Balazs Lorincz, Michal Vrablik, Ramanathan Murali, Eva Kubala Havrdova, Dana Horakova, Jan Krasensky, Manuela Vaneckova, Tomas Uher
{"title":"Lipid and brain volumetric measures in multiple sclerosis patients: findings from a large observational study","authors":"Balazs Lorincz, Michal Vrablik, Ramanathan Murali, Eva Kubala Havrdova, Dana Horakova, Jan Krasensky, Manuela Vaneckova, Tomas Uher","doi":"10.1007/s13760-024-02676-w","DOIUrl":"10.1007/s13760-024-02676-w","url":null,"abstract":"<div><h3>Objectives</h3><p>This study aimed to investigate relationships between cholesterol profile, brain volumetric MRI, and clinical measures in a large observational cohort of multiple sclerosis (MS) patients.</p><h3>Materials and methods</h3><p>We included 1.505 patients with 4.966 time points including complete lipid, clinical, and imaging data. The time among lipid, brain MRI and clinical measures was under 90 days. Cross-sectional statistical analysis at baseline was performed using an adjusted linear regression and analysis of longitudinal lipid and MRI measures data was performed using adjusted linear mixed models.</p><h3>Results</h3><p>We found associations between higher high-density lipoprotein cholesterol (HDL-C) and lower brain parenchymal fraction (BPF) at cross-sectional analysis at baseline (B = −0.43, CI 95%: −0.73, −0.12, <i>p</i> = 0.005), as well as in longitudinal analysis over follow-up (B = −0.32 ± 0.072, χ<sup>2</sup> = 36.6; p = < 0.001). Higher HDL-C was also associated with higher T2-lesion volume in longitudinal analysis (B = 0.11 ± 0.023; χ<sup>2</sup> = 23.04; p = < 0.001). We observed a weak negative association between low-density lipoprotein cholesterol (LDL-C) levels and BPF at baseline (B = −0.26, CI 95%: −0.4, −0.11, p = < 0.001) as well as in longitudinal analysis (B = −0.06 ± 0.03, χ<sup>2</sup> = 4.46; <i>p</i> = 0.03). T2-LV did not show an association with LDL-C. We did not find any association between lipid measures and disability. The effect of lipid levels on MRI measures and disability was minimal (Cohen f2 < 0.02).</p><h3>Conclusions</h3><p>Our results contradict the previously described exclusively positive effect of HDL-C on brain atrophy in patients with MS. Higher LDL-C was weakly associated with higher brain atrophy but not with higher lesion burden.</p></div>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":"124 6","pages":"1981 - 1988"},"PeriodicalIF":2.0,"publicationDate":"2024-10-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s13760-024-02676-w.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142492758","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}
Luis Rueda-Carrillo, Mingyu Li, Klepper A Garcia, Manan Shah, Nilufer Yalcin
{"title":"Pupillary changes in pentobarbital coma mimicking a neurocritical care emergency.","authors":"Luis Rueda-Carrillo, Mingyu Li, Klepper A Garcia, Manan Shah, Nilufer Yalcin","doi":"10.1007/s13760-024-02674-y","DOIUrl":"https://doi.org/10.1007/s13760-024-02674-y","url":null,"abstract":"","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142492759","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}
Miguel Schön, Catarina Falcão Campos, Ana Patrícia Antunes, Luísa Albuquerque, Isabel Conceição
{"title":"Severe progression of a young-onset non-paraneoplastic Lambert-Eaton myasthenic syndrome.","authors":"Miguel Schön, Catarina Falcão Campos, Ana Patrícia Antunes, Luísa Albuquerque, Isabel Conceição","doi":"10.1007/s13760-024-02657-z","DOIUrl":"https://doi.org/10.1007/s13760-024-02657-z","url":null,"abstract":"<p><p>The Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare neuromuscular disorder characterized by proximal muscle weakness, hyporeflexia or areflexia, and dysautonomia. Ocular and bulbar symptoms may also occur, though respiratory failure is uncommon; we report the case of a 21-year-old woman diagnosed with LEMS, without evidence of a tumor, who was initially treated with symptomatic medication, immunoglobulin, and steroids, resulting in significant clinical improvement. However, she later developed psychotic symptoms, prompting the discontinuation of steroids. Brain MRI and antineuronal antibody tests were negative. Subsequently, her condition deteriorated, leading to respiratory distress that required urgent intubation, and prolonged dysphagia that necessitated the insertion of a gastrostomy tube for nutrition, along with the maintenance of a tracheostomy. Plasmapheresis was performed, resulting in partial motor recovery. Rituximab was then introduced, leading to sustained improvement in her neuromuscular symptoms, although her neuropsychiatric symptoms persisted; this case highlights a severe progression of young-onset LEMS, marked by prominent bulbar dysfunction and respiratory distress. Neuromuscular improvement followed rituximab treatment, while the concurrent psychotic symptoms appeared to follow an independent course, suggesting a primary psychiatric comorbidity.</p>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142492761","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":"Upper extremity functions, spinal posture, and axial rigidity in patients with parkinson's disease.","authors":"Buse Korkmaz, Mustafa Ertuğrul Yaşa, Rıza Sonkaya","doi":"10.1007/s13760-024-02656-0","DOIUrl":"https://doi.org/10.1007/s13760-024-02656-0","url":null,"abstract":"<p><strong>Objective: </strong>Upper extremity dysfunction is frequently seen in Parkinson's disease (PD). Existing research has shown that bradykinesia, which is main symptom of PD, is primarily responsible but the combined effects of spinal posture and axial rigidity on upper extremity functions were not investigated yet. The aim of this study was to investigate upper extremity functions in patients with PD and to evaluate relationship of these with spinal posture and axial rigidity.</p><p><strong>Methods: </strong>This prospective controlled study included 40 patients with PD and 40 healthy controls. Upper extremity function was measured with the 9-Hole Peg Test. Spinal posture and axial rigidity were measured with a Spinal Mouse.</p><p><strong>Results: </strong>Compared with the control group, a decrease in upper extremity functions (p < 0.001), decreased lumbar lordosis (p = 0.003), and posterior sacral tilt (p = 0.021) were determined in patients' group. Thoracic and lumbar mobility in the sagittal (all p < 0.001) and frontal planes (p = 0.004, p < 0.001) was found to be reduced in the patient group. A correlation was determined between upper extremity functions and lumbar mobility in the sagittal (p = 0.022, r= -0.362) and frontal planes (p = 0.045, r= -0.319) and lumbar lordosis (p = 0.048, r = 0.302).</p><p><strong>Conclusions: </strong>The results of this study demonstrated that altered spinal posture and increased axial rigidity were related with decreased upper extremity functions in patients with PD. There is a need for further studies to investigate effect of trunk-based therapies on upper extremity function in patients with PD.</p>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142455449","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":"Cultural adaptation, validity and reliability of the Turkish version of north star ambulatory assessment.","authors":"Güllü Aydın-Yağcıoğlu, Numan Bulut, Fatma Uğur, Öznur Yılmaz, İpek Alemdaroğlu-Gürbüz","doi":"10.1007/s13760-024-02670-2","DOIUrl":"https://doi.org/10.1007/s13760-024-02670-2","url":null,"abstract":"<p><strong>Purpose: </strong>The North Star Ambulatory Assessment (NSAA) is a functional motor outcome measure originally developed for patients with Duchenne muscular dystrophy (DMD). The aim of this study was to perform the cultural adaptation and investigate the validity and reliability of the Turkish version of the NSAA (T-NSAA) in DMD.</p><p><strong>Methods: </strong>After translation process, internal consistency, interrater and test-retest reliability of the NSAA were determined by using the Cronbach Alpha Coefficient and Intraclass Correlation Coefficient (ICC), respectively. Absolute reliability was determined by using the Standard Error of Measurement (SEM) with minimal detectable change at 95% limits of confidence (MDC<sub>95</sub>). Lower limb functionality of children was evaluated by Vignos Lower Extremity Scale (VLERS). To establish convergent validity, the correlations between T-NSAA and Motor Function Measure (MFM-32), 6-minute walk test (6-MWT), and VLERS were analyzed by using the Spearman's correlation coefficient.</p><p><strong>Results: </strong>The study was completed with 86 patients with DMD whose age were mean 104.56 ± 24.66 months. The internal consistency (Cronbach's α = 0.94), intra-reliability (ICC = 0.977) and inter-reliability (ICC = 0.972) of T-NSAA were excellent. SEM and MDC values were low indicating satisfactory absolute agreement (< %10). The T-NSAA had strong correlations with the MFM-total score, 6-MWT, and VLERS (p < 0.01).</p><p><strong>Conclusion: </strong>T-NSAA is a valid and reliable tool to assess ambulatory status of Turkish-speaking DMD population.</p><p><strong>Clinical trial number: </strong>NCT05549999, Date of registration: September 19, 2022.</p>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142455529","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}
Federico Marrama, Alfredo Paolo Mascolo, Fabrizio Sallustio, Mario Bovino, Alessandro Rocco, Federica D'Agostino, Valerio Da Ros, Daniele Morosetti, Francesco Mori, Giordano Lacidogna, Ilaria Maestrini, Fana Alemseged, Valentina Panetta, Marina Diomedi
{"title":"Safety and efficacy of bridging intravenous thrombolysis plus mechanical thrombectomy versus direct mechanical thrombectomy in different age groups of acute ischemic stroke patients.","authors":"Federico Marrama, Alfredo Paolo Mascolo, Fabrizio Sallustio, Mario Bovino, Alessandro Rocco, Federica D'Agostino, Valerio Da Ros, Daniele Morosetti, Francesco Mori, Giordano Lacidogna, Ilaria Maestrini, Fana Alemseged, Valentina Panetta, Marina Diomedi","doi":"10.1007/s13760-024-02672-0","DOIUrl":"https://doi.org/10.1007/s13760-024-02672-0","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the safety and efficacy of bridging intravenous thrombolysis plus mechanical thrombectomy (MT) versus direct MT in different age groups of patients with acute ischemic stroke (AIS) secondary to large vessel occlusion (LVO) of the anterior circulation.</p><p><strong>Methods: </strong>Consecutive patients from the prospective endovascular stroke registry of the Comprehensive Stroke Center, University of Rome Tor Vergata, Italy, between January 2015 and June 2021 were retrospectively analyzed. Patients were divided into age groups (≤ 80 years old and > 80 years old); for each age group, they were further divided in the bridging therapy group and the direct MT group. We performed a propensity score analysis according to baseline characteristics. Safety outcomes were any intracerebral hemorrhage (ICH), symptomatic ICH (sICH) and 3-month mortality. Efficacy outcomes were successful recanalization (modified Thrombolysis in Cerebral Infarction, mTICI, score ≥ 2b) and 3-month functional independence (modified Rankin Scale, mRS, ≤ 2).</p><p><strong>Results: </strong>We included 761 AIS patients with anterior circulation LVO (mean age 73.5 ± 12.8 years; 44.8% males; mean baseline NIHSS 16 ± 5). After propensity score, there were 365 patients ≤ 80 years old (52% bridging therapy) and 187 patients > 80 years old (57% bridging therapy). In both age groups of patients, we found no statistically significant differences in the rates of any ICH, sICH, successful recanalization and 3-month mortality and functional independence between bridging therapy and direct MT groups.</p><p><strong>Conclusion: </strong>In our population, safety and efficacy outcomes of bridging therapy versus direct MT did not differ in both AIS patients ≤ 80 and > 80 years old.</p>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142455448","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}
Berkay Paker, Önder Ertem, Mehmetzeki Yıldız, Deniz Konya
{"title":"Adult-onset intramedullary teratomas: systematic review with outcome analysis.","authors":"Berkay Paker, Önder Ertem, Mehmetzeki Yıldız, Deniz Konya","doi":"10.1007/s13760-024-02667-x","DOIUrl":"https://doi.org/10.1007/s13760-024-02667-x","url":null,"abstract":"<p><strong>Background and objective: </strong>Intramedullary mature teratomas in adults are rare tumors that pose significant diagnostic and therapeutic challenges due to their diverse tissue composition and intramedullary location. This review, which includes an illustrative case, aims to provide a comprehensive overview of the clinical presentation, diagnosis, treatment, and outcomes of adult intramedullary mature teratomas through a systematic review of 89 cases.</p><p><strong>Methods: </strong>A systematic review was conducted following PRISMA guidelines, using keyword combinations in PubMed, MEDLINE, and Web of Science databases until June 2024. Inclusion criteria were adult patients (> 18 years) with histopathologically confirmed intramedullary mature teratomas. Statistical analyses examined relationships between the extent of resection (EoR), tumor location, and patient outcomes.</p><p><strong>Results: </strong>Among the 89 cases, the mean age was 39.94 ± 13.52 years, with a male-to-female ratio of 1.39:1. Tumors were most commonly located in the conus region (51.2%). Surgical resection was the primary treatment, with 43 cases undergoing partial resection and 33 gross total resection. No statistically significant differences in outcomes were found between EoR, sex, associated anomalies, or specific outcomes. However, younger patients showed improved outcomes for lower limb weakness and sphincter dysfunction. Recurrence was noted in three cases, all located in the conus.</p><p><strong>Conclusion: </strong>Intramedullary mature teratomas require a nuanced approach that balances complete resection and neurological preservation. Early diagnosis and individualized surgical planning are crucial for optimizing outcomes. Despite the challenges, effective management is achievable, and ongoing research is essential to refining treatment strategies for this rare tumor.</p>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142455526","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}
Hansashree Padmanabha, Nagarathna Chandrashekar, Dhaval Shukla, M Pooja, Rohan Mahale
{"title":"Cerebral abscesses due to Pseudallescheria boydii mycoses: a diagnostic and therapeutic conundrum.","authors":"Hansashree Padmanabha, Nagarathna Chandrashekar, Dhaval Shukla, M Pooja, Rohan Mahale","doi":"10.1007/s13760-024-02661-3","DOIUrl":"https://doi.org/10.1007/s13760-024-02661-3","url":null,"abstract":"","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142455527","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":"From cerebellum to skeleton: a case report of SHH-activated medulloblastoma with extraneural spread.","authors":"Sami Marzouki, Nando De Vulder, Sven Dekeyzer","doi":"10.1007/s13760-024-02663-1","DOIUrl":"https://doi.org/10.1007/s13760-024-02663-1","url":null,"abstract":"","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142455530","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}