Neurologia i neurochirurgia polska最新文献

The effect of botulinum toxin A (BoNTA) on non-motor symptoms (NMS) in patients with cervical dystonia remains an area of significant clinical interest, given the profound impact of these symptoms on patients' quality of life. While the therapeutic efficacy of BoNTA in alleviating motor symptoms of cervical dystonia is well established, its impact on NMS such as depression, anxiety disorder, and sleep disturbance requires further investigation. This systematic review synthesizes the latest evidence on the effects of BoNTA on these selected non-motor symptoms. A comprehensive search of the PubMed, Web of Science, and Scopus databases identified 266 articles, of which eight studies met our strict inclusion criteria. Pre- and post-intervention changes in depression, anxiety, and sleep disturbance were assessed in a total of 280 adult patients with cervical dystonia treated with BoNTA. The results indicate that BoNTA has a positive effect on depressive symptoms, with most studies showing a statistically significant improvement after treatment. Similarly, studies are reporting significant reductions in anxiety scores following BoNTA treatment. However, the effects of this treatment method on sleep disturbances were less conclusive, with none of the reviewed studies showing significant improvements in sleep quality or daytime sleepiness. The results highlight the potential of BoNTA to positively influence non-motor symptoms, particularly depression and anxiety, in patients with cervical dystonia, although its effects on sleep remain unclear. These findings underscore the need for further research to fully understand the mechanisms underlying the non-motor effects of BoNTA and to develop comprehensive treatment strategies.

Introduction: Functional movement disorders (FMD) are defined by diverse phenotypes of altered movements that lack corresponding pathology in an anatomical region, and are typically characterized by inconsistent findings on neurological examination.

State of the art: While there are several suggestive clinical features indicating FMD, objective biomarkers are still lacking. We conducted a systematic review of the literature with an emphasis on literature published after February 2019 aiming to summarise current knowledge on biomarkers of FMD. We divided our findings into four main categories: genetic, biofluid, neuroimaging, and electrophysiological biomarkers. For the differential diagnosis of functional tremor, functional tic-like behaviours (FTLB), and functional myoclonus, previous studies support the use of electrophysiological biomarkers. Evidence from neuroimaging research supports the multi-network model of FMD as a condition affecting the attentional, sensorimotor, self-agency/multimodal integration, and limbic/salience circuits. Biomarkers such as neurofilament light chain, inflammatory, and autoimmune factors should still be considered experimental, since results are based on small sample sizes. There is preliminary evidence from a genetic study that in FMD there is a complex interaction between individual predisposing risk genes involved in the serotonergic pathway.

Clinical implications: Although the diagnosis of FMD remains challenging, and depends mainly on clinical judgement, research is underway to identify potential biomarkers to improve diagnostic confidence. Previous studies indicate that, in addition to psychological symptoms, biological changes can be detected in patients with FMD. This is evidenced by different patterns of neurotransmission related to stress responses and emotional regulation.

Future directions: We believe it is vital to conduct larger trials in diverse populations from different regions of the world in order to find more reliable biomarkers of FMD.

Introduction: Multiple sclerosis (MS) is a progressive disease leading to disability, that mostly affects young and middle-aged adults. This study was aimed at evaluating the impact of sociodemographic and clinical factors, including changes in access to disease-modifying therapies (DMTs), on the quality of life (QoL) of Polish individuals with MS and to compare the findings to earlier national studies.

Material and methods: Data was collected from patients with MS across 19 MS treatment centres in Poland between February and April 2024. QoL was assessed using the EuroQol five-dimension, five-level (EQ-5D-5L) index and the EQ Visual Analogue Scale (EQ-VAS). Sociodemographic and clinical data was obtained via a standardised questionnaire.

Results: The study included 3,165 patients with MS (mean age: 42.03 years; disease duration: 10.6 ± 7.85 years; female-to-male ratio 2.2:1). The mean EQ-5D-5L index score was 0.89 (± 0.15), and the mean EQ-VAS score was 71.58 (± 18.67), indicating an overall moderate-to-good QoL in the studied cohort. Pain/discomfort (80.7%) and anxiety/depression (79.6%) were the most frequently reported problems. Higher QoL scores were associated with younger age, relapsing-remitting MS, lower disability (EDSS ≤ 3.5), shorter disease duration, lack of comorbidities, employment, urban residence, childlessness, DMT usage, and infrequent DMTs switches (p < 0.001). When compared to earlier Polish studies, these results suggest a meaningful improvement in QoL over the past decade.

Conclusions: The QoL of Polish patients with MS remains diminished compared to that of the general population. However, there has been a notable improvement compared to previous national data. This trend probably reflects advances in MS care, including wider access to novel DMTs and optimised treatment strategies.

Aim of the study: The objective of the study was to evaluate local brain volume in short and long follow-up periods in the same group of women with multiple sclerosis (MS), which was evaluated in our in previous study during the peri-pregnancy period (PPP).

Clinical rationale for the study: In our previous study we observed a significant reduction in thalamus volume (TV) during PPP in clinically stable patients with MS. In this follow-up study we determined whether the observed regional brain volume change during the PPP was also a long-term effect.

Materials and methods: We included 11 patients with MS and clinical and magnetic resonance imaging (MRI) data, obtained at 4 time points: time point 1 (TP1) up to 6 months before pregnancy; time point 2 (TP2) up to 6 months after delivery; time point 3 (TP3) > 12 months after TP2, "short-term follow-up"; time point 4 (TP4) > 24 months after TP2, "long follow-up". MRI analysis including assessment of T2 lesion volume, T2LV; T1 lesion volume, T1LV; number of gadolinium-enhanced lesions, T1GD+; TV and corpus callosum volume (CCV) was performed at each time points.

Results: At TP3, CCV was significantly lower compared with TP2 (p = 0.02). We also found that T1LV significantly increased at TP3 compared with TP2 (p = 0.02). In the short follow-up we observed a slightly greater change in CCV than in the primary study (p = 0.06). At TP4, CCV was significantly lower and T1LV was significantly higher compared with TP2 (p = 0.02, p = 0.02, respectively). We observed a significant increase of T1LV at all time points beginning with TP1.

Conclusion: Our study demonstrates that in clinically stable patients with multiple sclerosis in the peri-pregnancy period regional brain atrophy and burden of focal lesions progress in the short and long term despite re-initiating the same therapy. These observations suggest that the protective effects of pregnancy on the pathological processes in MS are limited.

Clinical implications: Our findings provide relevant clinical information. Local brain atrophy and increase of focal lesion burden should be considered for decision-making in the treatment of women with MS early in the postpartum period to prevent disease progression.

Aim of the study: The Unified Wilson's Disease Rating Scale (UWDRS) was first introduced in 2007 to assess neurologic status and impairment in patients with Wilson's disease (WD). However, major issues have been raised, including the lack of assessment of mental and psychiatric status, the lack of instructions for the assessment of certain motor functions, difficulties with gait and posture assessment, and heterogenous response options for questions across the scale.

Clinical rationale for the study: The development of a modified version of the UWDRS (mUWDRS) was proposed.

Materials and methods: A new version of UWDRS with revised wording and options was created, incorporating items from the Movement Disorder Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS), the Unified Dyskinesia Rating Scale, and the Pantothenate Kinase Associated Neurodegeneration Disease Rating Scale (PKAN-DRS). As in the original, part I of the mUWDRS assesses the level of consciousness. Part II is now divided into two partly self-reported subscales: part IIa assesses 10 daily living activities and a new part IIb addresses cognitive functioning, mental and behavioral status. Part III assesses neurologic status as before, but with modifications for gait and posture items. After 2 rounds of cognitive pretesting, mUWDRS was validated in 7 patients by 8 neurologists for interrater agreement (Fleiss kappa or Kuder Richardson Coefficient) and for internal consistency (Cronbach's alpha).

Results: In the pilot test, interrater agreement with mUWDRS was almost perfect or substantial, with index values of 1 for part I, 0.90 for part IIa, 0.93 for cognitive functioning and 0.86 for behavioral status in part IIb, and 0.826 for part III. A high level of internal consistency was observed, with Cronbach's α of 0.82 for part II and 0.84 for part III.

Conclusions and clinical implications: The mUWDRS, designed to assess both motor and nonmotor symptoms of WD and functional impairment, performed well in pilot testing. Since the modified version of the WD rating scale has a good interrater agreement, a proper validity study is planned to be conducted. The scale is available on: https://www.movementdisorders.org/MDS/MDS-Clinical-Outcome-Assessment.htm.