Neurologia i neurochirurgia polska最新文献

A working group convened by the Section of Multiple Sclerosis and Neuroimmunology of the Polish Neurological Society, the Polish Society of Family Medicine, and the Polish Society of Vaccinology has developed a consensus on supplementary data to the recommendations of the expert group of the Polish Society of Vaccinology, the Polish Society of Family Medicine, the Polish Dermatological Society, the Polish Association for the Study of Pain, and the Polish Neurological Society, and ECTRIMS/EAN of 2023 with regard to the currently available in Poland recombinant herpes zoster vaccine (RZV). It is intended for the prevention of herpes zoster and postherpetic neuralgia in individuals aged > 50 and individuals aged ≥ 18 who belong to herpes zoster risk groups. In Poland it is available with 50% reimbursement exclusively for patients aged 65 and older who have an increased risk of developing herpes zoster. This statement is based on the literature available as of 12 July 2024. The guidance will be updated as new data emerges. All data regarding the above-mentioned vaccine comes from clinical trials which have been reviewed, published and approved by the regulatory authorities and an increasing number of recommendations that might have an impact on real world data.

Aim of study: This study aimed to evaluate changes in prescription practices for treating idiopathic generalized epilepsy (IGE) in women of childbearing age, and to assess how switching from valproate (VPA) affects seizure outcomes. IGE accounts for 15-20% of all epilepsy cases. While VPA is the most effective treatment, its teratogenic risk limits its use in women of reproductive age, leading to recommendations for safer alternatives such as lamotrigine (LTG) and levetiracetam (LEV).

Material and methods: We retrospectively analysed the data from 130 women aged 18-49 diagnosed with IGE from 2000 to 2022.

Results: Of the 107 who used VPA, 44 remained on it until the last follow-up. 74% of participants achieved seizure freedom at some point, and 62% remained seizure-free at the last follow-up. The attempt to switch from VPA to other medications was unsuccessful in 23 (21.5% out of 107) patients due to adverse effects or loss of seizure control. Seizure freedom rates after 12 months were similar between VPA and alternative ASMs like LEV and LTG.

Conclusions and clinical implications: Our study indicates that LEV and LTG are effective alternatives to VPA for many women with IGE. However, some patients still require VPA for optimal seizure control. Further large-scale, randomised studies are needed to confirm these findings.

Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is a late-onset, autosomal recessive neurodegenerative disorder characterised by the triad of cerebellar ataxia, bilateral vestibular areflexia, and sensory neuropathy. First identified more than 30 years ago, its clinical phenotype has since expanded to include chronic cough, dysautonomia, and pain, with isolated neuronopathy reported in some cases. The discovery of biallelic AAGGG repeat expansions in intron 2 of the RFC1 gene in 2019 established the genetic basis for CANVAS, with the pathogenic expansions disrupting gene function via secondary structures such as G-quadruplexes. Despite this breakthrough, the precise pathophysiological mechanisms behind CANVAS remain elusive, necessitating further research into the molecular, clinical, and genetic aspects of this disease. This review consolidates the current understanding of CANVAS, encompassing the expanding spectrum of RFC1-related disorders, clinical manifestations, molecular underpinnings, and epidemiology, while exploring future directions for diagnostics and therapeutic advancements.

Introduction: An expert panel of the Section of Multiple Sclerosis and Neuroimmunology of the Polish Neurological Society has developed principles for the management of neuromyelitis optica spectrum disorders (NMOSD). These principles are based on expert opinion and data from the literature published up to May 2023. Recommendations were developed based on the results of the most recent clinical trials, guidelines of foreign and international scientific societies, and the authors' clinical experience.

Clinical implications: The principles for diagnosing NMOSD are discussed, with particular emphasis on serological and neuroimaging diagnosis. Recommendations for the treatment of relapses and chronic immunosuppressive treatment, including the most recent methods of immunotherapy, are also presented. Additionally, the principles of monitoring treatment efficacy and safety are included. Therapy regimens are completed with recommendations for symptomatic treatment. The paper also includes an algorithm for vaccination in patients with NMOSD. Therapeutic management in pregnant women with NMOSD is discussed.

Introduction: Multiple sclerosis (MS) is known to be an inflammatory disease of the central nervous system (CNS), with its typical neuropathological characteristics being demyelinating white matter lesions, relative preservation of axons, and astrogliosis. Although the criteria for MS diagnosis have been regularly updated, two main elements have remained unchanged: the obligatory proof for dissemination of pathological lesions in both time and space. However, not all presentations are clear, and doubts about the diagnosis sometimes arise. The central vein sign (CVS) is a neuroimaging feature that has attracted growing interest in terms of MS diagnosis, differentiation, and general management. The evidence regarding the use of CVS as an MS imaging marker has accumulated rapidly, with diverse CVS assessment rules being proposed for implementation into the diagnostic criteria.

Material and methods: We aimed to thoroughly assess the utility of CVS in MS management. A systematic review was conducted according to the Preferred Research Items for Systematic Reviews and Metanalyses (PRISMA 2020) guidelines. The PubMed and Embase databases were searched, and, after detailed analysis, 48 of the most recent studies were included in this review.

Results: Significant differences in terms of CVS positivity have been found between MS and its mimics. Moreover, CVS is able to distinguish patients with radiologically isolated syndrome (RIS) from subjects with non-MS conditions. Furthermore, some of the analysed studies have reported a promising performance of CVS rules in MS diagnosis.

Conclusions: The results of the reviewed studies are undoubtedly encouraging, reinforcing CVS's role as a valuable tool in multiple sclerosis management.

Aim of study: To compare transient ischemic attack (TIA), transient global amnesia (TGA), and transient ischemic attack with lesions found in magnetic resonance imaging/diffusion-weighted imaging (MRI-DWI) scans, in order to find similarities and differences in their clinical picture.

Clinical rationale for study: Magnetic resonance imaging scans account for a substantial part of the financial burden associa-ted with cerebrovascular events. Finding initial clinical features that differentiate transient brain ischemic events will be useful in developing standardized procedures for selecting patients who require further radiological imaging, thereby reducing overall costs.

Material and methods: A total of 9701 patients hospitalized in two major tertiary hospitals in the Silesian voivodeship in Poland between January 2016 and July 2024 with a diagnosis of TGA, TIA, and ischemic stroke were analyzed. The final group consisted of 947 patients, who were further divided into three categories: 425 TIA (44.87%), 125 TGA (13.19%), and 387 TIA with MRI-DWI lesions (41.92%). The data of patients were statistically analyzed.

Results: Patients with transient focal symptoms and confirmed DWI lesions in MRI scans were significantly older. They were more likely to have coronary heart disease, had higher C-reactive protein (CRP) levels, more severe symptoms, and were less likely to receive antiplatelet treatment than TGA and TIA patients. Transient global amnesia patients had higher systolic blood pressure on admission compared to other groups.

Conclusions: The presence of DWI-MRI lesions is associated with a higher initial clinical burden. Our results confirm that the lack of stroke prevention therapies may have determined the more severe course of the vascular event. This study supports a sudden rise in blood pressure being a contributing factor in TGA patients.

Clinical implications: Older patients with TIAs, having several vascular risk factors, but lacking prevention therapies are likely to present with cerebral lesions on DWI-MRI. These patients should undergo additional imaging procedures.

Introduction: Fatigue is one of the most common and distressing non-motor symptoms in Parkinson's disease, affecting up to half of patients. As a multidimensional symptom - encompassing physical, emotional, and cognitive aspects - it significantly reduces quality of life and daily functioning. It often appears in the early stages of the disease, and its subjective nature complicates its clear clinical definition. Despite its importance, fatigue is often underdiagnosed and rarely considered in routine care.

Clinical rationale for the study: Fatigue in Parkinson's disease is often an underestimated symptom, despite its significant impact on quality of life and daily functioning, often more so than motor symptoms. Its impact on physical activity - a key factor in slowing disease progression - underscores the need to better recognize and incorporate this symptom into clinical practice. The study results may support the development of more effective and personalized therapeutic strategies.

Material and methods: The study included 107 patients with Parkinson's disease (PD), with Hoehn and Yahr scale (H&Y) disease stages I [n = 13 (12%)], II [n = 15 (14%)], III [n = 53 (50%)], and IV [n = 25 (23%)]; ages ranged from 35 to 86 years; and disease duration ranged from 0 to 33 years (Median = 7.00 years). The study tool was a questionnaire. Fatigue levels were assessed using two scales: the Parkinson's fatigue scale (PFS-16) and the fatigue severity scale (FSS). Physical activity and quality of life were examined with the Parkinson's disease questionnaire (PDQ-39).

Results: The questionnaires used (FSS, PFS, PDQ-39) demonstrated high reliability (Cronbach's alpha: 0.93-0.96) and no floor/ /ceiling effects. Fatigue was reported in 57% of the participants, slightly more often in women, although the differences were not statistically significant. Disease duration was significantly longer in men (p < 0.01). Fatigue correlated with activity level (p < 0.01) and quality of life across all PDQ-39 domains.

Conclusions: Fatigue is a common and significant problem across all stages of Parkinson's disease. The FSS, PFS, and PDQ-39 questionnaires are reliable tools for assessing fatigue in people with PD. Fatigue has a significant impact on quality of life and activity levels. Further, multifaceted research is needed to clarify this issue.