Neurological Sciences最新文献

{"title":"Lipid metabolism in moyamoya disease: Emerging evidence, vascular remodeling, and therapeutic implications.","authors":"Xinglin Chen, Chutong Guo, Meiqi Huang, Yuting Luo, Shaoqing Wu, Xunsha Sun","doi":"10.1007/s10072-026-09419-0","DOIUrl":"10.1007/s10072-026-09419-0","url":null,"abstract":"<p><strong>Background: </strong>Moyamoya disease (MMD), historically termed spontaneous occlusion of the circle of Willis, is a rare steno-occlusive cerebrovascular disease. Its etiology and pathogenesis remain incompletely understood, and lipid metabolism has recently attracted attention as a potential correlate of its clinical and biological heterogeneity.</p><p><strong>Methods: </strong>This scoping review summarizes evidence on lipid metabolic abnormalities in MMD, including clinical associations, lipid-related markers, multi-omics findings, potential mechanisms, and therapeutic implications of lipid-modulating approaches.</p><p><strong>Results: </strong>Epidemiological studies suggest that lipid abnormalities, including hypertriglyceridemia, low high-density lipoprotein cholesterol, and elevated lipoprotein(a), are common in MMD and are associated with disease phenotype, progression, stroke risk, and postoperative outcomes. Multi-omics studies reveal lipid metabolic alterations across peripheral blood, cerebrospinal fluid (CSF), and cerebrovascular tissue, suggesting a pattern of peripheral depletion, central enrichment, and tissue remodeling. Alterations in apolipoproteins such as APOE and lipid classes including sphingolipids, cardiolipins, and lysophosphatidylcholine may be associated with endothelial and vascular smooth muscle cell dysfunction, inflammatory responses, oxidative stress, and pathological vascular remodeling. Clinical studies suggest that statin use may be associated with improved collateral formation, although the underlying mechanisms remain uncertain.</p><p><strong>Conclusions: </strong>This review maps emerging evidence linking lipid metabolism to MMD and highlights future directions for biomarker discovery, mechanistic validation, and therapeutic exploration.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":"47 10","pages":""},"PeriodicalIF":2.7,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149070031","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":"Routine versus clinically indicated cerebrospinal fluid sampling for infection in external ventricular drains: a systematic review.","authors":"Helbert de Oliveira Manduca Palmiero, Eberval Gadelha Figueiredo","doi":"10.1007/s10072-026-09432-3","DOIUrl":"10.1007/s10072-026-09432-3","url":null,"abstract":"<p><strong>Background: </strong>External ventricular drains (EVDs) are commonly used in neurosurgery but carry a risk of ventriculostomy-related infection. Routine cerebrospinal fluid (CSF) sampling is commonly performed for surveillance, although its clinical value and potential risks are still uncertain.</p><p><strong>Methods: </strong>A systematic review was performed following PRISMA 2020 guidelines. PubMed was searched from inception to identify studies assessing CSF sampling strategies and/or clinical and laboratory screening for infection in patients with EVDs. Eligible studies included retrospective and prospective cohorts, case-control, and case series reporting infection-related outcomes. Data were extracted and categorized by causal relevance. Due to heterogeneity in studies, findings were summarized descriptively.</p><p><strong>Results: </strong>Fourteen studies were included, covering various study designs and sampling methods. Only 2 studies directly compared routine versus clinically indicated CSF sampling and found no benefit of routine daily sampling for detecting or reducing infections. Most studies were noncomparative and demonstrated the limited predictive value of routine clinical and laboratory parameters. Indirect evidence suggested that catheter duration, system manipulation, and patient-related factors were more strongly associated with infection risk than sampling frequency. Infection rates across studies ranged from 4.1% to 16.3%, with considerable variation in definitions and methodologies.</p><p><strong>Conclusion: </strong>Routine CSF sampling is not supported by high-quality evidence as a strategy to improve detection or prevent EVD-related infections. A selective, clinically guided approach may be more appropriate, with emphasis on reducing catheter manipulation and optimizing overall catheter management. Further prospective studies with standardized protocols are necessary to clarify the role of CSF sampling strategies.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":"47 10","pages":""},"PeriodicalIF":2.7,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13615090/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149063242","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":"Beyond β-amyloid: transthyretin cerebral amyloid angiopathy.","authors":"Carla Vera-Cáceres, Carolina Montecino Romanini, Montserrat González Platas, Irene Sánchez-Miranda Román, Alberto López Traba, Yeilen Aguilera, Abel Díaz Díaz, Azuquahe Pérez Hernández, Beatríz Báez Acosta, Daysi Chi","doi":"10.1007/s10072-026-09398-2","DOIUrl":"10.1007/s10072-026-09398-2","url":null,"abstract":"<p><p>Cerebral amyloid angiopathy (CAA) is most commonly associated with β-amyloid deposition, but other amyloid proteins may accumulate in leptomeningeal and superficial cortical vessels. We report a man first evaluated neurologically at 44 years of age after approximately one year of recurrent upper-limb and perioral sensory symptoms with speech disturbance, retrospectively considered transient focal neurological episodes (TFNEs). During follow-up, he developed bilateral vitritis and progressive behavioral, psychiatric, and cognitive impairment. Brain computed tomography showed diffuse linear and serpiginous leptomeningeal hyperdensities, consistent with calcified amyloid deposits with focal cortical subarachnoid hemorrhagic lesions SAH); angiography showed no underlying vascular abnormality. Follow-up neuroimaging demonstrated persistent leptomeningeal abnormalities and hemorrhagic lesions. Leptomeningeal biopsy revealed Congo red- and thioflavin S-positive vascular and meningeal amyloid deposits, with negative β-amyloid and amyloid A immunohistochemistry. A subcutaneous adipose tissue biopsy supported systemic amyloid deposition, and genetic testing identified the heterozygous TTR c.265T > C, p.(Tyr89His) variant. Electroneurography showed no large-fiber peripheral neuropathy. An off-label trial of tolcapone produced no clinical benefit. The patient subsequently developed severe cognitive and functional disability and progressive hydrocephalus, and died at 55 years of age from acute respiratory failure. This case highlights TTR-related cerebral amyloid angiopathy (TTR-CAA) as an important non-β-amyloid cause of TFNEs, calcified leptomeningeal infiltration, focal superficial hemorrhage, and progressive neuropsychiatric decline.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":"47 10","pages":""},"PeriodicalIF":2.7,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13616015/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149063143","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":"Acquired paroxysmal dysarthria-ataxia syndrome: A systematic review of clinical and MRI findings.","authors":"Krzysztof Smolik, Pedro Fraiman, João Claudio Urbano, Rodrigo Alencar, Maria Chiara Bassi, Annalisa Gessani, Sara Montepietra, Manuela Napoli, Francesco Cavallieri, Franco Valzania, Elena Moro, Clecio Godeiro Junior","doi":"10.1007/s10072-026-09413-6","DOIUrl":"10.1007/s10072-026-09413-6","url":null,"abstract":"<p><p>Paroxysmal dysarthria-ataxia syndrome (PDAS) is a rare neurological syndrome initially described in Multiple Sclerosis (MS) and subsequently reported in other conditions. This systematic review focuses on acquired forms of PDAS associated with structural or immune-mediated aetiologies. After screening 3802 articles, 25 studies (31 patients) were included. Twenty-two patients (71%) were male, and the mean age of PDAS onset was 52 years. The most frequent diagnosis was MS, observed in 12 patients (39%), followed by anti-contactin associated protein receptor 2 encephalitis in 6 cases (19%). Lesions were most commonly located in the midbrain, reported in 20 patients (65%). Both the duration and frequency of PDA episodes varied greatly. Outcomes were favorable, with complete resolution of symptoms after pharmacological treatment in 29 patients (93%). Treatment mainly consisted of antiepileptic drugs (AEDs), most commonly carbamazepine, used in 14 patients (45%). These findings show that PDAS is not exclusively associated with MS and that, when treated, its prognosis is generally very good.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":"47 10","pages":""},"PeriodicalIF":2.7,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149063092","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":"C9ORF72-associated bulbar-onset ALS in a patient with secondary progressive multiple sclerosis and conjugal ALS.","authors":"Kamran Moradi, Yaesshna Pillay, Christina J Azevedo, Leila Darki, Said R Beydoun","doi":"10.1007/s10072-026-09425-2","DOIUrl":"10.1007/s10072-026-09425-2","url":null,"abstract":"","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":"47 10","pages":""},"PeriodicalIF":2.7,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149063154","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":"Painless legs and moving toes phenotype in corticobasal syndrome with biomarker evidence of Alzheimer's disease: a case report.","authors":"Nobuko Kawakami, Takafumi Hasegawa, Kazuo Kakinuma, Chifumi Iseki, Shigenori Kanno","doi":"10.1007/s10072-026-09429-y","DOIUrl":"10.1007/s10072-026-09429-y","url":null,"abstract":"","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":"47 10","pages":""},"PeriodicalIF":2.7,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149063260","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":"Comparative efficacy of brain-computer interface-coupled robotic rehabilitation, robot-assisted therapy, and motor imagery for upper-limb motor recovery after stroke: a systematic review and network meta-analysis.","authors":"Yunting Wu, Ran Zhang, Fei Wu, Minghui Liu, Yuying Wang, Weige Sun, Weixin Cai","doi":"10.1007/s10072-026-09375-9","DOIUrl":"10.1007/s10072-026-09375-9","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objective: &lt;/strong&gt;To compare and rank the efficacy of brain-computer interface-coupled robotic rehabilitation (BCI-robot), robot-assisted therapy, motor imagery (MI), and conventional rehabilitation for upper-limb recovery after stroke, and to explore whether treatment effects differed according to key clinical and intervention-related characteristics.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;A systematic search was conducted in 11 databases from inception to March 31, 2026. Randomized controlled trials enrolling adults with post-stroke upper-limb motor impairment were included. The primary outcome was the Fugl-Meyer Assessment of Upper Extremity (FMA-UE). Secondary outcomes included the Action Research Arm Test (ARAT), Wolf Motor Function Test (WMFT), and Modified Barthel Index (MBI). Direct pairwise meta-analyses were performed using mean differences (MDs) with 95% confidence intervals (CIs), and a frequentist network meta-analysis was conducted to synthesize direct and indirect evidence and rank interventions using P-scores.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Twenty-three reports representing 22 independent study cohorts were included, comprising 727 participants. Network meta-analysis showed no significant inconsistency, and the consistency model was adopted. BCI-robot ranked highest for improving FMA-UE according to the P-score analysis (P-score = 0.992), followed by robot-assisted therapy (0.641), MI (0.240), and conventional rehabilitation (0.127). In direct comparisons, BCI-robot produced a statistically significant improvement in FMA-UE versus conventional rehabilitation (MD = 6.80, 95% CI 4.29 to 9.30). The point estimate exceeded the 5.25-point (minimal clinically important difference) MCID, although the lower confidence limit did not. Compared with robot-assisted therapy, BCI-robot showed a statistically significant but small improvement (MD = 2.04, 95% CI 0.29 to 3.78), with both the point estimate and the entire 95% CI remaining below the MCID. Follow-up-duration subgroup analyses suggested that BCI-robot remained favorable over conventional rehabilitation in both &lt; 3-month and ≥ 3-month subgroups, whereas no significant follow-up advantage over robot-assisted therapy was observed. For secondary outcomes, BCI-robot significantly improved WMFT versus conventional rehabilitation (MD = 8.94, 95% CI 5.22 to 12.65) and MBI versus conventional rehabilitation (MD = 3.37, 95% CI 0.10 to 6.63). A significant benefit for MBI versus robot was also observed (MD = 8.61, 95% CI 1.76 to 15.46), although this result was not robust in sensitivity analysis. No significant advantage was found for ARAT. Exploratory subgroup analyses suggested that, compared with robot-assisted therapy, the additional benefit of BCI-robot was more apparent in studies delivering more than four sessions per week.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;BCI-robot ranked highest for improving FMA-UE and may provide a clinically important benefit over conventional rehabilitatio","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":"47 10","pages":""},"PeriodicalIF":2.7,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149063255","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":"Longitudinal dynamics and clinical significance of acetylcholine receptor blocking antibodies in myasthenia gravis.","authors":"Aigli G Vakrakou, Maria Belimezi, Eleni Strataki, Eleftherios Kontizas, Dimitra Tzavella, Dimitrios Panaretos, Emmanouil Angelakis, Vasiliki Zouvelou","doi":"10.1007/s10072-026-09423-4","DOIUrl":"10.1007/s10072-026-09423-4","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate the longitudinal clinical relevance of acetylcholine receptor (AChR) blocking antibodies in myasthenia gravis (MG).</p><p><strong>Methods: </strong>We prospectively studied 47 AChR-positive MG patients with 121 serial antibody measurements over a mean follow-up of 2.8 years. AChR-binding antibodies were measured by a conventional radioimmunoprecipitation assay, whereas AChR-blocking activity was measured using a competitive radioimmunoassay. Disease severity was assessed using Myasthenia Gravis Foundation of America (MGFA) classification. Longitudinal associations were analyzed using generalized estimating equations (GEE).</p><p><strong>Results: </strong>Blocking antibodies (> 20% inhibition) were associated with MGFA severity across repeated observations in longitudinal GEE analysis (β = 0.045, p < 0.001), whereas binding antibodies were not significant. Among 13 patients with ocular-onset MG, all 8 who progressed to generalized disease were blocking antibody-positive, while none of the 5 patients who remained ocular tested positive. Blocking antibodies were detected in 90.5% of generalized MG patients, whereas all persistently ocular patients were negative.</p><p><strong>Conclusions: </strong>AChR blocking antibodies are associated with MG disease severity over time and may help identify ocular MG patients at risk of generalization, supporting their potential clinical relevance in MG.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":"47 10","pages":""},"PeriodicalIF":2.7,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13612679/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149040015","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":"Aggressive brainstem listeria abscess in an immunocompetent individual: A case report.","authors":"Pankhuri Banerjee, Destiny Marquez, Alexandra S Reynolds, Fernanda Carvalho Poyraz, Dana Klavansky","doi":"10.1007/s10072-026-09434-1","DOIUrl":"10.1007/s10072-026-09434-1","url":null,"abstract":"<p><p>Listeria monocyotogenes, a gram-positive bacterium is transmitted to humans through ingestion of contaminated foods, including deli meats, soft cheeses, and raw vegetables but can also thrive in soil, water, sewage, manure and animal feed. Its infection in immunocompetent patients is fairly limited to gastrointestinal distress but carries a high mortality rate in immunocompromised patients especially when affecting the central nervous system. We present a challenging case of an immunocompetent patient in their late 50s with neurolisteriosis presenting as multifocal brainstem abscesses with an extensive negative infectious workup and complex antibiotic regimen. This case underscores the diagnostic and therapeutic difficulties of managing invasive neurolisteriosis and aims to enhance critical thinking, particularly in the context of recent recurring listeria outbreaks. Despite being critically ill for months, this patient has had a dramatic recovery and is a reminder that neurologic infections can ultimately be associated with good outcome if the infection is aggressively and promptly treated.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":"47 10","pages":""},"PeriodicalIF":2.7,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149063094","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":"Effectiveness of atogepant in migraine with and without medication overuse: Pooled real-world analysis of two independent clinical cohorts.","authors":"Marcella De Luca, Ludovica Ferraù, Maria Stella Adragna, Andrea Cafarchio, Rosario Grugno, Gianluca Vita, Riccardo Lo Presti, Domenico Cosenza, Massimo Autunno","doi":"10.1007/s10072-026-09428-z","DOIUrl":"10.1007/s10072-026-09428-z","url":null,"abstract":"<p><strong>Background: </strong>Medication-overuse headache (MOH) is common in treatment-resistant chronic migraine and may complicate preventive management. The aim of this study was to evaluate the short-term effectiveness of atogepant 60 mg once daily in a pooled real-world population of patients with migraine, including treatment-resistant chronic migraine, and to explore whether baseline MOH influenced treatment response.</p><p><strong>Methods: </strong>This pooled observational study combined two independent real-world cohorts of patients treated with atogepant 60 mg once daily for migraine prevention. The database included 78 patients, with short-term follow-up recorded at 12 weeks in one cohort and at 3 months in the other. Monthly migraine days (MMD), monthly symptomatic medication use, Migraine Disability Assessment (MIDAS), Headache Impact Test-6 (HIT-6), and treatment documentation were harmonized. Effectiveness was analyzed in the pooled population, while exploratory comparisons were performed according to baseline medication-overuse headache (MOH) status.</p><p><strong>Results: </strong>The pooled database included 78 patients, and paired MMD measurements were available for 73. Median MMD decreased from 13.0 [10.0-20.0] to 4.0 [3.0-8.0], with a median reduction of 7.0 [6.0-11.0] days/month (p < 0.001). A reduction of at least 50% was achieved by 54/73 patients (74.0%). MIDAS, HIT-6, and monthly symptomatic medication use also improved significantly (all p < 0.001). MOH status was available for 77 patients: 24 had MOH and 53 did not. Patients with MOH had a greater baseline burden and larger absolute reductions in MMD and symptomatic medication use; however, MMD percentage reduction and responder rates did not differ significantly by MOH status. Adverse-event documentation was available for 70 patients, of whom 19 had at least one recorded event.</p><p><strong>Conclusions: </strong>In this pooled real-world analysis, atogepant was associated with clinically meaningful short-term improvement in migraine frequency, disability, headache impact, and symptomatic medication use. Baseline MOH was associated with greater initial disease burden but did not appear to attenuate proportional treatment response. Because safety and persistence were documented differently in the two cohorts, pooled tolerability estimates should be interpreted descriptively.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":"47 10","pages":""},"PeriodicalIF":2.7,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149039966","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}