European Neurology最新文献

This article presents the historical and clinical analysis of Auguste-Joseph Bellinghen (1818-1872), whose medical course was documented in 1853 and later published in 1871. These records provide a rare longitudinal account of a patient with progressive muscular atrophy (PMA), a condition first described by Aran in 1850 and later redefined within the spectrum of motor neuron diseases by Charcot. The 1853 manuscript depicts a 35-year-old artisan with asymmetrical weakness, fasciculations-described by the patient as a "gelatinous oscillation"-and progressive muscular wasting, while the 1871 report shows him severely disabled, socially marginalized, and institutionalized. Together, these sources illustrate both the slow, relentless course of PMA and the therapeutic practices of the time, including cauterization, sulfur baths, and electrotherapy, all of which proved ineffective. The case also reflects the evolution of nosological frameworks: from Aran's clinical description without neuropathological confirmation to Charcot's integration of anatomical lesions into disease classification, thereby laying the foundations of modern neurology. Beyond clinical insights, the documents highlight the value of hospital archives, medical iconography, and the role of interns in constructing medical memory. Bellinghen's case thus exemplifies how individual trajectories illuminate both the biological and social dimensions of chronic illness in 19th-century France, while providing historiographic depth to the early history of motor neuron disease.

Introduction: Given the limited exploration of sex differences in OSA, this study aims to systematically compare the clinical presentation, hematological features, and metabolic profiles between male and female patients.

Methods: We conducted a retrospective analysis of 1567 adults (1173 men and 394 women) at the Sleep Center of Zhongda Hospital, Southeast University. Participants were categorized into mild (AHI 5-14), moderate (AHI 15-29), severe (AHI ≥30) OSA groups, and healthy controls (HC). Data included demographic characteristics, blood routine, metabolic panels, thyroid function tests, and comorbidity prevalence.

Results: Females with OSA were significantly older than males (p < 0.001). Males had higher rates of traditional risk factors like smoking, alcohol use, and loud snoring (p < 0.005), while females reported more insomnia. Hematologically, males with OSA showed progressively increasing red blood cell counts, hematocrit, monocyte counts, and systemic inflammation with disease severity (p < 0.01). Metabolically, males exhibited more pronounced dyslipidemia, higher glycosylated hemoglobin, and uric acid levels in severe OSA (p < 0.05). In contrast, females demonstrated significant alterations in thyroid hormones (free triiodothyronine, free thyroxine) with increasing OSA severity (p < 0.01).

Conclusions: OSA shows significant sex dimorphism. Men are characterized by "metabolic disorders, aggravated inflammation, high severity of OSA". Women are characterized by "atypical symptoms, advanced age, thyroid abnormalities". These findings underscore the critical necessity for gender-tailored approaches in OSA screening, diagnosis, and therapeutic management.

Introduction Sleep-disordered breathing (SDB), particularly obstructive sleep apnea (OSA), increases the risk of first and recurrent stroke. SDB is common after stroke or transient ischemic attack (TIA) and is linked with fatigue, sleepiness, poorer cognitive and functional outcomes. Continuous positive airway pressure (CPAP) therapy may mitigate these effects, but its efficacy in post-stroke populations remains uncertain. Methods Following a preregistered PROSPERO protocol (CRD420251078235), we systematically searched MEDLINE, Embase, and Cochrane CENTRAL. Eligible studies included adults with stroke or TIA and SDB, comparing CPAP with sham, usual care, or non-adherence. Primary outcomes were recurrent stroke or TIA and all-cause mortality; secondary outcomes included cardiac events, functional recovery, cognition, sleepiness, and fatigue. Random-effects models were used separately for RCTs and observational studies. Results From 7,132 records, 30 studies (19 RCTs, 11 observational studies; n = 3,381) met inclusion criteria. Seven RCTs assessed recurrent stroke or TIA and showed CPAP reduced the odds of re-stroke (OR 0.49, CI 95% 0.25-0.95; I² = 0%). All-cause mortality was unchanged in short-term RCTs, but long-term unadjusted observational data showed lower all-cause mortality with CPAP (OR 0.50, CI 95% 0.39-0.63; I² = 0%). High CPAP adherence was linked to improved functional and cognitive outcomes and reduced sleepiness, though data on fatigue and cardiac events were limited. Conclusion CPAP therapy after stroke or TIA may reduce stroke recurrence and all-cause mortality, while improvements in sleepiness, functional, and cognitive outcomes appear less consistent and largely adherence-dependent.

Background: Rapid eye movement sleep behavior disorder (RBD) represents a parasomnia marked by impaired muscle inhibition during REM sleep, commonly manifesting as vivid dream enactment with potentially violent movements. Its strong association with neurodegenerative diseases, particularly α-synucleinopathies such as Parkinson's disease, has drawn increasing attention. This study presents a bibliometric overview of global RBD research from 2010 to 2025.

Methods: We analyzed 4,204 publications from the Web of Science Core Collection and Scopus databases. Tools including CiteSpace, VOSviewer, Python, R (bibliometrix), Excel, and Charticulator were used to examine publication trends, geographic and institutional distribution, author influence, journal preferences, co-citation networks, and keyword evolution.

Results: RBD-related publications increased steadily, with a marked rise after 2015. The United States, China, and Canada were leading contributors, and institutions such as the University of Toronto, Mayo Clinic, and Peking University showed strong productivity and collaboration. Key researchers included Schenck CH, Boeve BF, and Postuma RB. Top journals were Sleep, Neurology, and Movement Disorders. Research has shifted from clinical characterization to biomarker identification and early intervention. Recent themes include α-synuclein pathology, prodromal Parkinson's disease, and melatonin therapy.

Conclusion: RBD research has progressed toward translational approaches focused on early detection and neurodegenerative risk prediction, supported by global collaboration and growing interest in mechanistic insights.

A 19th-century painting by Franz Paczka, Un cas grave (La leçon de médecine; ca. 1875-1880), depicts a young girl supported by physicians in a striking clinical tableau suggestive of an acute neurological emergency. The scene shows generalized rigidity with cervical extension, abnormal upper-limb posturing, and a cloth placed at the mouth. A small wound on the left hemithorax is historically described as a dog bite. These elements invite a differential diagnosis between generalized tetanus and rabies. Tetanus is supported by the apparent sustained rigidity and possible jaw involvement, and remains biologically plausible after a dog bite through secondary environmental contamination of a devitalized wound. Rabies, recently proposed for this artwork, remains a strong alternative diagnosis, although several defining features such as hydrophobia/aerophobia, hypersalivation, and fluctuating agitation cannot be confidently inferred from a single static image. Rather than claiming diagnostic certainty, this iconodiagnostic analysis argues that the painting is best approached as a structured differential diagnosis exercise illustrating both the clinical reasoning prompted by visual clues and the methodological limits of retrospective diagnosis from art.

Introduction: Dilated perivascular spaces (PVS) are associated with small vessel disease in the aging population. We sought to investigate the incidence and dynamic evolution of MRI-detectable PVS progression in patients with cerebral amyloid angiopathy (CAA).

Methods: Patients with symptomatic CAA who underwent baseline and follow-up MRI scans >2 years apart were included. The severity of both basal ganglia (BG) and centrum semiovale (CSO) PVS were rated. Multivariable logistic regression was used to determine the risk factors for PVS progression.

Results: We included 90 patients with CAA (mean age 72.6 years, SD 8.0 years), of which 53 (58.9%) had intracerebral hemorrhage (ICH) at baseline. During a median follow-up of 4.8 years (IQR 3.6 - 6.6 years), PVS progression was observed in 24 patients (26.7%) at follow-up MRI. After adjusting for age, hypertension and time between baseline and follow-up MRI, cerebral microbleed (CMB) progression (OR 4.12, 95% CI 1.31 - 12.95; p=0.015) and presence of ICH at baseline (OR 8.61, 95% CI: 2.09 - 35.52; p=0.003) were independent predictors of PVS progression. In multivariable regression analysis, presence of ICH (OR 8.78, 95% CI 1.74 - 44.35; p=0.009) and hypertension (OR 5.73, 95% CI 1.25 - 26.29; p=0.025) were associated with BG-PVS progression. However, only CMB progression (OR 10.17, 95% CI 1.84 - 56.35; p=0.008) was associated with CSO-PVS progression.

Conclusion: PVS progression occurs in a subset of CAA patients reimaged after a median of 4.8 years and is associated with CMB progression. PVS progression might be a useful neuroimaging marker for visualizing CAA-related vascular changes.

Introduction: Brain-derived neurotrophic factor (BDNF) plays an important role in the survival of dopaminergic neurons. Clinical studies have suggested that serum BDNF levels are reduced in patients with Parkinson's disease (PD). However, no study has investigated peripheral BDNF levels and BDNF Val66Met polymorphism in the prodromal stage of PD and their relationship with disease conversion.

Methods: 120 patients with video-polysomnographically (v-PSG) confirmed iRBD and 120 healthy controls (HCs) were enrolled. Genetic analyses were performed, and plasma levels of BDNF were measured. All patients with iRBD underwent comprehensive clinical testings, and 107 iRBD patients were prospectively followed-up.

Results: Plasma BDNF levels were significantly lower in the iRBD group than in HCs (18878.85 pg/ml vs. 24649.85 pg/ml, p = 0.002), but no differences were observed in BDNF Val66Met carrier rates between the two groups. Plasma BDNF levels did not differ significantly between BDNF Val66Met carriers and non-carriers. Notably, higher plasma BDNF levels were associated with an increased risk of short-term disease con-version (Hazard Ratio = 3.418, 95% CI 1.520-7.684, p = 0.003), whereas BDNF Val66Met carrier rates showed no such association.

Conclusion: Our findings suggest that plasma BDNF is significantly associated with iRBD and may likely serve as a prognostic biomarker for the development of neurodegenerative dis-ease. However, the BDNF Val66Met polymorphism may not be involved in the path-ogenesis of iRBD as well as phenoconversion in the studied population.

Introduction: Recently, a nationwide stroke care consisting of stroke care units (SCUs) and stroke centers (SCs) was established in the Republic of Georgia.

Patients and methods: A hospital-based prospective registry to evaluate the quality of stroke care and an additional nationwide survey on factors leading to prehospital delay and subsequent low rate of intravenous thrombolysis (IVT) and endovascular thrombectomy (EVT).

Results: A total of 5,385 consecutive patients with acute stroke (3,636 ischemic, 1,302 TIA, and 447 hemorrhagic) were included in the stroke registry, of whom 2,459 (46%) were female, with a mean age of 70.3±11.3 years. A total of 1,538 (28.6%) patients presented <6 h (1,160 [21.5%] <4.5 h). Of 3,636 patients with ischemic stroke, 121 (3.3%) patients received IVT, 72 (2.0%) received EVT, and 17 (0.5%) received both. An additional 1,067 patients with acute ischemic stroke (898) and TIA (169) were interviewed to understand the factors of prehospital delay. Of these, 472 (44.2%) were female, with a mean age of 70.5 ± 11.4 years, and 339 (31.8%) patients arrived <6 h (219 [20.5%] <4.5 h). Forty-two patients (4.7%) received IVT, 37 (4.1%) received EVT, and 6 (0.7%) received both. Pre-ictal education level, high socio-economic status, stroke awareness, calling the ambulance, and a knowledgeable ambulance team transferring patients to the stroke-ready hospital predicted timely arrival at the SC.

Conclusions: The study reveals major problems in the prehospital management of acute stroke.

Discussion: There is a great need for a nationwide geographic transfer plan for acute stroke service, connecting ambulances, SCUs, and SCs and for the educational campaigns to increase stroke awareness among the population.

Introduction: Identification of acute ischemic stroke (AIS) patients within the 4.5-h therapeutic window is critical for therapy. Diffusion-weighted imaging (DWI) and fluid-attenuated inversion recovery (FLAIR) sequences are an approach to determine whether the time since stroke (TSS) is within 4.5 h. However, interobserver variability and limited accuracy are observed in visual assessments. We aimed to develop a transfer learning model for predicting AIS onset within 4.5 h.

Materials and methods: A total of 266 AIS patients with known TSS who underwent imaging scans before treatment were retrospectively analyzed, divided into a training set (n = 211) and a validation set (n = 55). The model was built using DWI and FLAIR sequences. After image preprocessing and data augmentation, a 3D ResNet-18 pretrained on the Kinetics dataset was selected and adapted via transfer learning with DWI-FLAIR input. The model performance was compared with human visual assessment, which was based on the DWI-FLAIR mismatch principle. Partial mismatch was defined as hyperintense infarct on DWI with a smaller corresponding hyperintense area on FLAIR.

Results: Baseline characteristics did not differ between the training and validation sets. On the validation set, the model achieved sensitivity of 0.833 (0.703-0.941), specificity of 0.880 (0.737-1.000), and AUC of 0.929 (0.758-0.935), outperforming human visual assessment (sensitivity 0.767 [0.613-0.903]; specificity 0.360 [0.185-0.560]; AUC 0.563 [0.451-0.693]). For partial DWI-FLAIR mismatch cases, the model correctly classified all 15 cases, whereas humans classified 4.

Conclusion: The 3D ResNet-18 model shows promise in identifying AIS within 4.5 h, including partial DWI-FLAIR mismatch, but requires multicenter validation before use.

Introduction: Hemorrhagic transformation is one of the most common complications of acute ischemic stroke. However, the association between hemorrhagic transformation and internal carotid artery (ICA) tortuosity has rarely been investigated. This study investigated the effect of ICA tortuosity on hemorrhagic transformation after acute ischemic stroke.

Methods: We conducted a retrospective analysis on stroke patients' data collected from prospective hospital-based stroke registry. The baseline demographics, clinical and radiological variables, including the Fazekas scale, advanced Fazekas scale (grade 0-9), white matter hyperintensity volume, presence of cerebral microbleeds, and hemorrhagic transformation, were compared based on the severity of ICA tortuosity and divided into quartiles (Q1: <25%, Q2+Q3: 25%-75%, and Q4: >75%). ICA tortuosity was examined using in-house vessel analysis software. The primary outcome was hemorrhagic transformation.

Results: A total of 146 patients were analyzed, including 167 ICAs, which were vascular territories with acute cerebral infarction. Among the various risk factors considered, intravenous thrombolysis, endovascular thrombectomy, stroke severity, and ICA tortuosity index were associated with the occurrence of hemorrhagic transformation. In multivariate analysis, ICA tortuosity was also significantly associated with the occurrence of hemorrhagic transformation, while the severity of ICA tortuosity was negatively correlated (Q1: reference, Q2+Q3: 0.27 [0.09-0.84], Q4: 0.14 [0.03-0.70]; p = 0.023 for Q2+Q3, p = 0.016 for Q4).

Conclusions: We identified a paradoxical relationship between ICA tortuosity and hemorrhagic transformation after acute ischemic stroke. Patients with more severe tortuosity experienced fewer hemorrhagic transformations, and vice versa. These findings highlight the potential of ICA tortuosity as a marker for identifying high-risk patients.