JOURNAL OF NEUROLOGY神经病学杂志
JOURNAL OF NEUROLOGY(英文缩写 J NEUROL),ISSN 0340-5354,eISSN 1432-1459,中文译名:神经病学杂志 是一本学术期刊。本页汇总该期刊的最新影响因子、分区信息以及最新收录于 PubMed 的文献,帮助您快速了解期刊全貌。
发文量统计区间：2025-10-01 至 2026-09-30，按本站收录文献的发表日期统计。
期刊介绍
历年影响因子趋势
| JCR 数据年份 | 影响因子 | JCR 分区 |
|---|---|---|
| 2021 | 6.682 | Q1 |
| 2022 | 6.000 | Q1 |
| 2023 | 4.800 | Q1 |
| 2024 | 4.600 | Q1 |
| 2025 | 5.400 | Q1 |
JOURNAL OF NEUROLOGY 最新收录文献
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1. Cerebrospinal fluid inflammatory proteomic profiling identifies biomarkers linked to disease progression in amyotrophic lateral sclerosis.
1. 脑脊液炎症蛋白质组学分析识别出与肌萎缩侧索硬化症疾病进展相关的生物标志物PMID:日期:2026-10-04Neuroinflammation is a key component of amyotrophic lateral sclerosis (ALS), but its association with disease progression heterogeneity remains unclear. We aimed to identify CSF inflammation-related proteins associated with disease progression phenotype in patients with ALS. Patients with ALS were stratified into slow progressors (SP) and fast progressors (FP) according to the disease progression rate. CSF inflammatory proteins were measured using the Olink Target 96 Inflammation panel. Disease-associated biomarkers were identified using limma package and elastic net (EN) analysis. We assessed the discriminatory power of disease-associated biomarkers using receiver operating characteristic curve analysis and estimated the optimism-adjusted area under the curve (AUC) using the bootstrapping method. Among 77 patients (SP: n=40; FP: n=37), limma analysis identified 11 differentially expressed proteins, with functional enrichment in chemokine signaling, TNF-related responses and NF-κB pathways. EN analysis identified 12 candidate proteins, among which CST5, CCL11, SIRT2, CD6, CCL4, MMP-1, TNFRSF9, CCL19, and MCP-4 showed positive associations with the SP phenotype. A model combining SIRT2, MMP-1, BMI, and site of onset yielded an apparent area under the curve of 0.769 (95% CI 0.662 to 0.876) and an optimism-corrected area under the curve of 0.729. Our study uncovered a CSF inflammatory proteomic signature associated with slow disease progression in ALS. These findings indicate that specific CSF neuroinflammatory protein signatures reflect biological heterogeneity in ALS disease progression rather than non-specific neurodegeneration. SIRT2 and MMP-1 warrant further investigation as components of progression-stratification models, although validation in larger, longitudinal, and independent cohorts is required.
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3. Non-motor symptoms burden and motor recovery following STN-DBS in Parkinson's disease: a data-driven longitudinal analysis.
PMID:日期:2026-10-01Subthalamic nucleus-deep brain stimulation (STN-DBS) reliably improves cardinal motor symptoms in Parkinson's disease (PD). However, gait disorders and non-motor symptoms (NMS) may persist or emerge following surgery, diminishing quality of life and complicating clinical management. To characterize the dominant axes of clinical covariance across motor and non-motor domains before and after STN-DBS, and to determine whether non-motor symptom severity moderates post-DBS gait and mobility change. 100 PD patients undergoing STN-DBS (mean age 60.2 ± 7.6 years; 67% male) were studied. Principal component analysis (PCA) was performed on thirteen motor and non-motor sub-scores at pre-DBS, 1-year follow-up (1-y FU), and a subset (n = 33) at 3-5-year follow-up. Moderation was tested using analysis of covariance (ANCOVA): each 1-year motor outcome was regressed on its pre-DBS baseline, a non-motor moderator, and their interaction, adjusting for age and disease duration, with a significant interaction indicating that baseline non-motor severity was associated with the degree of subsequent motor recovery. PCA identified a core motor triad: gait, mobility, and freezing of gait as the primary source of clinical variance at all timepoints (PC1: 35% pre-DBS, declining to 28% at 3-5-y FU), PC1 was the only component to exceed a permutation null (p < 0.001), with memory-related impairment increased at longer-term follow-up. Moderation analysis showed that higher pre-operative mood burden specifically for psychomotor and psychosomatic symptoms were associated with poorer recovery of mobility (Psychomotor β = 1.16, p < 0.05). At 1-y FU anxiety and psychosomatic symptoms showed concurrent associations with mobility and overall motor burden (Anxiety β = 2.30, p < 0.05). STN-DBS outcomes extend beyond global motor improvement and may be shaped by associations spanning the motor, cognitive, and mood domains. Higher NMS baseline burden was associated with poorer mobility recovery, and mood symptoms at follow-up were associated with concurrent motor burden. These exploratory findings support considering multi-domain pre-operative assessment and post-operative mood monitoring, as potential targets for optimizing motor outcomes.
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4. Multimodal PET-MRI profiling predicts dementia with Lewy bodies in isolated REM sleep behaviour disorder.
PMID:日期:2026-10-01Despite isolated REM sleep behaviour disorder (iRBD) reliably identifying individuals at high risk of developing Parkinson's disease (PD) or dementia with Lewy bodies (DLB), with more than 90% converting, the neurobiological markers of the conversion phenotype are not fully understood. Most previous studies have found univariate imaging biomarkers of phenoconversion, and most of these lack specificity for PD and DLB. Using a well-characterised iRBD cohort (N=21), receiving multiple PET and MRI scans, we aimed to assess if the inter-modality relationship can specifically predict conversion to PD or DLB. The patients received [F]-DOPA, [C]-Donepezil and [C](R)-PK11195 PET and structural MRI scans to derive grey matter (GM) volume along with dynamic susceptibility contrast MRI to extract measures of microcirculatory dysregulation. We used a multimodal adaptation of the scaled sub-profile model to identify patterns of multimodal covariance across the PET and MRI scans. We identified a multimodal network characterised by high neuroinflammation, cholinergic dysfunction, GM atrophy and microcirculatory dysfunction with the covariance pattern converging in the medial occipito-parietal cortex (OPC). The combined pathology from the OPC and striatal [F]-DOPA uptake was able to specifically predict conversion into DLB (sub-distribution hazard ratio=38.68, 95% confidence interval: 7.545-198.3), and this survived correction for age and disease duration. These findings demonstrate the feasibility of integrating multiple modalities in risk assessment and suggest that posterior cortical pathology, along with striatal dopaminergic impairment, could hold predictive value for phenotype-specific conversion of iRBD patients, and that multiple pathological processes converge in this brain region.
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5. Spinal cord imaging in multiple sclerosis: from diagnosis to disease progression.
PMID:日期:2026-09-30Spinal cord (SC) involvement in multiple sclerosis (MS) is common and a major determinant of long-term disability in both relapsing and progressive disease phenotypes. Despite this, SC pathology remains under-assessed in both clinical practice and therapeutic trials, largely due to technical challenges inherent to spinal SC magnetic resonance imaging (MRI) conferred by the cord's small anatomical size and sensitivity to physiological motion and susceptibility artefact. This review synthesises current understanding of SC involvement in MS, integrating advances in pathophysiology, imaging, and clinical application. We describe the characteristic MRI features of MS SC lesions and their key inflammatory and non-inflammatory differentials, and examine the role of SC imaging in diagnosis, particularly in the context of the 2024 revisions to the McDonald criteria. We highlight the prognostic importance of SC lesions and atrophy, including their association with progression independent of relapse activity, and review the limited and heterogeneous evidence regarding the effects of disease-modifying therapies on SC pathology. We further evaluate current MRI acquisition strategies and consensus guidelines, and discuss emerging quantitative and advanced imaging techniques that detect microstructural and biochemical abnormalities beyond conventional imaging. Together, these developments support a more systematic integration of SC imaging into MS clinical care and trial design.
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6. Preliminary insights into whole-blood mitochondrial DNA copy number and telomere length in Parkinson's disease and multiple system atrophy.
PMID:日期:2026-09-29Parkinson's disease (PD) and multiple system atrophy (MSA) are α-synucleinopathies characterized by progressive neurodegeneration and overlapping clinical features but distinct pathological mechanisms with peripheral biomarkers remaining limited. Mitochondrial dysfunction and telomere attrition are hallmarks of cellular aging implicated in neurodegeneration, yet their combined role remains unclear. Whole-blood mitochondrial DNA copy number (mtDNA-CN) and telomere length (TL) were assessed in 58 PD patients, 35 MSA patients, and 62 healthy controls from Southern Italy. Mitochondrially encoded NADH dehydrogenase 1 (ND1) gene levels and TL were measured by quantitative PCR and normalized to the β-actin gene. Group differences were tested using multivariable linear regression adjusted for age and sex, with statistical significance defined as p < 0.05 after false discovery rate (FDR)-corrected Wald tests. ND1-CN was significantly reduced in PD (p = ) and MSA (p = ) compared to controls and was also significantly lower in PD with respect to MSA (p = ). TL was reduced in PD (p = ) and MSA (p = ), with smaller between-group differences (p = ). Our findings support the involvement of mitochondrial dysfunction and telomere attrition in PD and MSA. However, mtDNA-CN provided a clearer distinction between the two α-synucleinopathies than TL, suggesting that peripheral mtDNA-CN may represent a more informative non-invasive biomarker of disease-specific mitochondrial alterations. Further longitudinal studies in larger, independent cohorts are warranted to validate its diagnostic and prognostic utility.
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7. Scleromyxedema-Associated Myopathy and Vacuolar Myopathy with Monoclonal Gammopathy and Stiffness (VAMMGAS) as manifestations of Monoclonal Gammopathy of Clinical Significance (MGCS): a disease spectrum between autophagy dysfunction and inflammation.
PMID:日期:2026-09-29Scleromyxedema-Associated Myopathy (SAM) and Vacuolar Myopathy with Monoclonal Gammopathy and Stiffness (VAMMGAS) are rare clinical entities associated with Monoclonal Gammopathy of Clinical Significance (MGCS). We compared the clinical, neurophysiological, histopathological, and ultrastructural features of three patients (two with SAM and one with VAMMGAS), together with a review of the available literature, to further characterize the similarities and differences between these two conditions. Our analysis suggests that SAM and VAMMGAS may represent part of a clinical spectrum characterized by progressive proximal muscle weakness and prominent pathological spontaneous activity on electromyography. By definition, SAM is associated with scleromyxedema, which may occasionally develop after the onset of myopathy, whereas VAMMGAS is characterized by muscle stiffness. The underlying monoclonal gammopathy is most commonly IgG ? in SAM, while IgG ? appears to be more frequent in VAMMGAS. Histopathologically, both conditions show a myopathy with features suggestive of impaired autophagy and evidence of immune activation (abnormal HLA-I expression and sarcolemmal C5b-9 deposits), although inflammatory infiltrates appear to be more characteristic of SAM, providing insights into potentially shared pathogenic mechanisms. Taken together, these findings support the concept that SAM and VAMMGAS may represent related manifestations within the spectrum of MGCS-associated muscle disease. Recognition of their overlapping clinical, neurophysiological, and histological features may facilitate diagnosis and broaden our understanding of the underlying pathophysiological mechanisms, potentially informing therapeutic strategies. Importantly, both conditions appear to be treatable with immunomodulatory approaches (including intravenous immunoglobulins and plasma exchange) as well as clone-directed therapies.
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8. Stroke mimic caused by severe hypomagnesemia: a case report and literature review.
PMID:日期:2026-09-29Association of hypomagnesemia and neurological deficits is a well documented issue, but despite its importance, magnesium levels are rarely tested during the acute phase of a stroke. Focal neurological deficits have rarely been ascribed to hypomagnesemia. We report the case of a patient treated with Proton Pump Inhibitors and Metformin with a history of unexplained transient neurological deficits who developed stroke-like symptoms of hemiparesis and aphasia with recovery after correction of his hypomagnesemia. We also present a brief literature review regarding Magnesium's role in focal neurological deficits.
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9. Interlocking pentagons task and visuospatial deficit in parkinsonian syndrome: a quantitative approach.
PMID:日期:2026-09-28Visuo-spatial impairment is a common feature in parkinsonian disorders and differentiating idiopathic Parkinson's disease (PD) from atypical parkinsonian syndromes (APS), i.e. progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal degeneration (CBD), remains a major clinical challenge. The Qualitative Scoring Pentagon Test (QSPT), derived from the MMSE pentagon-copying item, provides quantitative visuo-constructive assessment but has not been systematically evaluated as a differential marker in parkinsonism. Aim of the study was to assess the discriminative value of the QSPT total score and sub-items across parkinsonian syndromes, and to examine associations with neuropsychological tests, motor severity, and MRI morphometric indices. Patients with PD and APS were enrolled and underwent clinical and neuropsychological evaluations including MMSE, Frontal Assessment Battery (FAB), Trail Making Test (TMT-A), and MDS-Unified Parkinson's Disease Rating Scale-Section III (MDS-UPDRS-III). A group of healthy controls (HC) were also enrolled performing MMSE. Pentagon drawings were scored quantitatively (QSPT). When available, MRI morphometric features were collected. Statistical analysis were performed to assess associations between QSPT, cognitive and clinical features, and to evaluate differences between patients and HC and across patients' groups. One-hundred and forty patients (PD=91, PSP=23, MSA=17, CBD=9) and 92 HC were enrolled. QSPT differed significantly considering HC performances (12.7 ± 0.5) versus patients groups, and across groups (p<0.001), with PD scoring the highest (11.5 ± 2.1) and CBD the lowest score (7.7 ± 4.4). PD differed from PSP (p<0.001), CBD (p<0.001) and MSA (p=0.040). Rotation and number-of-angles sub-items were the most discriminating features. QSPT significantly correlated with MMSE, FAB, TMT-A, and MRPI (all p<0.01). Multivariate models demonstrated that QSPT was independently associated with APS (OR=0.620, 95%CI 0.469-0.820, p<0.001). A QSPT score ≤ 8 points discriminated PD from APS with an AUC of 0.764 and a specificity of 92.3%, and ≤ 11 points discriminated synucleinopathies from tauopathies with a higher AUC (0.804). This study demonstrates that quantitative scoring of the MMSE pentagon-copying item could differentiate PD from APS. The presence of association between QSPT and MRI brainstem morphometry could also reflect a structural association, specifically identifying tauopathies. QSPT could be a simple and supportive clinical tool, embedded within the MMSE that could differentiates patients from HC and PD from APS.
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10. Intravenous thrombolysis use before interhospital transfer for thrombectomy: a systematic review and meta-analysis.
PMID:日期:2026-09-28Intravenous thrombolysis (IVT) before interhospital transfer for endovascular thrombectomy (EVT) in patients with large-vessel occlusion acute ischemic stroke (LVO-AIS) remains controversial. We aimed to evaluate the efficacy and safety of pre-transfer IVT in LVO-AIS patients who presented to primary stroke centers (PSCs) and required transfer to comprehensive stroke centers (CSCs) for EVT. We systematically searched PubMed, Embase, and ClinicalTrials.gov from inception to July 2, 2026. Studies comparing IVT versus no IVT prior to interhospital transfer for EVT in patients with LVO-AIS were included. Efficacy outcomes included the primary outcome of excellent functional outcome (modified Rankin Scale [mRS] 0-1 at 90 days), as well as secondary efficacy outcomes including good functional outcome (mRS 0-2 at 90 days), interhospital recanalization, and poor functional outcome (mRS 5-6). Safety outcomes included 90-day mortality, symptomatic intracranial hemorrhage (sICH), any intracranial hemorrhage (ICH), and parenchymal hematoma (PH). Pooled odds ratios (ORs) with 95% confidence intervals (CIs) were calculated using random-effects models, and adjusted estimates were synthesized using the generic inverse variance method. Sixteen observational studies were included. Pre-transfer IVT was associated with significantly higher rates of excellent functional outcome (OR 1.72, 95% CI 1.47-2.01, p<0.00001), good functional outcome (OR 1.64, 95% CI 1.35-1.99, p<0.00001), and interhospital recanalization (OR 7.71, 95% CI 4.08-14.55, p<0.00001), as well as significantly lower risks of poor functional outcome (OR 0.65, 95% CI 0.54-0.79, p<0.0001) and 90-day mortality (OR 0.69, 95% CI 0.59-0.81, p<0.00001). No significant differences were observed between groups in the risks of sICH (OR 0.75, 95% CI 0.50-1.11, p=0.15), any ICH (OR 0.91, 95% CI 0.76-1.09, p=0.31), or PH (OR 1.43, 95% CI 0.88-2.33, p=0.15). In the adjusted analyses, the associations remained significant for excellent functional outcome (aOR 1.47, 95% CI 1.19-1.81, p=0.0004), good functional outcome (aOR 1.61, 95% CI 1.25-2.07, p=0.0002), interhospital recanalization (aOR 8.06, 95% CI 4.71-13.82, p<0.00001), and 90-day mortality (aOR 0.64, 95% CI 0.49-0.84, p=0.001). The magnitude and direction of associations were generally consistent with the unadjusted analyses. Among LVO-AIS patients requiring interhospital transfer from PSCs to CSCs for EVT, pre-transfer IVT was associated with improved functional outcomes and interhospital recanalization, without increasing hemorrhagic risk. These findings support pre-transfer IVT as an effective reperfusion strategy in resource‑limited settings where timely EVT is not immediately accessible.