url":null,"abstract":"","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100524"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405650224000315/pdfft?md5=992b5fb83de8f413b3b56767edef22bb&pid=1-s2.0-S2405650224000315-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142150507","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":"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":"2024-12-01","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":"Is endocrine surveillance important in the care of Duchenne Muscular Dystrophy? Results from a national survey to patients and families on endocrine complications","authors":"Despoina Galetaki ,&nbsp;Vivian Szymczuk ,&nbsp;Melody Shi ,&nbsp;Nadia Merchant","doi":"10.1016/j.ensci.2024.100513","DOIUrl":"10.1016/j.ensci.2024.100513","url":null,"abstract":"<div><p>Glucocorticoids are standard of care for patients with Duchenne muscular dystrophy (DMD). Although prolonged exposure is associated with multiple endocrine side effects, current guidelines related to monitoring and management of endocrinopathies are suboptimal. We aim to explore community perceptions of endocrine related complications in patients with DMD, assess current level of understanding, and desire for further education. A 31-item online survey was sent through Parent Project to Muscular Dystrophy (PPMD) to Duchenne Registry members to be completed by patients or their caretakers. Response rate was 55% (<em>n</em> = 75). Steroids were taken by 93%, but only 50% were followed by endocrinology and 21% report never been seen by endocrinology. Bone health was discussed with 87% of patients and 60% were diagnosed with osteoporosis. Delayed puberty was discussed with 41% of patients with 23% receiving testosterone therapy. About half the patients reported a diagnosis of slowed growth. Only 51% of the participants recalled discussing adrenal insufficiency. Obesity was discussed with 59% of participants. Families felt education about steroid-induced endocrinopathies to be very or extremely important and prefer to discuss about this at the beginning of their steroid therapy. This demonstrates significant gaps in education and access to endocrine care in patients with DMD.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"36 ","pages":"Article 100513"},"PeriodicalIF":0.0,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405650224000200/pdfft?md5=69cc0a0ba5ae430c232f8a19911ec762&pid=1-s2.0-S2405650224000200-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141392362","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":"Epileptic seizures revealing tuberous sclerosis in a tropical environment: A study of 12 case series","authors":"Souleymane M'Bara Diallo ,&nbsp;Mamadou Diallo ,&nbsp;I.S. Barry ,&nbsp;M.L. Touré ,&nbsp;M.C. Barry ,&nbsp;M.T. Diallo ,&nbsp;S.D. Barry ,&nbsp;S.Y. Aminou ,&nbsp;G. Carlos Othon ,&nbsp;B. Diallo ,&nbsp;N. Camara ,&nbsp;M.B. Diallo ,&nbsp;M. Zoumanigui ,&nbsp;E. Lamah ,&nbsp;M. Hinima ,&nbsp;Sindu Mukesh ,&nbsp;A.K.T. Barry ,&nbsp;A. Sacko ,&nbsp;Ramit Singla ,&nbsp;F.A. Cissé ,&nbsp;A. Cissé","doi":"10.1016/j.ensci.2024.100516","DOIUrl":"10.1016/j.ensci.2024.100516","url":null,"abstract":"<div><h3>Background</h3><p>Epilepsy remains a significant public health concern in Sub-Saharan Africa (SSA) where diverse etiological factors contribute to its prevalence. Among these factors are conditions originating from the neuroectoderm, such as tuberous sclerosis. Insufficient medical attention and a lack of comprehensive multidisciplinary care contribute to its under-recognition.</p></div><div><h3>Materials and methods</h3><p>We conducted a retrospective descriptive study, involving 12 patients admitted to the neurology and pediatric departments of the University Hospital Ignace Deen between 2010 and 2022 due to recurring epileptic seizures. Subsequently, these patients were diagnosed with Tuberous sclerosis using the Schwartz 2007 criteria. The aim of this study is to reassess this condition from a clinical and paraclinical point of view in a tropical environment.</p></div><div><h3>Results</h3><p>Tuberous sclerosis, also known as Bourneville disease, was diagnosed in 12 patients exhibiting focal motor seizures and complex focal seizures likely associated with cortical and subcortical tubers detectable by EEG and neuroimaging, including CT and MRI. Delayed treatment resulted in varying degrees of mental decline. Additionally, some patients displayed cardiac hamartomas and intracranial posterior and anterior aneurysms as minor diagnostic indicators.</p></div><div><h3>Conclusion</h3><p>The study reveals a consistent clinical presentation accompanied by deteriorating neurological and psychological symptoms attributed to delayed multidisciplinary management. These findings are utilized to assess therapeutic strategies and prognostic outcomes.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"36 ","pages":"Article 100516"},"PeriodicalIF":0.0,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405650224000236/pdfft?md5=229167222f435c158fcd55ae95af9ddc&pid=1-s2.0-S2405650224000236-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141850067","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":"Diagnostic uncertainty of steroid-modified Marburg's variant of multiple sclerosis even at autopsy: A case suggesting lymphoma and related myelin loss","authors":"Akira Hanazono ,&nbsp;Keita Yasuda ,&nbsp;Hinako Shimada ,&nbsp;Yoshiko Takahashi ,&nbsp;Homare Funasaka ,&nbsp;Yui Sanpei ,&nbsp;Masashiro Sugawara","doi":"10.1016/j.ensci.2024.100515","DOIUrl":"https://doi.org/10.1016/j.ensci.2024.100515","url":null,"abstract":"<div><p>MS (multiple sclerosis) has specific criteria to avoid misdiagnosis. However, the Marburg variant of MS is so fulminant that initial axonal damage and other atypical observations have been allowed in past reports. We present a 74-year-old autopsy case with a vanishing tumor after steroids and radiation therapy, which was pathologically diagnosed as a Marburg variant with initial axonal loss. The case displayed radiological lymphoma-like observations: mass effects protruding to the lateral ventricle, fused extension from the choroid plexus to white matter with C opening sign, a growing lesion from the skull dura mater, high in diffusion-weighted imaging and low in apparent diffusion coefficient on magnetic resonance imaging (MRI) suggesting high cell density lymphoma. In addition, clinical manifestations were atypical for MS: upper limb monoplegia without ipsilateral lower limb involvement, pleocytosis over 50 cells/μL, and class 3 cytological abnormality in cerebrospinal fluid. However, at autopsy following steroids and radiation therapy, there were no lymphoma-like lesions, such as mass effects, fused extensive lesions, masses on the skull dura mater, or high cell density lesions. Instead, there were only myelin losses corresponding to the MRI lesions, highlighting the potential for contamination by other diseases in steroid-modified Marburg's variant of multiple sclerosis, possibly due to lymphoma, even at autopsy.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"36 ","pages":"Article 100515"},"PeriodicalIF":0.0,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405650224000224/pdfft?md5=721f48bbb51000432f4a77e4ef2ec11e&pid=1-s2.0-S2405650224000224-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141606621","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":"Effects of vaccination on COVID-19 infection symptoms in multiple sclerosis patients","authors":"Parisa Sharifi ,&nbsp;Nasim Rezaeimanesh ,&nbsp;Amir Moradi ,&nbsp;Abdorreza Naser Moghadasi","doi":"10.1016/j.ensci.2024.100511","DOIUrl":"10.1016/j.ensci.2024.100511","url":null,"abstract":"<div><h3>Background</h3><p>Patients with multiple sclerosis (MS) are at higher risk of having infections due to receiving disease modifying therapies. The current study was conducted among Iranian MS patients who had experienced at least one episode of COVID-19 infection in order to evaluate the effects of COVID-19 vaccination on symptoms of their infection. Data on demographic information, MS characteristics, COVID-19 infection details, and vaccination status were collected. Statistical analyses, were performed to evaluate the association between vaccination and symptoms of COVID-19 infection.</p></div><div><h3>Methods</h3><p>This cross-sectional study was conducted on confirmed MS patients. Demographic data and COVID-19 related symptoms were gathered via an online questionnaire. Confirmation of patients' who declared to be vaccinated was checked by their COVID-19 vaccination card.</p></div><div><h3>Results</h3><p>A total of 236 MS patients participated in the study. The majority were female (79.7%), with a mean age of 36.1 ± 7.9 years. Among the participants, 72.5% had received the COVID-19 vaccine before their first episode of COVID-19 infection. The analysis showed a significant difference in the incidence of respiratory symptoms (<em>P</em>-value: 0.01) and headache (P-value: 0.04) between vaccinated and non-vaccinated individuals. Logistic regression analysis revealed that vaccinated MS patients had lower odds of developing respiratory symptoms (OR:0.29, 95% CI: 0.16 to 0.53, <em>P</em>-value&lt;0.001) or headache (OR: 0.50, 95% CI: 0.25 to 0.98, P-value: 0.04) during their next COVID-19 infection episode. Moreover, MS patients who were receiving immunosuppressive drugs were less likely to have respiratory symptoms (OR:0.35, 95% CI: 0.16 to 0.77, <em>P</em>-value:0.009) but not headache (OR: 0.69, 95% CI: 0.30 to 1.60, P-value: 0.39).</p></div><div><h3>Conclusion</h3><p>COVID-19 vaccination can reduce the incidence of respiratory symptoms and headaches in MS patients during COVID-19 infection episodes. Additionally, patients who are receiving immunosuppressive drugs may benefit from COVID-19 vaccination.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"36 ","pages":"Article 100511"},"PeriodicalIF":0.0,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405650224000182/pdfft?md5=10e489228c32598c74a318434d7b8e4c&pid=1-s2.0-S2405650224000182-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141414830","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}