Neurologia i neurochirurgia polska最新文献

Introduction: Prior research regarding multiple sclerosis (MS) has extensively examined the inflammatory processes, while the mechanisms of neurodegeneration, particularly cerebral atrophy, remain less understood. The purpose of this systematic review was to summarize the current knowledge about the degree and pattern of atrophy in various types of MS, and its relationship with the clinical progression of the disease and the impact of treatment on brain volume loss.

Material and methods: A systematic review was performed in accordance with the PRISMA guidelines. Relevant articles published between 1 January 2018 and 1 July 2024 available in the PubMed and Cochrane Library databases were obtained using a systematic search strategy. The following keywords were used to screen the publications: 'multiple sclerosis' and/or 'MS' and 'atrophy'. The eligibility criteria that identified and described outcomes were pre-established by the authors. Studies constituting clinical research protocols, theoretical inspections, articles describing other measurement methods or comparing measuring methods, conference abstracts and articles in a language other than English were excluded.

Results: Of the 1,911 records obtained from the systematic search based on keywords, 26 were included in the final review. Publications enabled the analysis of atrophy in patients compared to the healthy population, the evaluation of atrophy concerning different MS subtypes, its correlation with disability progression, the patterns of atrophy and the impact of disease-modifying therapies. Moreover, clinical trials involving atrophy measurements in MS patients were also collected.

Discussion: This systematic review confirmed the role of assessing volumetric changes in brain regions as a useful tool in the diagnosis of MS, assessment of its progression, and response to treatment. Given the relatively small number of new reports in the last six years, it also indicated the need for further research, especially using atrophy to assess the efficacy of therapy.

Aim of the study: We aimed to analyze cases of cerebrospinal fluid (CSF) rhinorrhea/fistula emerging after dopamine agonist (DA) therapy in patients with giant prolactinomas, and to identify clinical patterns and treatment strategies. We also sought to contextualize our findings through a systematic review of published cases.

Clinical rationale for the study: Dopamine agonist therapy is first line for prolactinomas; however, in invasive macroadenomas, rapid shrinkage may unmask skull base defects, leading to CSF leaks and infections like meningitis. These rare but critical events lack standard treatment protocols.

Material and methods: We reviewed six prolactinoma cases with post-cabergoline CSF rhinorrhea (2010-2024) and conducted a PubMed-based review (1980-2024) to compare clinical and treatment features.

Results: In our series (mean age: 49.3 years) CSF rhinorrhea developed between 14 and 420 days after therapy initiation. The sellar floor was the most frequent defect site (5 of 6 patients). All patients required surgical or CSF diversion procedures, including transsphenoidal or transcranial repair, external ventricular drainage, lumbar drainage, or ventriculoperitoneal shunting. One case resulted in fatal meningitis. The literature review included 68 cases across 33 studies. The median time to CSF leak onset was 90 days (range: 7-1460 days). Surgical repair, particularly transsphenoidal, was the most common treatment. Recurrence occurred in 25/55 cases (45.5%) and meningitis in 6/59 cases (10.2%); outcome data were not available for all patients.

Conclusions and clinical implications: Skull base erosion and rapid tumor shrinkage are major contributors. Our findings support early recognition, dose titration of DA therapy, close radiological follow-up, and timely surgical intervention. Further studies are needed to define risk stratification and evidence-based treatment protocols.

Introduction: Non-motor symptoms of Parkinson's disease (PD), particularly visual disturbances, often appear before a formal diagnosis and may serve as early signs or prodromal features of the disease. This review aims to evaluate ocular biomarkers associated with prodromal and early-stage PD, highlighting ophthalmic indicators that could support earlier diagnosis and aid in tracking disease progression.

Material and methods: PubMed and Scopus databases were systematically searched for all records available from their inception through May 2024, in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement extension for Scoping Reviews (PRISMA-ScR) guidelines. Two distinct PICO (PICO stands for Population, Intervention, Control, Outcome) frameworks were employed to guide the selection criteria: one for people with early Parkinson's disease and another for individuals at risk of prodromal PD.

Results: 30 studies were included - 4 solely on prodromal cohorts (RBD), 18 on patients with PD and 8 studies with both prodromal and clinical cohorts. Studies of people with RBD have shown abnormalities in pupillary light reflex, eye movements, and color discrimination. People with early PD showed impaired contrast sensitivity, impaired color discrimination, retinal structural changes, including retinal thinning (in the inner nuclear layer and ganglion cell-inner plexiform layer, altered pupillary responses, visual hallucinations, and ocular movement disorders).

Conclusions: Impairment of both color and contrast vision, as well as changes in optical coherence tomography (OCT), correlate with disease severity and cognitive decline. They can serve as an early, safe, noninvasive biomarker of neurodegeneration.

Introduction: Distal hereditary motor neuropathies (dHMNs) are a clinically and genetically diverse group of rare neuromuscular disorders characterized by progressive distal muscle weakness and atrophy, often with early onset and sparing of sensory function. One subtype, Jerash-type dHMN (dHMNJ), is caused by biallelic mutations in the SIGMAR1 gene and presents with pyramidal signs in addition to distal weakness.

Material and methods: A literature review was conducted by searches of the MEDLINE and PubMed databases using selected terms. Relevant original articles, case reports, case series, and reviews were selected as data sources.

Discussion: SIGMAR1-related disorders (SIGMAR1-RD) encompass a broad clinical spectrum including dHMN and juvenile amyotrophic lateral sclerosis (ALS) phenotypes. The Sigma-1 receptor plays a key role in cellular stress responses, ER-mitochondria interaction, and neuronal survival. Clinical presentation often includes distal muscle weakness and atrophy with pyramidal signs.

Pathogenic variant reported by authors: We present a 12-year-old boy with distal muscle weakness, foot drop, and pyramidal signs. Genetic testing identified a homozygous c.247T>C (p.Phe83Leu) SIGMAR1 variant, previously classified as a variant of uncertain significance (VUS).

Conclusion: This article supports the pathogenicity of the c.247T>C (p.Phe83Leu) SIGMAR1 variant and underlines the need for broader genetic testing in hereditary motor neuropathies.

Introduction: Immunosenescence is a natural process of immune system aging, which leads to significant changes in the functioning of both innate and adaptive immunity. Alterations in T and B lymphocytes can significantly impact the progression of neurological diseases including multiple sclerosis (MS), Alzheimer's disease (AD), Parkinson's disease (PD), and amyotrophic lateral sclerosis (ALS).

State of the art: Immunosenescence affects T and B cell subsets, reducing their proliferative capacity and altering cytokine profiles. In MS, these changes promote disease progression and diminish responses to immunomodulatory therapies. In AD and PD, dysfunctional T and B cells contribute to sustained neuroinflammation, exacerbating neurodegeneration. ALS is similarly associated with altered adaptive immunity.

Clinical implications: Recognizing how immunosenescent T and B cells contribute to disease in older adults is crucial for refining treatment strategies. These age-related immune changes may explain varied responses to therapies and highlight the need for novel approaches targeting the aged immune system in neurodegenerative diseases.

Future directions: Future research should focus on identifying the mechanisms by which immunosenescent lymphocytes modulate neuroinflammation and neurodegeneration in aging populations. Novel biomarkers and immunomodulatory therapies tailored to older adults could significantly improve outcomes in patients with neurological diseases.

Rationale for the study: Burnout syndrome is a prolonged response to chronic work-related stress, characterized by exhaustion accompanied by mental and physical discomfort, a sense of reduced effectiveness and motivation, dysfunctional attitudes and behaviors at work. Neurologists are recognized as a high-risk group, but no large-scale study has previously examined burnout in this professional group in Poland.

Aim of the study: This study aimed to determine the prevalence of burnout among Polish neurologists and to identify individual and organizational predictors, as well as perceived work-related barriers to effective practice.

Material and methods: A cross-sectional online survey [Computer-Assisted Web Interview (CAWI)] was conducted among members of the Polish Neurological Society (n = 342). Burnout was assessed using the Oldenburg Burnout Inventory (OLBI), alongside validated measures of depressive symptoms, insomnia, loneliness, occupational hardiness, occupational self-efficacy, and psychosocial work conditions. Multiple linear regression analyses were performed to identify predictors of the dimensions of burnout: exhaustion and disengagement.

Results: A high prevalence of burnout was found: 87.0% of respondents met the criteria for exhaustion and 77.5% for disengagement. Being of a younger age was a predictive factor for exhaustion, while living in a larger city slightly reduced disengagement. Severity of depression and work pace were significant predictors of higher levels of both burnout dimensions. More severe exhaustion was additionally associated with higher emotional and quantitative job demands, insomnia, and - unexpectedly - greater peer support. Protective factors included workplace commitment, occupational self-efficacy, professional development opportunities, and occupational hardiness. The most frequently cited barriers in the workplace were administrative overload (85.4%), high work pace (64.0%), and staff shortages (56.7%).

Conclusions: Burnout is highly prevalent among Polish neurologists, driven by both individual vulnerabilities (belonging to a younger age group, depression, insomnia) and organizational stressors (workload, administrative burden). Strengthening professional resources such as self-efficacy and hardiness, enhancing career development opportunities, and reducing bureaucratic workload are essential strategies. Targeted interventions are needed, particularly for early-career neurologists, in order to maintain well-being and ensure sustainable neurological care.

Aim of the study: To assess the agreement between continuous invasive (IBP) and intermittent non-invasive blood pressure (NIBP) monitoring during endovascular treatment (EVT) of acute ischemic stroke (AIS) under general anesthesia, and to evaluate the frequency of clinically relevant discrepancies between both methods and the potential impact of IBP use on treatment initiation times.

Clinical rationale for the study: Intraprocedural hypotension during mechanical thrombectomy (MT) is associated with worse outcomes in AIS patients. While continuous IBP monitoring is frequently used during procedures with high-risk of hypotension, it is not universally adopted in stroke EVT. Accurate, real-time hemodynamic monitoring may be essential to guide timely therapeutic interventions and optimize outcomes.

Materials and methods: In this prospective observational study, 30 AIS patients undergoing MT under general anesthesia were included. Simultaneous IBP and NIBP measurements were recorded throughout the procedure. Non-invasive blood pressure was measured every 5 minutes on one arm, while IBP was continuously recorded from a radial arterial catheter in the contralateral upper extremity. Paired readings were analyzed using Bland-Altman plots. The proportion of time with clinically relevant discrepancies between measurements was calculated. Additionally, door-to-groin (DTG) times were compared between patients with and without intended IBP monitoring.

Results: A total of 481 paired IBP and NIBP readings were analyzed. While mean differences for systolic blood pressure (SBP) and mean arterial pressure (MAP) were small (-0.64 mm Hg and -0.99 mm Hg, respectively), limits of agreement were wide (SBP: -40.6 to 39.4 mm Hg; MAP: -28.5 to 26.5 mm Hg). Diastolic blood pressure (DBP) showed poor agreement with a mean bias of -7.64 mm Hg. Discrepancies of ≥ 20 mm Hg for SBP occurred in 41.0% (IQR = 28.0-59.4%) of the procedure time, and discrepancies > 15 mm Hg for MAP in 29.6% (IQR = 22.8-58.5%). DTG times did not differ significantly between the IBP and NIBP groups (median: 41 vs. 38 minutes, p = 0.217).

Conclusions and clinical implications: Non-invasive blood pressure may show limited agreement with IBP and miss clinically relevant hemodynamic changes due to its intermittent nature and overestimation of blood pressure during hypotensive episodes. Invasive monitoring does not appear to delay treatment initiation and may improve blood pressure control in AIS patients undergoing EVT under general anesthesia. These findings, from a pilot study, should be interpreted with caution but provide a basis for larger prospective investigations.