Neurological Sciences最新文献

{"title":"MR findings of intravascular large B-cell lymphoma of the central nervous system: report of three cases.","authors":"Lingxu Chen, Xiaochen Wang, Sihui Wang, Shengjun Sun","doi":"10.1007/s10072-025-08099-6","DOIUrl":"10.1007/s10072-025-08099-6","url":null,"abstract":"<p><strong>Background: </strong>Intravascular large B-cell lymphoma (IVLBCL) of the central nervous system (CNS) is a rare subtype of diffuse large B-cell lymphoma. Diagnosing CNS-limited IVLBCL is particularly challenging due to its non-specific clinical presentation and laboratory findings. We present three cases of CNS-limited IVLBCL, expanding the understanding of its imaging spectrum and enhancing preoperative diagnostic accuracy.</p><p><strong>Case report: </strong>Case 1 involved a 58-year-old male presenting with mild neurological symptoms, initially misdiagnosed as cerebrovascular disease. MRI showed patchy areas of diffusion restriction adjacent to the posterior horn of the left lateral ventricle, progressing over four months to a patchy hemorrhagic lesion in the right temporoparietal region with leptomeningeal enhancement. Case 2 described a 53-year-old male with progressive limb weakness and cognitive decline, initially managed as a demyelinating disease. MRI demonstrated extensive white matter hyperintensities and multiple areas of diffusion restriction. Case 3 involved a 67-year-old female with recurrent cognitive decline and gait disturbance. MRI findings included patchy T2 hyperintensities in the white matter, gyrus-like hyperintensity on T1WI, and punctate and linear enhancement. Cases 1 and 2 were diagnosed as IVLBCL post-biopsy, while in Case 3, IVLBCL was considered based on imaging features and differentiated from CNS vasculitis.</p><p><strong>Conclusion: </strong>These cases highlight the variable and often non-specific MRI characteristics of CNS-IVLBCL, including infarct-like lesions, persistent diffusion restriction, and SWI abnormalities. Early recognition of these imaging findings can aid in timely diagnosis and treatment, potentially improving patient outcomes.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"3959-3963"},"PeriodicalIF":2.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144021405","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":"Enhancing postural control in stroke patients: advances in mechanisms and functional recovery analysis of core stability training.","authors":"Tingyu Zhang, Jiejiao Zheng","doi":"10.1007/s10072-025-08119-5","DOIUrl":"10.1007/s10072-025-08119-5","url":null,"abstract":"<p><p>Stroke remains the primary cause of mortality and morbidity in the adult population in China. Postural control dysfunction is a significant and persistent issue commonly observed in stroke patients. Core stability training has been shown to improve postural control in stroke patients, but the accuracy and efficacy of subjective scales used to assess the quality of resulting improvements remain uncertain. The first part of this manuscript reviews the origins and development of core stability training. The second part provides a brief examination of the mechanism by which core stability training affects postural control in post-stroke individuals. The third part reviews the functional recovery outcomes of core stability training as assessed through instrumental gait analysis, with gait spatio-temporal and kinematic parameters enhancing motor control, center of gravity trajectory and kinetic parameters enhancing postural stability, and electromyographic activity parameters enhancing neuromuscular recovery of core muscle groups.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"3457-3467"},"PeriodicalIF":2.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267367/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143796027","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Systematic review of pharmacological treatment options for orthostatic tremor in prospective patient cohorts and randomized controlled trials.","authors":"Lena Sauthoff, Maider Iza Achutegui, Lan Ye, Clara Niesmann, Florian Wegner, Matthias Höllerhage, Martin Klietz","doi":"10.1007/s10072-025-08129-3","DOIUrl":"10.1007/s10072-025-08129-3","url":null,"abstract":"<p><strong>Introduction: </strong>Orthostatic tremor is an infrequent movement disorder characterized by a high-frequency tremor manifesting primarily in the standing position. This condition can lead to relevant restrictions of mobility in everyday life and adversely affect the quality of life. The etiology has not been conclusively clarified. To date, there are few therapy studies of sufficient quality.</p><p><strong>Aim: </strong>The aim of the present literature analysis is to systematically evaluate the existing evidence of pharmacological therapies for orthostatic tremor.</p><p><strong>Materials and methods: </strong>This study searched for publications via PubMed and Google Scholar using the terms \"orthostatic tremor\" AND \"therapy\" and \"shaky legs syndrome\" AND \"therapy\". The main inclusion criteria were a subject number ≥ 5, pharmacological treatment approaches and the presence of a prospective experimental design.</p><p><strong>Results: </strong>The evaluation of the results indicated the most positive evidence for therapy with gabapentin. Additionally, other drugs such as perampanel and levodopa also showed positive outcomes regarding specific endpoints. In contrast, there is a lack of evidence supporting the efficacy of levetiracetam and botulinum toxin in the context of primary orthostatic tremor.</p><p><strong>Discussion and conclusion: </strong>According to current evidence, gabapentin is the drug with the most robust data. However, further studies are needed to support the evidence for different pharmacological therapeutic approaches for orthostatic tremor. Future investigations should emphasize larger sample sizes, placebo-controlled, double-blinded methodologies and a longer follow-up to be able to make more precise recommendations with greater generalizability.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"3419-3425"},"PeriodicalIF":2.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267365/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143743520","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Role of anxiety in exercise intolerance and autonomic nervous system dysfunction post-concussion.","authors":"Aaron J Zynda, Alicia M Trbovich, Faith Kehinde, Christopher Burley, Michael W Collins, David O Okonkwo, Anne Mucha, Sarah Ostop, Cyndi Holland, Courtney Perry, Melissa N Womble, Sabrina Jennings, Sheri Fedor, Christina Dollar, Kori J Durfee, R J Elbin, Anthony P Kontos","doi":"10.1007/s10072-025-08204-9","DOIUrl":"10.1007/s10072-025-08204-9","url":null,"abstract":"<p><strong>Objective: </strong>To compare autonomic nervous system (ANS) response using the Buffalo Concussion Bike Test (BCBT) between adult post-concussion patients with and without pre-existing and post-injury anxiety.</p><p><strong>Design: </strong>Cross-sectional cohort study of participants from the Targeted, Multidomain (T-MD) Treatment for Concussion Randomized Controlled Trial.</p><p><strong>Setting: </strong>Two concussion specialty clinics.</p><p><strong>Participants: </strong>Ages 18-49 years, 8 days to 6 months from injury, without a history of cardiovascular/neurological/vestibular disorders. 256 participants were eligible, 120 declined, 136 enrolled, and 30 were excluded.</p><p><strong>Exposures: </strong>Participants completed the BCBT at a baseline study visit following initial clinical evaluation.</p><p><strong>Main outcomes and measures: </strong>Number of BCBT stages completed and Visual Analogue Scale (VAS), heart rate (HR), blood pressure (BP), and Ratings of Perceived Exertion (RPE) pre- and post-BCBT.</p><p><strong>Results: </strong>106 participants (age = 25.5 [12.5], 66 [62.3%] female) were included, 44 (41.5%) with pre-existing anxiety, 61 (57.6%) without pre-existing anxiety, 33 (31.1%) with post-injury anxiety, and 73 (68.9%) without post-injury anxiety. Eighteen (17.0%) were in both anxiety groups. Results supported a significant group x time interaction in participants with pre-existing anxiety for increased diastolic BP (F[1,97] = 5.03, p = 0.03) and VAS (F[1,99] = 5.05, p = 0.03) while controlling for covariates. Participants with pre-existing anxiety (median = 8[4]) and post-injury anxiety (median = 8[5]) completed fewer BCBT stages than those without pre-existing (median = 11[6], p < 0.01) and post-injury (median = 10[6], p = 0.03) anxiety.</p><p><strong>Conclusions: </strong>Adults with pre-existing and post-injury anxiety experienced altered physiological responses to exercise post-concussion and completed fewer stages of the BCBT. Anxiety should be considered by clinicians when interpreting findings from exercise intolerance tests such as the BCBT.</p><p><strong>Trial registration: </strong>www.</p><p><strong>Clinicaltrials: </strong>gov ; identifier: NCT04549532; https://clinicaltrials.gov/study/NCT04549532.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"3909-3918"},"PeriodicalIF":2.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144030557","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":"Bilateral optic neuropathy as first neurological sign of Central Nervous System (CNS) involvement in indolent chronic lymphocytic leukemia.","authors":"Paola Ciasca, Sebastiano Crisafulli, Ludovica Gargiulo, Stefania Bianchi Marzoli, Massimo Magagnoli, Marco Moscatelli, Elena Anghileri","doi":"10.1007/s10072-025-08118-6","DOIUrl":"10.1007/s10072-025-08118-6","url":null,"abstract":"","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"4087-4090"},"PeriodicalIF":2.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267301/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143710841","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The role of neurology in the development of community healthcare within the Italian national health service. The position of the Italian society of neurology (SIN).","authors":"Pietro Ferrara, Vincenzo Andreone, Fabio Bandini, Massimo Del Sette, Marco Longoni, Roberto Marconi, Carlo Alberto Mariani, Francesca Romana Pezzella, Rocco Quatrale, Carla Zanferrari, Maria Luisa Zedde, Alessandro Padovani","doi":"10.1007/s10072-025-08237-0","DOIUrl":"10.1007/s10072-025-08237-0","url":null,"abstract":"<p><p>Neurological disorders rank within the leading causes of disability and premature death in Europe and Italy, and their prevalence is expected to rise due to the aging population. In Italy, the current approach to neurological care is primarily centered in hospitals. However, an effective community-based neurology care system would ensure that patients receive appropriate care in the right setting, from the right healthcare professional, and at the right time. This \"precision\" approach would also alleviate the burden on acute neurology facilities. To address the growing demand for neurological care services, experts from the Italian Society of Neurology (SIN) have launched a nationwide initiative aimed at supporting a transformative program in local health and care systems, enabling them to better meet the needs of neurological patients. This document presents a set of consensus recommendations for redesigning neurological care in Italy, with the objective of aligning services with the health requirements of neurological patients and establishing an integrated model for managing neurological disorders.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"3363-3375"},"PeriodicalIF":2.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267357/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144310196","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intrathecal treatment of Anti-N-Methyl-D-Aspartate receptor encephalitis: a promising approach for refractory cases.","authors":"Xiaobo Yang","doi":"10.1007/s10072-025-08134-6","DOIUrl":"10.1007/s10072-025-08134-6","url":null,"abstract":"<p><strong>Background: </strong>Anti-N-methyl-D-aspartate receptor encephalitis is a severe neurological disorder. While standard treatments work for many patients, up to 25% are resistant to these treatments. In such challenging cases, there is growing interest in using intrathecal approaches.</p><p><strong>Methods: </strong>A narrative review of relevant papers was conducted.</p><p><strong>Results: </strong>Case reports and case series have demonstrated the successful use of intrathecal methotrexate, either alone or in combination with steroids, particularly in pediatric patients. Additionally, intrathecal rituximab has shown promise in select cases.</p><p><strong>Conclusions: </strong>Although outcomes vary, this approach appears to be safe and has the potential to rescue refractory cases.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"3495-3504"},"PeriodicalIF":2.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143699593","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":"Short neck as a cause of stroke? Bilateral vertebral artery dissection in a patient with Klippel-Feil Syndrome.","authors":"Laura Del Pino Tejado, Marta Vales Montero, Ana María Iglesias Mohedano, Andrés García Pastor, Fernando Díaz Otero, Pilar Vázquez Alén, Yolanda Fernández Bullido, Antonio Gil Núñez","doi":"10.1007/s10072-025-08157-z","DOIUrl":"10.1007/s10072-025-08157-z","url":null,"abstract":"<p><p>Vertebral dissections are a recognized cause of stroke in young people. Its association with Klippel-Feil Syndrome (KFS) is unusual. We describe the case of a male with simultaneous bilateral vertebral artery dissection with SKF and review the related literature. A 30-year-old male, with no relevant medical history, was transferred to the hospital with code stroke due to dizziness, dysarthria and clumsiness of the right limbs of sudden onset. Eight days prior, he had experienced intense right-sided neck pain after making a sudden neck turn while playing football, which persisted over the days. Phenotypically, he had a noticeable short neck. A cranial CT scan showed an acute right cerebellar infarction and an AngioCT scan showed bilateral vertebral artery dissection. An MRI revealed vertebral artery wall signal hyperintensity, compatible with intramural hematoma. The findings were confirmed by arteriography. Fusion of the cervical vertebrae C2-C3 compatible with SKF was observed at the same level where both dissections were observed. The patient was discharged asymptomatic with antiplatelet treatment. He received single antiplatelet therapy with 100 mg aspirin, remaining stable without recurrences. A follow-up MR angiogram showed resolution of the lesions after 3 months. SKF is a rare malformation consisting of a triad of fusion of cervical vertebrae, short neck and low hairline implantation. Its association with stroke is scarce and there are few descriptions of cases, none with bilateral dissection.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"3993-3996"},"PeriodicalIF":2.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143780743","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":"Cerebral small vessel disease associated with COL4A1 and COL4A2 duplication: clinical and MRI features resembling CADASIL.","authors":"Lili Chen, Shuang Li, Fei Xie, Xingyue Hu, Wen Lv","doi":"10.1007/s10072-025-08175-x","DOIUrl":"10.1007/s10072-025-08175-x","url":null,"abstract":"<p><strong>Background: </strong>Large duplications or triplications involving the 13q33-34 chromosomal region, which encompass the COL4A1 and COL4A2 genes, have been reported in association with cerebral small vessel disease (CSVD) in a few patients. Herein, we report an additional case of CSVD linked to a duplication of COL4A1 and COL4A2 and provide a detailed summary of the associated clinical and MRI findings.</p><p><strong>Methods: </strong>A patient with CSVD underwent detailed clinical and neuroimaging evaluations. Targeted next-generation sequencing (NGS) and copy number variation sequencing (CNV-seq) based on whole genome sequencing were used to identify the genetic basis of the disease.</p><p><strong>Results: </strong>The patient experienced his first ischemic stroke at age 51. Cranial MRI revealed extensive acute and chronic lacunar infarcts and white matter hyperintensities across both cerebral hemispheres, with involvement of the anterior temporal lobe and the external capsule. Bilateral thalamic microbleeds were also noted. The clinical features and MRI findings are similar to those observed in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Targeted NGS and CNV-seq analysis identified a duplication in the region of chromosome 13, which included the COL4A1 and COL4A2 genes.</p><p><strong>Conclusions: </strong>This case provides further evidence supporting the association of CNVs in COL4A1 and COL4A2 with CSVD. When hereditary CSVD is suspected and no micro-mutations in CSVD-associated genes are identified, CNV analysis of the 13q region should be considered.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"3987-3991"},"PeriodicalIF":2.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144008508","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":"Clinical and imaging characteristics of corticosteroid-responsive chronic lymphocytic inflammation with Pontine perivascular enhancement syndrome (CLIPPERS): an analysis of 9 cases.","authors":"Xiaosa Sun, Xiaowen Li, Hafiz Khuram Raza, Jingwen Yang, Ying Ji, Xinyi Yuan, Yu Kong, Jin Tian, Yaqin Gu, Hao Chen, Li Chen","doi":"10.1007/s10072-025-08208-5","DOIUrl":"10.1007/s10072-025-08208-5","url":null,"abstract":"<p><strong>Purpose: </strong>This study aimed to elucidate the clinical and radiological profiles of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS), targeting an enhanced comprehension and more efficient diagnosis of this rare disorder among medical professionals.</p><p><strong>Methods: </strong>We conducted a retrospective cohort study, encompassing a detailed analysis of clinical and imaging data from nine CLIPPERS patients diagnosed between 2016 and 2023 at Xuzhou Medical University Affiliated Hospital and Beijing Xuanwu Hospital.</p><p><strong>Results: </strong>Our study included nine patients, comprising seven males and two females, with ages varying from 9 to 79 years.The clinical spectrum was broad, including lower limb weakness (5 cases), unsteady gait (6), lower limb numbness (2), visual impairments (4), dizziness and headaches (5), cognitive impairments (2), facial sensory abnormalities (2), language disturbances (4), nausea and vomiting (1), limb twitching (1), fever (1), and chest and abdominal discomfort (1).Brain MRI scans uniformly disclosed multifocal lesions with slightly prolonged T1 and pronounced T2 signal characteristics in the brainstem, notably in the pons, as well as in bilateral thalami, cerebellum, and basal ganglia. High signal intensity on FLAIR sequences and variable signal intensity on DWI sequences were observed, with post-contrast imaging exhibiting the pathognomonic punctate and curvilinear 'pepper-like' enhancement. Cervical spinal cord MRIs from two patients mirrored this enhancement pattern. Pathological examination via biopsy in two cases-one of brain tissue and one of spinal cord tissue-demonstrated perivascular infiltration of CD3 + T lymphocytes, accompanied by CD20+, CD5+, and CD68 + cell infiltrations. A favorable response to corticosteroid therapy was observed across all patients, with marked amelioration in clinical symptoms and radiological findings. Remarkably, one patient, diagnosed with classical Hodgkin's lymphoma two years post-onset, showed no signs of recurrence on subsequent PET-CT scans following standard chemotherapy. The remaining patients were either on steroid maintenance therapy or received a combination of steroids and immunosuppressants, with symptomatic improvement noted in all.</p><p><strong>Conclusions: </strong>CLIPPERS syndrome is distinguished by its characteristic MRI features and demonstrates a robust response to corticosteroid therapy, leading to rapid clinical and radiological improvement. In scenarios with ambiguous diagnosis, pathological tissue biopsy offers essential confirmation.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"3819-3826"},"PeriodicalIF":2.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144012667","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}