Neurological Sciences最新文献

Objective: The widespread use of botulinum toxin has been accompanied by an increase in complications, including iatrogenic botulism (IB). This study investigated repetitive nerve stimulation (RNS) findings in IB and myasthenia gravis (MG) patients.

Methods: Clinical and neurophysiological data were retrospectively reviewed in IB patients negative for both acetylcholine receptor and muscle-specific kinase antibodies, and in MG patients who were positive for either. RNS was performed on the ulnar nerve at 2, 3, 5, and 50 Hz, and on the facial and spinal accessory nerves at 2, 3, and 5 Hz.

Results: Fifteen IB and 23 MG patients were included. Compound muscle action potential (CMAP) amplitudes of the ulnar and facial nerves were lower in IB (p = 0.014, p = 0.006). A progressive decrement at low-frequency RNS occurred in 60% of IB and 17% of MG patients. At 5 Hz facial RNS, 1st-2nd (p = 0.044) and 1st-4th (p = 0.008) decrements were greater in MG, whereas the 4th-10th decrement was higher in IB (p = 0.031). Ulnar CMAP changes at 50 Hz were greater in IB (p < 0.001); nine patients showed increments > 40%, four > 60%, and one > 100%, while none of the MG patients exceeded 40%.

Conclusion: Reduced CMAP amplitude, an incremental response at high frequency, and a progressive decrement at low frequency were more commonly encountered in IB. In MG, a U-shaped pattern at low frequency and a more pronounced decrement between the 1st and 2nd responses were noted.

Introduction: The percentage of women neurologists is steadily increasing globally. Nevertheless women still face lower and slower levels of career progression and are less likely to reach leadership positions. In order to obtain a better understanding of the gender gap in the area of neurology in Italy, the Gender Equality Committee of the Italian Society of Neurology (SIN) conducted a survey among the society members.

Methods: Members of the SIN were invited to complete an anonymous online survey. The questions were grouped into different sections covering work environment, research, academic career, scientific societies, work/life balance and gender equality perception. The questions were structured using Likert-type scales.

Results: A total of 237 subjects (64.1% women; mean age 42.5 ± 13.6 years), representing 6.4% of the SIN members, participated in the survey. A significantly higher percentage (p < 0.0001) of women than men reported that gender influences career progression (51.5% vs 5%), academic career (91.8% vs 57.1%), access to research facilities (37.5% vs 17.1%), authorship of scientific articles (34.2% vs 8.6%), selection of contributions and speakers within scientific societies (37.9% vs 16.2%), and work-life balance (76% vs 37.5%). Overall, women felt that gender equality was poor in all the different sections.

Conclusions: In Italy women neurologists face a wide range of gender disparities affecting their professional and personal lives in the hospital and academic settings. Even if the low response rate could have affected the results, improving gender equality should be a priority in the near future.

Introduction: Nocturnal hypokinesia (NH) (i.e., reduced bed mobility) is common in people with Parkinson's Disease (PwPD), significantly affecting sleep quality. Physiotherapy showed encouraging results to treat this symptom but was poorly investigated. Moreover, telerehabilitation could be useful to increase treatment capillarity and to overcome logistic limitations. In this pilot study we investigated preliminary feasibility, safety and effectiveness of a telerehabilitation program in mild-to-moderate PwPD with NH.

Materials and methods: Sixteen PwPD with disease stage < 4, presence of NH and no cognitive impairment, were enrolled and 12 completed the study. Participants underwent a 6-week telerehabilitation program though a digital remote video call platform. The rehabilitation program included progressive exercises performed during both remote supervised sessions with a physiotherapist and self-conducted sessions through video-tutorials. PwPD were evaluated before (T0) and after the program (T1) using the Movement Disorder Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS) parts I-IV, Parkinson's disease questionnaire 39 (PDQ-39), Parkinson's Disease sleep Scale (PDSS2), Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS) and Nocturnal Hypokinesia Questionnaire (NHQ).

Results: Dropout rate was 25%. We found a significant improvement in PSQI score (p = 0.039, median difference: 2) and in item 2.9 of the MDS-UPDRS part II assessing NH (p = 0.026; median difference: 1). A tendency toward significance was found in NHQ score (p = 0.069). No significant adverse events were reported during treatment.

Conclusion: Our pilot study showed that telerehabilitation could be a feasible, safe and effective option to improve NH and sleep quality in mild-to-moderate PwPD.

Rasmussen's Encephalitis (RE) is a rare clinical syndrome of chronic progressive unihemispheric cortical inflammation and atrophy, with explosive focal onset seizures which are commonly intractable and may include epilepsia partialis continua (EPC), unilateral neurologic deficits, and cognitive decline. Despite awareness of this disease since characterization in 1958, the underlying pathogenesis of RE remains unknown. Neurosurgical intervention, via functional hemispherectomy of the affected cerebral hemisphere, remains the standard of care for RE. However, due to the invasive nature of this procedure, and emerging evidence for a neuroinflammatory pathogenesis, various immunologic mechanisms have been proposed and immunotherapies trialed for patients with RE. The rare nature of the condition and heterogenous data in the literature have made determination of the role of immunotherapies, such as rituximab, difficult to ascertain. Here we report a series of three patients with pediatric RE, all of whom received early administration of immunotherapy in the context of restricted oligoclonal bands in the cerebrospinal fluid. While two patients were non-responders, one patient exhibited no radiographic progression on rituximab and one of those patients had clinical improvement. We discuss the potential role and mechanism of rituximab as well as the proposed mechanisms and available data for immunotherapy in RE more broadly. Although data remains limited, biomarkers of immunologic dysregulation may serve as indications for the use of immunotherapy in individuals with RE. Further, selection of immunotherapy, guided by ancillary testing including CSF analysis, may serve as an adjunct to hemispherectomy and neurosurgical intervention in some patients.

Background: Motor heterogeneity in Parkinson's disease (PD) reflects the interplay between dopaminergic and nondopaminergic circuits. While dopaminergic therapy restores activity within basal ganglia-cortical motor pathways, residual ON-state motor impairment represents nondopaminergic dysfunction. The determinants of these two components and their relation to quality of life remain incompletely understood.

Objective: To identify independent clinical correlates of dopaminergic and nondopaminergic motor loads and to assess their combined effects on quality of life in PD.

Methods: This retrospective study included 688 idiopathic PD patients evaluated with MDS-UPDRS (I-IV), NMSS, FOG-Q, FES-I, VHI, HAM-A, HDRS, and PDQ-39. Dopaminergic motor improvement was defined as Δ (MDS-UPDRS III OFF-ON), and residual nondopaminergic severity as ON-state MDS-UPDRS III. Multivariable regression analyses identified independent predictors and quality-of-life determinants.

Results: Greater dopaminergic improvement was associated with longer disease duration (β = 1.60, p = 0.010) and higher FOG-Q scores (β = 2.10, p = 0.001). Among NMSS domains, Sleep/Fatigue correlated positively and Mood/Cognition negatively with dopaminergic responsiveness. Residual nondopaminergic motor load was predicted by older age (β = 0.147, p < 0.001), higher FES-I and VHI scores, and NMSS Mood/Cognition and Sexual domains. In quality-of-life models, both nondopaminergic motor load (β = 0.41, p < 0.001) and total non-motor burden (β = 0.87, p < 0.001) were independent determinants of poorer PDQ-39 outcomes.

Conclusion: Dopaminergic improvement declines with disease progression yet remains modulated by freezing-related responsiveness. Nondopaminergic motor load-shaped by axial, bulbar, and limbic-autonomic features-and overall non-motor burden jointly account for quality-of-life deterioration in PD. Addressing both dopaminergic and nondopaminergic domains is crucial for comprehensive management.

Background: The Hemoglobin Glycation Index (HGI), integrating acute and chronic glycemic dysregulation, may reflect metabolic state in critical illness. We aimed to evaluate whether HGI predicts 30-day mortality in intracerebral hemorrhage (ICH) patients and identify potential threshold effects.

Methods: We analyzed 1351 ICH patients from the MIMIC-IV database. HGI was calculated as the difference between observed and predicted hemoglobin A1c (HbA1c), derived from admission glucose. Patients were stratified into HGI quartiles (Q1-Q4). The primary outcome was 30-day mortality, assessed using Cox regression models adjusted for demographics, comorbidities, and severity scores. Nonlinear relationships were evaluated with restricted cubic splines and threshold effect analysis.

Results: The cohort exhibited a nonlinear, L-shaped association between HGI and mortality (P for nonlinearity = 0.0008). Compared to Q4 (highest HGI), Q1 (lowest HGI) had a 53% higher mortality risk (adjusted HR 0.47, 95% CI 0.32-0.68, P < 0.001). A threshold effect was identified at HGI = 0.78: below this value, each unit increase in HGI reduced mortality by 24% (HR 0.76, 95% CI 0.68-0.85, P < 0.001), while no significant association existed above it (HR 1.11, P = 0.319). Subgroup analyses confirmed robustness across age, diabetes status, and neurological severity, with a pronounced protective effect in males (P for interaction = 0.038).

Conclusions: Low HGI independently predicts increased 30-day mortality in ICH, with a critical threshold at 0.78. HGI may represent a potential biomarker for metabolic risk stratification in neurocritical care, offering insights beyond conventional glycemic metrics.

Objective: This study assessed periprocedural complications and high-risk anatomical features in endovascular treatment of symptomatic severe stenosis (> 70%) in the petro-cavernous segment (PC-ICA).

Methods: We conducted a retrospective analysis of 23 patients with acute ischemic stroke and confirmed stenosis of the petrous or cavernous internal carotid artery (PC-ICA) via magnetic resonance angiography. All patients underwent balloon angioplasty and stent placement at a tertiary center between March 2018 and February 2023. Adjunctive mechanical thrombectomy was performed in three cases.

Results: The lesions were located in the petrous segment in 7 patients (30.4%) and the cavernous segment in 16 patients (69.6%). Perioperative complications occurred in 47.8% (n = 11) of the cohort. Specific complications included in-stent thrombosis (45.5%, n = 5), vasospasm (18.2%, n = 2), plaque prolapse/extrusion (18.2%, n = 2), re-occlusion (9.1%, n = 1), and ocular ischemia (9.1%, n = 1). Although the incidence of new postoperative diffusion-weighted imaging lesions was higher in the complication group (81.8% vs. 41.7%), this difference was not statistically significant (P = 0.123). However, patients who experienced complications had significantly worse 90-day modified Rankin Scale scores ($P < 0.05$).

Conclusion: While endovascular treatment for PC-ICA stenosis is technically feasible, it carries a substantial risk of periprocedural complications, particularly in-stent thrombosis associated with heavy calcification. The fixed bony tortuosity and osseoligamentous encasement limit the vessel's compliance, predisposing it to plaque protrusion. Therefore, strategies focusing on aggressive thrombus management and careful patient selection are essential in this anatomically complex region.