Journal of Neurology最新文献

{"title":"Recurrent posterior stroke-like lesions heralding COQ8A-related coenzyme Q10 deficiency: a potentially treatable ataxia mistaken for stroke: a case report.","authors":"Sarika Mutyala, Prateek, Ashmit Gupta, Chiranjeevi Kona","doi":"10.1007/s00415-026-14209-x","DOIUrl":"https://doi.org/10.1007/s00415-026-14209-x","url":null,"abstract":"","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 11","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149182558","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acute cerebellitis presenting predominantly with vestibular symptoms: reversed corrective saccades and frequency-dependent VOR hyperactivity as clues to cerebellar disinhibition.","authors":"Hyesoo Kwon, Hae Won Kim, Hyun Ah Kim","doi":"10.1007/s00415-026-14204-2","DOIUrl":"https://doi.org/10.1007/s00415-026-14204-2","url":null,"abstract":"","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 11","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149182523","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"MR spectroscopy of the cerebellum in gluten ataxia and gluten encephalopathy.","authors":"M Hadjivassiliou, R A Grunewald, I Croal, N Hoggard","doi":"10.1007/s00415-026-14200-6","DOIUrl":"10.1007/s00415-026-14200-6","url":null,"abstract":"<p><strong>Objective: </strong>To identify any MR spectroscopy differences amongst patients with gluten ataxia and patients with gluten encephalopathy and interpret what this tells us about cerebellar functionality.</p><p><strong>Methods: </strong>Retrospective review of MR spectroscopy baseline data from 40 patients with gluten ataxia (GA), 40 patients with gluten encephalopathy (GE) and a group of 55 healthy controls.</p><p><strong>Results: </strong>The mean age at development of ataxia in the GA group was 50 years. In the GE group, the mean age at development of neurological symptoms was 37 years. The mean age of the healthy control group at the time of scanning was 41 years. Patients with GA showed highly significant reduction in the NAA/Cr (N-acetyl-aspartate/creatine) ratio from the vermis NAA/Cr 0.85 vs 0.99 in healthy controls (p = 0.0000105, Welch's t-test). Within this same group, the right hemisphere NAA/Cr area ratio was 0.969 vs 1.01 in healthy controls (p = 0.058). By contrast, patients with GE showed significant reduction of NAA/Cr area ratio in the right hemisphere 0.926 vs 1.01 in healthy controls (p = 0.00053, Welch's t-test) but no difference from control subjects when comparing vermis NAA/Cr.</p><p><strong>Conclusions: </strong>MR spectroscopy of the cerebellum demonstrates dysfunction in the vermis in patients with GA, whereas patients with GE have abnormal spectroscopy in the right cerebellar hemisphere in the absence of ataxia. The right cerebellar hemisphere may be implicated in cerebellar cognitive affective syndrome.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 11","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13661738/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149182556","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hemi-see-saw nystagmus with superimposed pendular oculopalatal tremor: case report and systematic review.","authors":"Jesus Gutierrez-Arratia, Fiorella Rivas-Sarmiento, Christoper A Alarcon-Ruiz, Tony Torres-Inuma, Guido Alban-Zapata, Justiniano Zea-Quispe","doi":"10.1007/s00415-026-14213-1","DOIUrl":"10.1007/s00415-026-14213-1","url":null,"abstract":"<p><strong>Background: </strong>Hemi-see-saw nystagmus (HSSN) is an uncommon subtype, characterized by elevation and intorsion of one eye with contralateral depression and extorsion, typically in a jerk pattern. It is associated with unilateral lesions of the interstitial nucleus of Cajal and the medial longitudinal fasciculus, both of which are involved in the ocular tilt reaction (OTR). Although its association with oculopalatal tremor (OPT) has been described, it is rare.</p><p><strong>Methods: </strong>We performed a systematic review of seven databases on HSSN, extracting data on demographics, etiology, lesion location, oculomotor findings, nystagmus pattern, outcomes, and in addition we presented a representative case.</p><p><strong>Results: </strong>A systematic review of seven databases identified 18 reports comprising 57 cases of HSSN or closely related dissociated vertical-torsional nystagmus, including the present index case. The mean age was 56.2 ± 16.3 years, and 63.2% of patients were male. Ischemic stroke was the most frequent etiology (75.4%), followed by hemorrhagic stroke (14.0%). Less frequent causes included Chiari I malformation, demyelinating/inflammatory disease, cavernous malformation, ataxia with oculomotor apraxia type 2, Joubert syndrome, and brainstem encephalitis (1.8% each). Jerk nystagmus predominated (82.5%), whereas pendular nystagmus was identified in 17.5%, exclusively in the setting of OPT or inferior olivary hypertrophy. Among jerk HSSN cases, the pontine tegmentum was the most frequent lesion site (63.8%), with INO present in all pontine cases and OTR in 90.0%. Complete resolution was reported predominantly in cases associated with INO, whereas lack of improvement was confined to OPT-related cases, with one exception of spontaneous resolution after 20 years. We describe a 30-year-old man with a pontomedullary arteriovenous malformation presenting with pendular HSSN, right one-and-a-half syndrome, gaze-evoked nystagmus, OTR, incomplete Horner syndrome, and OPT.</p><p><strong>Conclusions: </strong>HSSN is an uncommon nystagmus, most often vascular in origin and frequently involving the pontine tegmentum. While it usually presents with a jerk waveform, association with OPT may produce a pendular phenotype, suggesting a distinct brainstem-cerebellar mechanism. Larger anatomically and oculographically characterized series are needed to clarify structure-function correlations and prognosis.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 11","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149182580","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Consensus-based perspectives on integrating IPX203 into Parkinson's disease management.","authors":"Günter Höglinger, Carsten Buhmann, Georg Ebersbach, Margherita Fabbri, Georg Kägi, Javier Pagonabarraga, Diego Santos García, Alessandro Tessitore, Michele Tinazzi","doi":"10.1007/s00415-026-14186-1","DOIUrl":"10.1007/s00415-026-14186-1","url":null,"abstract":"<p><p>Motor fluctuations are a common and burdensome complication of Parkinson's disease (PD) when the disease progresses, largely driven by the short half-life and variable absorption of immediate-release (IR) levodopa. IPX203 is a next-generation modified-release levodopa/carbidopa formulation that delivers rapid onset and sustained plasma levodopa concentrations through IR granules and extended-release pellets. Clinical studies have shown that IPX203 increases Good ON time (ON time without dyskinesia plus ON time with non-troublesome dyskinesia, equivalent to ON time without troublesome dyskinesia), reduces OFF time and improves motor symptom control compared with IR levodopa/carbidopa. In anticipation of the introduction of IPX203 in Europe, nine PD specialists convened to develop consensus recommendations for its use in clinical practice. Through a structured process incorporating predefined statements, voting and moderated discussion, the group reached high levels of agreement (88.9% to 100%) across seven topics. These included defining motor symptoms control, identifying patients most likely to benefit from IPX203, positioning of IPX203 within the treatment pathway, practical considerations for switching to IPX203 from current regimens and recommendations for monitoring and follow-up. The experts emphasised that IPX203 should be considered for patients with motor fluctuations insufficiently controlled on optimised oral levodopa therapy, before introduction of device-aided therapies. They highlighted the need for flexible dose adjustments, comprehensive patient and neurologist education and practical tools to support conversion to IPX203. This consensus insight provides practical guidance for integrating IPX203 into routine care of PD patients with motor fluctuations, aiming to increase mobility and reduce treatment burden for patients with PD.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 11","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13653810/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149166402","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"An update on neuro-otology and neuro-ophthalmology - focusing on new developments in the last three years.","authors":"Alexander A Tarnutzer, Michael L Strupp, Louisa Lehner","doi":"10.1007/s00415-026-14153-w","DOIUrl":"10.1007/s00415-026-14153-w","url":null,"abstract":"<p><p>Vertigo, dizziness, and imbalance are among the most frequent presenting symptoms in primary care and in the emergency department (ED). The clinical diagnosis is based on a structured patient history and bedside examination of the vestibular system and the ocular motor system, looking especially for central ocular motor signs. Recent guidelines, such as the GRACE-3 guideline on acute vertigo and dizziness, the increasing availability of bedside diagnostic algorithms, and the use of quantitative vestibular and ocular motor testing in both the ED-setting and in the outpatient center have facilitated and standardized the approach to the dizzy patient. Here we review recent developments in neuro-otology and neuro-ophthalmology, covering the period after the latest neuro-otology update was published in this journal in 2023. Both new-onset acute, episodic and chronic vestibular conditions are discussed separately, highlighting advancements in disease characterization, diagnostic criteria, and treatment strategies. While major progress has been made in terms of the pathophysiology, etiology, genetics, and diagnostic criteria of peripheral, central, and functional vestibular diseases as well as ocular motor disorders, this knowledge has to be transferred into clinical practice. Systematic teaching of doctors to increase knowledge and to improve skills in applying the different tests in clinical practice on a high level are identified challenges. In terms of treatment, there is still a very great need for state-of-the art clinical trials applying approved therapies.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 11","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13654878/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149165667","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"S.T. Narasimhan (1913-1959).","authors":"Shravan Harish, Nishanth Gowda, Nikita Balachandar","doi":"10.1007/s00415-026-14197-y","DOIUrl":"https://doi.org/10.1007/s00415-026-14197-y","url":null,"abstract":"","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 11","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149167158","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exercise modality and planned weekly exercise exposure for motor outcomes in Parkinson's disease: a systematic review, Bayesian network meta-analysis, and meta-regression.","authors":"Long Wang, Helong Wang, Fuxin Hong, Zhen Wan","doi":"10.1007/s00415-026-14189-y","DOIUrl":"10.1007/s00415-026-14189-y","url":null,"abstract":"<p><strong>Background: </strong>Exercise modality and planned weekly structured-exercise exposure may provide complementary information for Parkinson's disease motor outcomes, but their outcome-specific effects and evidential confidence remain uncertain.</p><p><strong>Methods: </strong>We synthesized 173 randomized trials using covariance-aware Bayesian random-effects network meta-analysis for motor symptoms, balance, walking speed, and Timed Up and Go (TUG). A key secondary analysis estimated exercise-class-adjusted associations per 250 metabolic equivalent task minutes/week, with pre-specified sensitivity analyses.</p><p><strong>Results: </strong>The category NMA networks included 110, 106, 89, and 96 trials for Unified Parkinson's Disease Rating Scale part III (UPDRS III), balance, walking speed, and TUG, respectively. Mind-body exercise had the largest posterior median versus non-exercise control for UPDRS III (g = 0.65, 95% credible interval [CrI] 0.42-0.87). In the reconstructed, exercise-category-adjusted dose models, planned weekly structured-exercise exposure was positively associated at the trial level with UPDRS III (0.121 SMD per + 250 MET-min/week, 95% CrI 0.055-0.194), balance (0.111, 0.047-0.184), and TUG (0.124, 0.058-0.190), but not walking speed (0.008, - 0.063 to 0.076). The TUG estimate attenuated after P95 dose truncation. All active-versus-control prediction intervals crossed zero; CINeMA confidence was low or very low. The evidence did not establish a universally best modality, an optimal dose, or an individual causal target. Higher planned weekly exposure was associated at the trial level with greater average improvement in UPDRS III, balance, and TUG, whereas walking speed showed no clear association. However, within-class randomized dose-contrast estimates were imprecise for all four outcomes, and the TUG association was sensitive to the high-dose tail.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 11","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149166337","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intracranial rescue stenting in the German stroke registry: a propensity score-matched multicenter study.","authors":"Malte Konrad, Lukas Meyer, Gabriel Broocks, Matthias Bechstein, Christian Thaler, Christian Heitkamp, Laurens Winkelmeier, Vincent Geest, Felix Schlicht, Alexander Heitkamp, Gregor Peter, Luca Meucci, Bogdana Tokareva, Tobias Faizy, Caspar Brekenfeld, Maximilian Schell, Götz Thomalla, Jens Fiehler, Susanne Gellißen, Helge Kniep, Fabian Flottmann","doi":"10.1007/s00415-026-14180-7","DOIUrl":"10.1007/s00415-026-14180-7","url":null,"abstract":"<p><strong>Background: </strong>Intracranial stenting (ICS) is used as a rescue strategy in patients with failed reperfusion after endovascular thrombectomy (EVT). However, real-world data on clinical outcomes and safety remain limited.</p><p><strong>Methods: </strong>This retrospective analysis of prospectively collected data from the German Stroke Registry-Endovascular Treatment (GSR-ET) included patients with acute ischemic stroke due to middle cerebral artery (MCA) occlusion treated between June 2015 and December 2023. Patients undergoing ICS were compared with a 1:1 propensity score-matched cohort with failed reperfusion after EVT (final modified Thrombolysis in Cerebral Infarction [mTICI] score <2b) who received no further endovascular treatment. Functional, safety, and subgroup outcomes were assessed.</p><p><strong>Results: </strong>Among the 14,714 eligible patients, ICS was performed in 352 (2.4%). After matching, 218 patients undergoing ICS were compared with 218 controls with failed reperfusion. ICS was associated with a shift toward better 90-day functional outcomes in unadjusted (cOR 0.52, 95% CI 0.37-0.73; p<0.01) and adjusted analyses (acOR 0.41, 95% CI 0.29-0.59; p<0.01). Malignant MCA infarction occurred less frequently in the ICS group (1% vs. 8%; p<0.01), as did 90-day mortality (26% vs. 37%; p=0.02). Symptomatic intracranial hemorrhage did not differ between groups (4% vs. 5%; p=0.63).</p><p><strong>Conclusions: </strong>Patients treated with ICS had a shift toward better functional outcomes and lower 90-day mortality than those with failed reperfusion who received no further endovascular treatment. Symptomatic intracranial hemorrhage did not differ between groups. These findings support further investigation of ICS after failed EVT, although randomized studies are needed to determine its efficacy and safety.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 11","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13652770/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149148515","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pre-diagnostic ALSFRS-R slope provides limited incremental information about the variability in subsequent functional decline in amyotrophic lateral sclerosis: a population-based study.","authors":"Rosario Vasta, Andrea Calvo, Cristina Moglia, Antonio Canosa, Umberto Manera, Giulia Gianferrari, Elisabetta Zucchi, Ilaria Martinelli, Gabriele Mora, Sara Cabras, Francesca Palumbo, Enrico Matteoni, Alessandra Maccabeo, Giorgio Pellegrino, Emilio Minerva, Daniela Pascariu, Stefano Callegaro, Fabrizio D'Ovidio, Letizia Mazzini, Fabiola De Marchi, Jessica Mandrioli, Adriano Chiò","doi":"10.1007/s00415-026-14177-2","DOIUrl":"10.1007/s00415-026-14177-2","url":null,"abstract":"<p><strong>Background: </strong>The pre-diagnostic ALSFRS-R slope is frequently used in a clinical trial design and clinical practice under the implicit assumption that it reflects subsequent functional decline. We assessed the association between the pre-diagnostic ALSFRS-R slope and subsequent functional decline.</p><p><strong>Methods: </strong>The Piemonte and Valle d'Aosta ALS Registry (PARALS) was used as the primary cohort. The associations between pre- and post-diagnostic slopes at 6, 12, and 18 months were examined using correlation coefficients and multivariable linear regression. Linear and quadratic mixed-effects models used all ALSFRS-R assessments obtained during the first 18 months to evaluate longitudinal trajectories and quantify the incremental contribution of the pre-diagnostic slope. All analyses were replicated in the Emilia Romagna Registry for ALS (ERRALS).</p><p><strong>Results: </strong>A total of 2,222 patients from PARALS and 1,543 from ERRALS were included. In PARALS, the pre-diagnostic ALSFRS-R slope showed only a modest monotonic association with post-diagnostic functional decline (Spearman's ρ = 0.19-0.30) and explained 6.1% of its variance at 6 months, with this proportion decreasing thereafter. Quadratic mixed-effects models estimated that, at 12 months, the difference in ALSFRS-R change between patients in the highest and lowest pre-diagnostic slope quartiles was 2.04 points in PARALS (95% CI 1.25-2.83) and 2.47 points in ERRALS (95% CI 1.07-3.86). However, the linear and quadratic interactions together increased the marginal R² by only 0.43 percentage points in PARALS and 1.68 percentage points in ERRALS.</p><p><strong>Conclusions: </strong>The pre-diagnostic ALSFRS-R slope identifies meaningful average differences in subsequent decline between patient groups but provides limited information for predicting individual trajectories.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 10","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13649887/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149137075","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}