Acta Neurologica Scandinavica最新文献

Objective: Delayed emergency responses in patients with large vessel occlusion stroke (LVOS) are associated with reduced access to timely reperfusion therapy and worse clinical outcomes. The present study was aimed at identifying modifiable factors contributing to delays before hospital arrival in LVOS patients undergoing endovascular treatment (EVT).

Methods: In this retrospective analysis of prospectively collected data, consecutive acute LVOS patients undergoing EVT at two comprehensive stroke centers between December 2020 and December 2021 were enrolled. Neurologists administered a standardized questionnaire to patients or their caregivers within 24 h after the procedure. Emergency response delay was defined as onset to groin (OTG) time, measured from symptom onset or last known normal to groin puncture, exceeding 6 h. Baseline characteristics, process times, and clinical data were collected for all enrolled patients, and factors influencing the emergency process and outcomes were analyzed.

Results: Of the 366 patients initially considered, 14 with in-hospital stroke were excluded, leaving 352 patients for analysis. The median age was 70 years (63, 76), and 135 patients (38.4%) experienced treatment delays. The median National Institutes of Health Stroke Scale (NIHSS) score was 14 (11, 18), and the median Alberta Stroke Program Early CT Score (ASPECTS) was 9 (7.85, 10). Multivariate analysis identified the main modifiable factors associated with reduced emergency response delay as early calling of emergency services (odds ratio [OR] = 0.41, 95% confidence interval [CI]: 0.22–0.76), initial consultation with a neurologist (OR = 0.35, 95% CI: 0.20–0.62), and stroke awareness (OR = 0.51, 95% CI: 0.29–0.89). Among elderly patients and those whose stroke onset occurred during sleep, early contact with emergency services (120) significantly reduced prehospital delays (OR = 0.48, 95% CI: 0.21–0.94 and OR = 0.30, 95% CI: 0.10–0.86).

Conclusion: Emergency physician involvement, stroke awareness, and early calling of emergency services (120) are modifiable factors that can reduce delays in the emergency response process. For patients eligible for EVT, minimizing prehospital delays may require prioritizing both community education on stroke recognition and system-level improvements to ensure rapid emergency activation and timely neurological assessment.

Introduction: This study is aimed at investigating brain injury biomarkers neurofilament light (NfL), tau, neuron-specific enolase (NSE), calcium-binding protein S100B (S100B) and glial fibrillary acidic protein (GFAP) in blood during general anaesthesia and abdominal surgery in patients without cerebral injury, to evaluate the effect of general anaesthesia and surgery per se on the release of these biomarkers.

Methods: This prospective observational study was conducted at Sahlgrenska University Hospital, Gothenburg, Sweden, between September and November 2021. Patients scheduled for mixed abdominal surgery under general anaesthesia were included. Vital parameters and near-infrared spectroscopy (NIRS) for cerebral perfusion were continuously monitored. Blood pressure was kept close to each patients’ preanaesthetic mean arterial pressure. Vasopressors and fluids were administered at the discretion of the attending physician, not influenced by the study.

Results: There were 23 patients (11 females [48%] and 12 males [52%]) included in the study. NfL, tau, NSE and S100B increased significantly when 2- and 24-h concentrations were compared with preoperative values, whilst GFAP did not. The continuous mean arterial blood pressure was 83.5 mmHg, with a 62.2–90.4 mmHg range. The mean NIRS was 77.5% (range 62.2–90.4). No patient had a drop in NIRS of 12% or more. Postoperative symptoms of confusion or neurological deficits were not observed in any patient within 48 h from the start of anaesthesia.

Conclusion: General anaesthesia and abdominal surgery in patients with well-maintained cerebral perfusion and no clinical signs of postoperative cerebral injury caused an increase in levels of brain injury biomarkers NfL, tau, NSE and S100B in blood. Interestingly, there was no increase in levels of GFAP in the blood. These data suggest that GFAP is the only biomarker, amongst the investigated biomarkers, which is not released into the bloodstream during general anaesthesia and surgery in patients with no suspected brain injury. More extensive studies on this subject are warranted.

Trial Registration: ClinicalTrials.gov identifier: NCT03919370.

Background: Tuberous sclerosis complex (TSC) is a genetic disorder commonly associated with drug-resistant epilepsy. Although epileptogenic tubers (ETs) can be localized in 60% of TSC patients, approximately 40% remain undetectable despite comprehensive multimodal evaluations. The functional network mechanisms underlying seizure generation and propagation in patients with TSC are poorly understood.

Methods: Resting-state fMRI (rs-fMRI) data from 10 surgically treated patients with TSC (postoperative seizure freedom for ≥ 3 years) and 10 age-matched healthy controls were analyzed. Functional connectivity (FC) between four thalamic subregions—mediodorsal thalamus (MDT), anterior thalamic nucleus (ANT), centromedian thalamus (CMT), and pulvinar—and ETs, non-ETs, or normal cortices was assessed. Secondary projection analysis mapped corticothalamic networks associated with ETs.

Results: MDT-ET connectivity was significantly reduced compared with MDT-non-ETs (p = 0.01) and MDT-normal cortices in controls (p = 0.03). Secondary analysis identified hyperconnectivity between ET-associated thalamic clusters and the left middle frontal gyrus (p_GFR < 0.001). No significant differences were observed in other thalamic subregions.

Conclusions: The selective reduction in MDT-ET connectivity highlights disrupted thalamocortical synchronization as a key network mechanism in TSC-related epilepsy. Enhanced left middle frontal gyrus–thalamic connectivity suggests compensatory cortical engagement within epileptogenic networks. These findings position rs-fMRI as a critical tool for delineating network-based biomarkers, advancing precision therapeutic strategies in TSC.

Background: While clinical trials have shown no differences between monthly and quarterly regimens of fremanezumab, limited real-life data exist for comparison. This study is aimed at comparing treatment regimens in real life.

Methods: This observational, multicentre study conducted a retrospective analysis of patients initiating monthly or quarterly fremanezumab. Primary endpoints were the comparison of monthly migraine days’ reduction, adverse effects, and treatment discontinuation rates at 3 and 6 months. Secondary endpoints included changes in headache and medication intake frequencies, response rates, and patient-reported outcomes.

Results: One hundred and eleven patients were included, with a median age of 48.5 years, 91% women, and 54.1% with chronic migraine. Sixty-four patients received a monthly regimen and 47 a quarterly. Baseline characteristics were similar. Reductions in monthly migraine days did not differ between treatment regimens (−5 [IQR −9, −1] for monthly versus −6 [IQR −8, −3] for quarterly at 3 months, p = 0.867, and −5 [IQR −10, −2] versus −5.5 [IQR −8.5, −3] at 6 months, p = 0.666, respectively). Adverse effects and discontinuation rates were similar between groups. Secondary endpoints were comparable, except for a higher PGIC scale for the quarterly group at 6 months (6 [IQR 4–6] versus 4 [IQR 2–6], p = 0.007). No differences were observed in the subgroup analysis of episodic or chronic migraine.

Conclusions: Monthly and quarterly fremanezumab demonstrated comparable effectiveness, tolerability, and adherence in real life. Quarterly regimen may result in a more favorable global impression of change.

Background/Aim: Moyamoya disease, a rare cerebrovascular disorder first identified in Japan, is characterised by spontaneous occlusion of the circle of Willis and is a significant cause of ischaemic and haemorrhagic strokes. When associated with autoimmune disorders like antiphospholipid syndrome, it is referred to as moyamoya syndrome. This study reviews the clinical features of moyamoya syndrome in patients with antiphospholipid antibody positivity, with and without antiphospholipid syndrome, focusing on clinical differences, treatments and outcomes.

Methods: To identify relevant studies, a comprehensive systematic literature review search was conducted using the terms ‘Moyamoya’, ‘antiphospholipid’, ‘anticardiolipin antibodies’, ‘anti-beta2-glycoprotein I antibodies’ and ‘lupus anticoagulant’.

Results: Twelve cases of moyamoya syndrome with antiphospholipid antibody positivity were reviewed. Eight met antiphospholipid syndrome criteria with diverse antibody profiles. Treatments included antiplatelet therapy, anticoagulants and bypass surgery. While eight patients experienced no recurrent strokes during follow-up, three had recurrent strokes, and two died from haemorrhagic events. Individualised management was crucial for balancing treatment benefits and risks.

Discussion: Moyamoya disease involves internal carotid artery stenoses, also seen in antiphospholipid syndrome. While the connection between moyamoya disease and antiphospholipid antibody positivity remains unclear, antiphospholipid syndrome should be ruled out during diagnosis. Surgical treatments are less frequent in antiphospholipid antibody-positive patients than in general moyamoya disease. Recurrent cerebrovascular events under medical treatment highlight the potential need for broader surgical interventions in these patients.

Conclusion: The limited number of reported cases restricts the generalisability of the current findings and calls for cautious interpretation. This underlines the need for further multicentre studies and randomised trials to optimise therapeutic strategies, elucidate the role of antiphospholipid antibodies and establish evidence-based guidelines for the management of moyamoya syndrome and its variants.

Introduction: Ocrelizumab (OCR) and rituximab (RTX) are monoclonal antibodies targeting CD20 on B cells, a promising approach for relapsing–remitting multiple sclerosis (RRMS) and primary progressive MS (PPMS). They aim to modulate the immune system and reduce B cells, potentially leading to fewer relapses and delayed disease progression. Xacrel, the Iranian-made OCR biosimilar, requires further investigation for its effectiveness in MS treatment. The Timed 25-Foot Walk (T25FW) has been one of the key implements for assessing mobility in MS patients for over two decades, and recent studies confirmed that comprehensive treatment—especially with fampridine and OCR—significantly improves T25FW performance.

Objective: We aim to assess the effectiveness of Xacrel (Iranian OCR) for MS treatment by evaluating alteration in Expanded Disability Status Scale (EDSS) score and T25FW test. This study also explores the potential benefits of switching patients’ drug from RTX to OCR.

Material and Methods: This prospective cohort study at Qaem Hospital (February 2022–May 2024) on 143 MS patients evaluates Xacrel in MS patients using EDSS and T25FW scores before treatment and at 6 and 12 months posttreatment. Additionally, we assessed 29 MS patients whose drug transitioned from RTX to OCR to compare the effectiveness of these treatments. For this purpose, MS progression was assessed using the EDSS score and T25FW test at baseline, 6 months, and 12 months after switching their medication.

Results: In our study, the average age was 38.48 ± 8.73 years, and over 70% were women. 76.2% were between 30–50 years old, with a mean disease duration of 6 years. About 19.6% were treatment-naive, with dimethyl fumarate as the most common first-line drug. Over 12 months, significant declines in EDSS scores and increases in T25FW tests were noted at 6 and 12 months compared to baseline (all p < 0.05), but not between 6 and 12 months. Significant factors were RRMS for 6-month EDSS score changes (p = 0.011) and treatment-naive patients for T25FW at 6 months (p = 0.018) and 12 months (p = 0.004). Switching from RTX to OCR showed no significant changes in EDSS or T25FW scores, despite trends of decreases in EDSS and increases in T25FW times at 6 and 12 months. Subgroup analyses by gender, age, disease duration, type, and previous medication history showed no significant differences.

Conclusion: In MS patients—particularly treatment-naive individuals and those with RRMS—Xacrel (an Iranian-produced biosimilar of OCR) effectively inhibited EDSS progression, significantly reduced EDSS scores, and enhanced T25FW performance. In contrast, switching from RTX to Xacrel did not result in significant changes in mobility outcomes or disability status.

Introduction: Patients with Parkinson’s disease (PD) show impairments of motor and perceptual learning; however, their abilities to apply and generalize skills learned in one condition to new situations (learning transfer) remain unclear. Herein, we investigated motor and perceptual learning transfer abilities and their underlying neural substrates in patients with PD.

Materials and Methods: Forty-four patients with PD and 42 healthy controls (NCs) were investigated. Motor learning transfer ability (MLTA) and perceptual learning transfer ability (PLTA) were assessed immediately after learning (i.e., in the early phase) and were defined, respectively, as the ability to adapt to angle changes in a reaching task with rotation perturbation and to adapt to domain changes during a categorization task. Additionally, late-phase retention of motor learning and transfer performance were evaluated the day after the early-phase assessment.

Results: MLTA and PLTA in the early phase, as well as retention and transfer performance of motor learning in the late phase, were more impaired in patients with PD than in NCs. In the early phase, the MLTA score was significantly positively correlated with the PLTA score and the number of categories correctly answered in the Modified Card Sorting Test (MCST_categories) in patients with PD. In the late phase, the transfer performance of motor learning was significantly positively associated with the degree of motor learning retention. The MLTA score, PLTA score, and MCST_categories value were all positively correlated with blood flow in the right inferior parietal lobule (IPL).

Discussion: Dysfunction of the right IPL region in patients with PD may be associated with impairments in early-phase motor and perceptual learning transfer, as well as poor MCST performance. Furthermore, late-phase motor learning transfer performance may depend on long-term (24 h) motor learning retention.

Objective: This study is aimed at developing a differential diagnostic model for Guillain–Barré syndrome (GBS) from other central nervous system diseases based on clinical and laboratory indicators.

Materials and Methods: A retrospective approach was conducted for the GBS patients and patients with other neurological diseases (non-GBS group, including viral encephalitis, peripheral neuropathy, multiple sclerosis, transverse myelitis, neuromyelitis optica spectrum disorders, and myasthenia gravis). The least absolute shrinkage and selection operator (LASSO) technique was integrated with multivariable logistic regression to perform predictor selection. The logistic regression model was established as the predictive framework, followed by the application of the Shapley additive explanation (SHAP) framework to quantify contributions of selected variables within the model. After that, patient data were collected for model validation.

Results: A total of 161 patients with GBS and 644 patients with non-GBS diseases were enrolled. Upper limb weakness, visual impairment, areflexia, hyperreflexia, total bilirubin (TBIL), mean corpusular hemoglobin (MCH), platelet large cell ratio (P-LCR), cerebral spinal fluid–protein (CSF-protein), dyslipidemia index, and oligoclonal band-serum/cerebral spinal fluid (SOB-CSF) emerged as independent predictors of GBS development. The logistic regression classifier demonstrated robust predictive performance, achieving an area under the curve (AUC) of 0.915 in the testing set, with an accuracy of 0.876, sensitivity of 0.823, and specificity of 0.889.

Conclusion: We developed and validated a logistic regression model incorporating multiple clinical indicators to differentiate GBS from other inflammatory neurological disorders (including MS, NMOSD, MG, TM, VE, and PN). The model demonstrated high diagnostic accuracy (AUC 0.92), supporting its potential as a supplementary tool for clinical decision-making.

Aim: This study is aimed at determining the prevalence of patients with Type 2 diabetes mellitus (T2DM) affected by abnormal ventricular enlargement (normal pressure hydrocephalus).

Materials and Methods: Data were collected on diabetic patients from an outpatient diabetology clinic and nondiabetic patients who underwent brain CT or MRI, including sex, age, diabetes onset date, fasting blood glucose, HbA1c, Evans Index, and the time between diabetes diagnosis and neuroimaging. The metabolic profile was assessed by BMI, blood pressure, waist circumference, glomerular filtration rate, total cholesterol, LDL, HDL, and triglycerides. The use of ongoing therapies was recorded and categorized by drug class. Common comorbidities, such as ischemic heart disease, diabetic retinopathy, renal failure, and carotid atherosclerosis, were also documented.

Results: Neuroradiological data were available for 272 diabetic patients (mean age: 71.6 ± 11.2 years) and 275 nondiabetic patients (mean age: 70.4 ± 12.5 years). Pathological ventricular enlargement was identified in 116 of 547 individuals (21%), with a higher prevalence among males (68%; p = 0.002). Ventricular enlargement was noted in 25% of diabetic patients and 17.1% of nondiabetic patients (p = 0.02). Diabetic patients with ventricular enlargement were significantly older (mean age: 76.9 years vs. 69.4 years) than those without enlargement (p = 0.01). The duration of diabetes was also significantly longer in patients with enlargement (mean: 16.5 years) compared to those without (p = 0.009). Age, male sex, longer diabetes duration, history of ischemic heart disease, beta-blocker use, and antiplatelet therapy were significantly associated with a pathological Evans Index. In multivariate analysis, antiplatelet therapy was the strongest predictor of abnormal ventricular enlargement in T2DM patients after adjusting for confounding factors (p = 0.01).

Conclusion: There is a high prevalence of pathological ventricular enlargement in patients with T2DM. Advanced age, male sex, longer disease duration, and the use of antiplatelet therapy are significantly associated with abnormal ventricular enlargement.

Objectives: The study’s objective is to assess long-term experience with rechargeable (r-IPG) and nonrechargeable implant pulse generators (nr-IPGs) for deep brain stimulation (DBS) in Parkinson’s disease (PD).

Material and Methods: Qualitative semistructured interviews, clinical outcomes, and care load estimations were retrospectively collected for a PD-DBS population implanted at our center from 2006 to 2022.

Results: Thirty-seven nr-IPG patients (follow-up 85.3 ± 32.0 months) and 43 r-IPG patients (follow-up 73.1 ± 7.7 months) were analyzed. Long-term satisfaction was sustained in both groups (100% of r-IPG carriers and 75.7% of nr-IPGs, p = 0.001). In r-IPGs, 97.7% recharged the battery easily, and recharging time did not impact everyday life. The percentage of malfunctioning problems (32.6%) in the r-IPG group was in line with previous observations on short-term follow-ups. The size of the IPG was considered too big for 16.2% and 4.2% for nr-IPGs and r-IPGs (p = 0.086), and concerns of interventions for IPG replacements were still present in the nr-IPG group (48.6%). The total amount of days of hospitalization (19.6 ± 9.9 vs. 9.3 ± 4.8, p < 0.001) and the number of complications after the first implant (13 vs. 5, p < 0.05) and during subsequent admissions for IPG substitutions (4 vs. 0, p < 0.05) were higher for the nr-IPGs.

Conclusions: The overall level of long-term satisfaction with IPGs is consistent over time regardless of type. R-IPGs reported no discomfort with recharging even in the long-term evaluation. IPG replacement surgeries and sizes are still a concern, especially for the nr-IPG carriers, but did not affect a high level of sustained satisfaction. Resource burden remains higher for nr-IPGs even in the long term.