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Effectiveness of CT perfusion in posterior circulation stroke: evaluation of perfusion abnormalities and associated clinical signs.
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-02-22 DOI: 10.1007/s00415-025-12933-4
Giovanni Furlanis, Edoardo Ricci, Miloš Ajčević, Filippo Spigariol, Emanuele Vincis, Gabriele Prandin, Laura Mancinelli, Federica Palacino, Magda Quagliotto, Paola Caruso, Maja Ukmar, Marcello Naccarato, Paolo Manganotti
{"title":"Effectiveness of CT perfusion in posterior circulation stroke: evaluation of perfusion abnormalities and associated clinical signs.","authors":"Giovanni Furlanis, Edoardo Ricci, Miloš Ajčević, Filippo Spigariol, Emanuele Vincis, Gabriele Prandin, Laura Mancinelli, Federica Palacino, Magda Quagliotto, Paola Caruso, Maja Ukmar, Marcello Naccarato, Paolo Manganotti","doi":"10.1007/s00415-025-12933-4","DOIUrl":"10.1007/s00415-025-12933-4","url":null,"abstract":"<p><strong>Purpose: </strong>Acute posterior circulation stroke (PCS) is characterized by often non-specific clinical signs, with neuroimaging playing a pivotal role in assessment in the emergency setting. The aim of this study was to investigate the effectiveness of CT Perfusion (CTP) maps in detecting acute PCS and to identify clinical factors associated with perfusion abnormalities.</p><p><strong>Methods: </strong>We retrospectively analyzed clinical and radiological data of consecutive patients with acute PCS admitted to our Stroke Unit that underwent CTP. Follow-up NECT or MRI was performed to confirm the diagnosis of PCS. The effectiveness of CTP to identify PCS was evaluated as the ratio of the CTP in which perfusion abnormalities, compatible with an ischemic event, were present in at least one CTP map among MTT, CBF, TTP, and CBV. Multivariate logistic regression analysis was conducted to identify clinical factors associated with perfusion abnormalities.</p><p><strong>Results: </strong>CTP showed alterations in 69 of 107 PCS (64.5%) included in final analysis and MTT proved to be the most sensitive. Multivariate analysis showed that atrial fibrillation (OR = 8.571, CI 95% 2.224-33.037, p = 0.002), dyslipidemia (OR = 0.285, CI 95% 0.100-0.814, p = 0.019), visual field deficits (OR = 3.372, CI 95% 1.020-11.150, p = 0.046), and higher neurological deficit (NIHSS > 5) (OR = 4.054, CI 95% 1.147-14.331, p = 0.030) were significantly associated with perfusion abnormalities on CTP.</p><p><strong>Conclusion: </strong>CT perfusion can be a valuable resource for detecting acute PCS showing a moderately high positivity rate, higher than that of NECT alone or CTA. These findings contribute to the growing body of evidence supporting the use of perfusion imaging in acute posterior circulation stroke.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"225"},"PeriodicalIF":4.8,"publicationDate":"2025-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11846719/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476561","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}
引用次数: 0
Invasive therapies for Parkinson's disease: an adapted excerpt from the guidelines of the German Society of Neurology.
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-02-22 DOI: 10.1007/s00415-025-12915-6
René Reese, Thomas Koeglsperger, Christoph Schrader, Lars Tönges, Günther Deuschl, Andrea A Kühn, Paul Krack, Alfons Schnitzler, Alexander Storch, Claudia Trenkwalder, Günter U Höglinger
{"title":"Invasive therapies for Parkinson's disease: an adapted excerpt from the guidelines of the German Society of Neurology.","authors":"René Reese, Thomas Koeglsperger, Christoph Schrader, Lars Tönges, Günther Deuschl, Andrea A Kühn, Paul Krack, Alfons Schnitzler, Alexander Storch, Claudia Trenkwalder, Günter U Höglinger","doi":"10.1007/s00415-025-12915-6","DOIUrl":"10.1007/s00415-025-12915-6","url":null,"abstract":"<p><strong>Background: </strong>Parkinson's disease (PD) is characterized by hypokinetic motor symptoms, tremor, and various non-motor symptoms with frequent fluctuations of symptoms in advanced disease stages. Invasive therapies, such as deep brain stimulation (DBS), ablative therapies, and continuous subcutaneous or intrajejunal delivery of dopaminergic drugs via pump therapies are available for the management of this complex motor symptomatology and may also impact non-motor symptoms. The recent update of the clinical guideline on PD by the German Neurological Society (Deutsche Gesellschaft für Neurologie e.V.; DGN) offers clear guidance on the indications and applications of these treatment options.</p><p><strong>Methods: </strong>The guideline committee formulated diagnostic questions for invasive therapies and structured them according to the PICOS framework (Population-Intervention-Comparisons-Outcome-Studies). A systematic literature review was conducted. Questions were addressed using the findings from the literature review and consented by the guideline committee.</p><p><strong>Results: </strong>Specific recommendations are given regarding (i) the optimal timing for starting invasive therapies, (ii) the application of DBS, (iii) the use of pump therapies in advanced PD, (iv) the indications for ablative procedures, and (iv) selecting the most appropriate therapy according to individual patient characteristics.</p><p><strong>Conclusion: </strong>This review is an adapted excerpt of the chapters on the use of invasive therapies in PD of the novel German guideline on PD. Clear recommendations on the use of treatment options for advanced PD are provided.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"219"},"PeriodicalIF":4.8,"publicationDate":"2025-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11846738/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476585","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}
引用次数: 0
Richard Caton (1842-1926).
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-02-22 DOI: 10.1007/s00415-025-12935-2
Andrew J Larner
{"title":"Richard Caton (1842-1926).","authors":"Andrew J Larner","doi":"10.1007/s00415-025-12935-2","DOIUrl":"10.1007/s00415-025-12935-2","url":null,"abstract":"","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"221"},"PeriodicalIF":4.8,"publicationDate":"2025-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476592","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}
引用次数: 0
Specific contribution of cognitive and motor impairments with functional capacity and dependence in Huntington's disease.
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-02-22 DOI: 10.1007/s00415-025-12982-9
Andres Gil-Salcedo, Marine Lunven, Charlotte Jacquemot, Renaud Massart, Anne-Catherine Bachoud-Levi
{"title":"Specific contribution of cognitive and motor impairments with functional capacity and dependence in Huntington's disease.","authors":"Andres Gil-Salcedo, Marine Lunven, Charlotte Jacquemot, Renaud Massart, Anne-Catherine Bachoud-Levi","doi":"10.1007/s00415-025-12982-9","DOIUrl":"10.1007/s00415-025-12982-9","url":null,"abstract":"<p><strong>Background: </strong>Huntington's disease (HD) leads to increasing dependence. Unlike psychiatric disorders, motor and cognitive deficits evolve progressively over time. Understanding their specific impact on daily activities is crucial for preserving autonomy. However, because cognitive tasks in HD rely on motor functions, and motor tasks demand cognitive processing, disentangling their specific impact remains a challenge.</p><p><strong>Objective: </strong>To identify the specific contribution of cognitive and motor impairments on global functional capacity, basic and instrumental activities of daily living (ADL/IADL), and work-related activities (WRA) in HD.</p><p><strong>Methods: </strong>158 HD mutation carriers, enrolled in the BioHD (NCT01412125) and RepairHD (NCT03119246) studies, were evaluated with the Unified Huntington's Disease Rating Scale and the SelfCog. The SelfCog assesses motor processing separately from memory, language, executive functions and visuospatial processing. Linear regressions were fitted to assess how functional capacity declined with motor and cognition impairments. Odds of dependence in ADLs, IADLs and WRAs were estimated using logistic regressions.</p><p><strong>Results: </strong>Cognitive and motor performance were independently associated with functional capacities, though motor performance showed a stronger association than cognitive performance. Decline of all SelfCog cognitive domains contributed to functional decline, with stronger association with global and executive scores compared to language, visuospatial, and memory domains. Higher global and executive deficits were associated with an increased risk of dependence in ADLs, IADLs, and WRAs.</p><p><strong>Conclusion: </strong>The independent contributions of motor, followed by cognitive-mainly executive-functions to functional decline suggest targeted interventions to preserve autonomy and quality of life in HD.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"224"},"PeriodicalIF":4.8,"publicationDate":"2025-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11846732/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476600","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}
引用次数: 0
Seizure freedom and therapy discontinuation in patients with idiopathic generalized epilepsy: retrospective cohort study from a tertiary epilepsy outpatient service.
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-02-22 DOI: 10.1007/s00415-025-12890-y
Davide G Curti, Anna Bellini, Marco Cursi, Jacopo Lanzone, Fabio Minicucci, Giovanna F Fanelli, Federica Agosta, Massimo Filippi
{"title":"Seizure freedom and therapy discontinuation in patients with idiopathic generalized epilepsy: retrospective cohort study from a tertiary epilepsy outpatient service.","authors":"Davide G Curti, Anna Bellini, Marco Cursi, Jacopo Lanzone, Fabio Minicucci, Giovanna F Fanelli, Federica Agosta, Massimo Filippi","doi":"10.1007/s00415-025-12890-y","DOIUrl":"10.1007/s00415-025-12890-y","url":null,"abstract":"<p><strong>Background: </strong>Idiopathic generalized epilepsy (IGE) affects young individuals and is typically successfully managed with anti-seizure medications (ASMs). Discontinuing therapy in IGE patients is a critical decision due to the risk of seizure recurrence. This study aims to identify factors influencing seizure freedom (SF) or relapse after ASM discontinuation.</p><p><strong>Methods: </strong>We retrospectively reviewed the medical records of patients seen at our clinic between 2002 and 2024. Collected data included demographics, disease history, seizure types, ASMs used, EEG findings, outcomes related to SF and ASM withdrawal.</p><p><strong>Results: </strong>We identified 322 records, with a mean age of 30 ± 12.4 years and an age at onset of 16 ± 5.9 years. On average, patients tried 1.9 ASMs, 23% on polytherapy. The main seizure types were generalized tonic-clonic seizures (GTCS) in 53.1%, myoclonic seizures in 31.7%, absences in 15.2%. SF was achieved by 76.6%. Patients with GTCS as main seizure type or presenting with GTCS in the first year of disease experienced a delayed achievement of SF. ASM discontinuation was attempted in 64 patients. Predictors of relapse after discontinuation were myoclonic and generalized seizures as principal seizure type and higher seizure frequency. Early SF and lower seizure frequency were associated with successful discontinuation. EEG predictors of discontinuation failure included worsening during treatment tapering and specific abnormalities, such as spike waves, photosensitivity, and hyperpnoea sensitivity.</p><p><strong>Conclusions: </strong>This study provides long-term follow-up data on IGE patients, highlighting key predictors of seizure control, including GTCS or myoclonic seizures and a rapid initial ASM response. EEG emerges as a valuable tool for the longitudinal monitoring of patients undergoing ASM discontinuation.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"218"},"PeriodicalIF":4.8,"publicationDate":"2025-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476593","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}
引用次数: 0
Genetic therapies for movement disorders - current status.
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-02-22 DOI: 10.1007/s00415-025-12940-5
J Sartorelli, J Ng, A A Rahim, S N Waddington, M A Kurian
{"title":"Genetic therapies for movement disorders - current status.","authors":"J Sartorelli, J Ng, A A Rahim, S N Waddington, M A Kurian","doi":"10.1007/s00415-025-12940-5","DOIUrl":"10.1007/s00415-025-12940-5","url":null,"abstract":"<p><p>Movement disorders are a group of heterogeneous neurological conditions associated with alterations of tone, posture and voluntary movement. They may either occur in isolation or as part of a multisystemic condition. More recently, the advent of next generation sequencing technologies has facilitated better understanding of the underlying causative genes and molecular pathways, thereby identifying targets for genetic therapy. In this review, we summarize the advances in genetic therapy approaches for both hyperkinetic and hypokinetic movement disorders, including Parkinson's Disease, Huntington's Disease and rarer monogenic conditions of childhood onset. While there have been significant advances in the field, multiple challenges remain, related to safety, toxicity, efficacy and brain biodistribution, which will need to be addressed by the next generation of genetic therapies.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"220"},"PeriodicalIF":4.8,"publicationDate":"2025-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11846774/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476565","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}
引用次数: 0
GAD antibodies in neurological disease: a critical evaluation of the utility and treatment implications of GAD antibodies in clinical practice.
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-02-22 DOI: 10.1007/s00415-025-12926-3
Rachel L Brown, Gilbert Thomas-Black, Hector Garcia-Moreno, Michael Chou, Zofia Fleszar, Michael S Zandi, Miles Chapman, Andrew J Church, Melanie Hart, Paola Giunti, Angela Vincent, Michael P Lunn
{"title":"GAD antibodies in neurological disease: a critical evaluation of the utility and treatment implications of GAD antibodies in clinical practice.","authors":"Rachel L Brown, Gilbert Thomas-Black, Hector Garcia-Moreno, Michael Chou, Zofia Fleszar, Michael S Zandi, Miles Chapman, Andrew J Church, Melanie Hart, Paola Giunti, Angela Vincent, Michael P Lunn","doi":"10.1007/s00415-025-12926-3","DOIUrl":"10.1007/s00415-025-12926-3","url":null,"abstract":"<p><strong>Background: </strong>The interpretation of antibodies to glutamic acid decarboxylase 65 (GAD-Abs) in neurological practice is challenging. GAD-Abs are not considered directly pathogenic and immunotherapy guidelines are lacking.</p><p><strong>Methods: </strong>We undertook a single-center retrospective service evaluation of GAD-Abs, documenting clinical features, immunotherapy responses, and outcomes of 335 patients with positive GAD-Abs measured by indirect ELISA between 2012 and 2020. The serum:CSF ratio of GAD-Ab values was used as a surrogate for intrathecal synthesis.</p><p><strong>Results: </strong>168 (50%) patients had diagnosed neurological disorders (GAD-ND). Ninety-six had neurological disorders often or sometimes associated with GAD-Abs, i.e., stiff person syndrome spectrum disorders (SPS-SD, n = 26), cerebellar ataxia (n = 21), epilepsy (n = 19), encephalitis (n = 18), or any combination of these (\"mixed\", n = 12). Seventy-two had other neurological disorders (ONDs) not typically associated with GAD-Abs. We defined a cut-off of 10,000 IU/mL a priori and a posteriori for GAD-Ab associated NDs, but identified values > 10,000 IU/mL in 21% and 11% of patients with ONDs or diabetes respectively, and < 10,000 IU/mL in 39% patients with classical GAD-Ab syndromes, indicating low assay specificity and sensitivity. Low serum: CSF GAD-Ab ratios were consistent with intrathecal synthesis in 12/19 tested; 25/54 patients had oligoclonal bands. 30/50 patients given adequate immunotherapies had partial (n = 17) or good (n = 13) responses, particularly those with SPS-SD or limbic encephalitis. Within the limitations of small subgroups and routine laboratory titrations, patients with GAD-Ab values > 10,000 IU/mL, intrathecal synthesis of GAD-Abs, or oligoclonal bands, were not more likely to improve with immunotherapies than those with GAD-Ab values < 10,000 IU/mL and a non-inflammatory CSF. Rather, treatment response correlated with disease group, principally SPS-SD and encephalitis.</p><p><strong>Conclusions: </strong>These results suggest caution in over-interpreting GAD-Abs values. Better biomarkers for identifying patients with immunotherapy responsive GAD-Ab disease are needed.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"222"},"PeriodicalIF":4.8,"publicationDate":"2025-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11846749/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476564","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}
引用次数: 0
Associations between connectivity in functional brain networks and gait speed in older adults with and without multiple sclerosis.
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-02-19 DOI: 10.1007/s00415-025-12955-y
Siddharth Nayak, Mark E Wagshul, Frederick W Foley, Robert W Motl, Roee Holtzer
{"title":"Associations between connectivity in functional brain networks and gait speed in older adults with and without multiple sclerosis.","authors":"Siddharth Nayak, Mark E Wagshul, Frederick W Foley, Robert W Motl, Roee Holtzer","doi":"10.1007/s00415-025-12955-y","DOIUrl":"10.1007/s00415-025-12955-y","url":null,"abstract":"<p><strong>Objective: </strong>To assess whether resting-state functional connectivity (RSFC) brain networks are associated with gait speed in a sample of older adults with and without multiple sclerosis (MS).</p><p><strong>Methods: </strong>Older adults with MS (OAMS: n = 82, mean age = 64.4 ± 4.1 years) and controls (n = 85, mean age = 68.6 ± 7.1 years) underwent brain MRI, cognitive assessment, and motor testing. RSFC brain networks were computed from resting-state functional scans based on a data-driven approach. The timed-25-foot-walk test (T25FW), an established measure of disability in aging and clinical populations, served as the outcome measure.</p><p><strong>Results: </strong>Analyses adjusted for confounders revealed that faster gait speed was significantly associated with higher RSFC in left fronto-parietal (p = 0.002) network in the full cohort. Among OAMS, significant associations between faster gait speed and higher RSFC were found in left fronto-parietal (p = 0.002), cerebellar (p = 0.023), and language (p = 0.046) networks. In contrast, among control participants, there were no significant associations between RSFC and gait speed.</p><p><strong>Conclusion: </strong>In aging, greater functional brain support of walking speed, operationalized using RSFC in empirically derived networks, is required in MS compared to healthy control participants.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"216"},"PeriodicalIF":4.8,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143449368","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}
引用次数: 0
Quantifying neurodegeneration within subdivisions of core motor pathways in amyotrophic lateral sclerosis using diffusion MRI.
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-02-19 DOI: 10.1007/s00415-025-12920-9
Hannes Almgren, Colin J Mahoney, William Huynh, Arkiev D'Souza, Sienna Berte, Jinglei Lv, Chenyu Wang, Matthew C Kiernan, Fernando Calamante, Sicong Tu
{"title":"Quantifying neurodegeneration within subdivisions of core motor pathways in amyotrophic lateral sclerosis using diffusion MRI.","authors":"Hannes Almgren, Colin J Mahoney, William Huynh, Arkiev D'Souza, Sienna Berte, Jinglei Lv, Chenyu Wang, Matthew C Kiernan, Fernando Calamante, Sicong Tu","doi":"10.1007/s00415-025-12920-9","DOIUrl":"10.1007/s00415-025-12920-9","url":null,"abstract":"<p><strong>Background: </strong>Diffusion MRI is sensitive to white matter changes in amyotrophic lateral sclerosis (ALS). The current study aimed to establish disease profiles across core motor pathways, and their relevance to clinical progression in ALS.</p><p><strong>Methods: </strong>Sixty-five participants (ALS = 47; Control = 18) were recruited for the study. White matter integrity of motor, somatosensory, and premotor subdivisions within the corticospinal tract and corpus callosum were quantified by fibre density, fibre-bundle cross-section, structural connectivity, and fractional anisotropy. Analyses focused on identifying diffusion metrics and tract profiles sensitive to ALS pathology, and their association with clinical progression.</p><p><strong>Results: </strong>Reduced fibre density of the motor subdivision of the corpus callosum (CC) and corticospinal tract (CST) demonstrated best performance in classifying ALS from controls (area-under-curve: CC<sub>motor</sub> = 0.81, CST<sub>motor</sub> = 0.76). Significant reductions in fibre density (CC<sub>motor</sub>: p < 0.001; CST<sub>motor</sub>: p = 0.016), and structural connectivity (CC<sub>motor</sub>: p = 0.008; CST<sub>somatosensory</sub>: p = 0.012) indicated presence of ALS pathology. Reduced fibre density & cross-section significantly correlated with severity of functional impairment (ALSFRS-R; CC<sub>motor</sub>: r = 0.52, p = 0.019; CST<sub>motor</sub>: r = 0.59, p = 0.016). The largest effect sizes were generally found for motor and somatosensory subdivisions across both major white matter bundles.</p><p><strong>Conclusion: </strong>Current findings suggest that ALS does not uniformly impact the corticospinal tract and corpus callosum. There is a preferential disease profile of neurodegeneration mainly impacting primary motor fibres. Microstructural white matter abnormality indicated presence of ALS pathology while macrostructural white matter abnormality was associated with severity of functional impairment. Quantification of white matter abnormality in corticospinal tract and callosal subdivisions holds translational potential as an imaging biomarker for neurodegeneration in ALS.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"215"},"PeriodicalIF":4.8,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11839792/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143449373","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}
引用次数: 0
Clinical, diagnostic and therapeutic challenges in CNS fungal infections: a single-center retrospective data analysis.
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-02-18 DOI: 10.1007/s00415-025-12954-z
I Masouris, M Klein, C Schichor, J Stemmler, U Koedel, H W Pfister
{"title":"Clinical, diagnostic and therapeutic challenges in CNS fungal infections: a single-center retrospective data analysis.","authors":"I Masouris, M Klein, C Schichor, J Stemmler, U Koedel, H W Pfister","doi":"10.1007/s00415-025-12954-z","DOIUrl":"10.1007/s00415-025-12954-z","url":null,"abstract":"<p><strong>Background: </strong>Central nervous system (CNS) involvement is a rare but serious complication of fungal infection with increasing incidence, especially in immunocompromised patients. Candida spp., Cryptococcus spp. Aspergillus spp. and Mucorales spp. are the most common pathogens. Despite continuous advancement, diagnosing remains challenging. This can lead to significant delays in diagnosis and therapy, thereby negatively affecting outcomes. To date, data on clinical symptoms, diagnostics and treatment of CNS fungal infections remain scarce.</p><p><strong>Methods: </strong>We retrospectively analyzed data from patients ≥ 18 years old with CNS fungal infection treated between 01/01/2007 and 31/12/2023 in the Departments of Neurology, Neurosurgery, and Oncology at the University Hospital of LMU, Munich. Data included biometrical data, clinical symptoms, laboratory and microbiological results, brain imaging, treatment, and clinical course.</p><p><strong>Results: </strong>58 patients with CNS fungal infections were identified. Our key findings were: (1) the most common fungus was Aspergillus spp, followed by Cryptococcus spp, Mucoracae spp and Candida spp; (2) clinical symptoms and immunosuppressive factors varied between pathogens; (3) candidiasis and cryptococcosis mostly manifested as meningitis, while mucormycosis and aspergillosis presented as mass lesions; (4) time to diagnosis was longer for Candidiasis patients than for other fungal infections; (5) antifungal regimens varied among and within each CNS fungal infection; (6) two-year-survival was substantial for candidiasis and cryptococcosis but worse for aspergillosis and mucormycosis.</p><p><strong>Conclusions: </strong>Overall, patients with clinical suspicion of neuroinfection and neurologic deficits should be examined for fungal infections, especially if immunocompromised. Given their increasing frequency, CNS fungal infections will become more relevant in daily neurological practice.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"214"},"PeriodicalIF":4.8,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143441097","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}
引用次数: 0
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