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Blood-brain barrier-associated serum proteins in epilepsy - clinical and diagnostic implications. 癫痫的血脑屏障相关血清蛋白-临床和诊断意义。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-09-19 DOI: 10.5603/pjnns.107937
Daniel Matovu
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引用次数: 0
Primary optic nerve lymphoma masquerading as neuromyelitis optica spectrum disorder. 原发性视神经淋巴瘤伪装成视神经脊髓炎。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-09-08 DOI: 10.5603/pjnns.107023
Maksymilian A Brzezicki, Jakub Moskal, Wojciech Wierzchołowski, Natalia Pawlak, Szymon Jurga, Radosław Kaźmierski
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引用次数: 0
Predictors for long-term incomplete nidus obliteration following stereotactic radiosurgery for brain arteriovenous malformations: a systematic review and meta-analysis. 立体定向放射治疗脑动静脉畸形后长期不完全病灶闭塞的预测因素:一项系统回顾和荟萃分析。
IF 2.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-01-01 Epub Date: 2025-03-13 DOI: 10.5603/pjnns.103718
Maurycy Rakowski, Natalia A Koc, Samuel D Pettersson, Tomasz Klepinowski, Piotr Zieliński, Tomasz Szmuda

Introduction: This study aimed to identify predictive factors for long-term incomplete nidus obliteration following stereotactic radiosurgery (SRS) for brain arteriovenous malformations (AVMs).

Material and methods: A systematic search across the PubMed, Web of Science, and Scopus databases identified observational studies reporting such factors. Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed. The study protocol was registered through PROSPERO. Each eligible study's quality was assessed using the modified Newcastle-Ottawa Scale. Odds ratios (ORs) were calculated for dichotomous parameters.

Results: Two high-quality prospective cohort and three high-quality retrospective cohort studies were included, covering patients with complete (n = 638) and incomplete (n = 297) nidus obliteration. The mean age of the patients was 25.54 ± 12.81 years and the mean follow-up time was 95.98 ± 27.64 months. Predictors for incomplete obliteration of nidus included: AVM classified as Spetzler-Martin (SM) grade ≥ IV (odds ratio (OR) 10.57, 95% confidence interval (CI) 2.00-55.96, p = 0.006), the presence of multiple (> 1) feeding arteries (OR 6.47, 95% CI 2.20-19.10, p = 0.0007), nidus volume > 10 mL (OR 5.08, 95% CI 1.68-15.33, p = 0.004), and the occurrence of intranidal aneurysm (OR 3.33, 95% CI 1.10-10.08, p = 0.03). No statistically significant difference in proportions of patients with incomplete nidus obliteration was found between paediatric (≤ 18 years) and adult (> 18) patient cohorts (p = 0.95).

Conclusions: The following factors were found to be predictive for long-term incomplete nidus obliteration post-SRS for brain AVMs: SM grade equal to or higher than IV; the presence of multiple feeding arteries; AVM nidus volume exceeding 10 mL; and the occurrence of intranidal aneurysm. These findings will be beneficial in refining patient selection for radiosurgical treatment.

本研究旨在确定立体定向放射手术(SRS)治疗脑动静脉畸形(AVMs)后长期不完全病灶闭塞的预测因素。材料和方法:在PubMed, Web of Science和Scopus数据库中进行系统搜索,确定了报告这些因素的观察性研究。遵循系统评价和荟萃分析(PRISMA)指南的首选报告项目。研究方案通过PROSPERO注册。每个符合条件的研究的质量采用改良的纽卡斯尔-渥太华量表进行评估。计算二分类参数的比值比(ORs)。结果:纳入2项高质量前瞻性队列研究和3项高质量回顾性队列研究,涵盖了完全(n = 638)和不完全(n = 297)病灶闭塞患者。患者平均年龄25.54±12.81岁,平均随访时间95.98±27.64个月。病灶不完全闭塞的预测因素包括:AVM分类为Spetzler-Martin (SM)分级≥IV级(优势比(OR) 10.57, 95%可信区间(CI) 2.00-55.96, p = 0.006),存在多条(>)供血动脉(OR 6.47, 95% CI 2.20-19.10, p = 0.0007),病灶体积> 10ml (OR 5.08, 95% CI 1.68-15.33, p = 0.004),以及膜内动脉瘤的发生(OR 3.33, 95% CI 1.10-10.08, p = 0.03)。在儿童(≤18岁)和成人(≤18岁)患者队列中,不完全病灶闭塞患者的比例无统计学差异(p = 0.95)。结论:以下因素可预测脑AVMs术后长期不完全病灶闭塞:SM分级≥IV级;有多条供血动脉;AVM病灶体积超过10ml;以及膜内动脉瘤的发生。这些发现将有助于改进放射外科治疗的患者选择。
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引用次数: 0
Response to: DNAJC30 variants can also manifest phenotypically as Leigh/LHON overlap syndrome. DNAJC30变异也可表现为Leigh/LHON重叠综合征。
IF 2.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-01-01 Epub Date: 2025-01-20 DOI: 10.5603/pjnns.103974
Karol Chojnowski, Kamil Dzwilewski, Magdalena Krygier, Marta Zawadzka, Maria Mazurkiewicz-Bełdzińska
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引用次数: 0
Does botulinum toxin type A treatment influence non-motor symptoms in cervical dystonia patients? A systematic review. A 型肉毒毒素治疗会影响颈肌张力障碍患者的非运动症状吗?系统综述。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-01-01 Epub Date: 2025-02-14 DOI: 10.5603/pjnns.102672
Małgorzata Dudzic, Anna Pieczyńska, Artur Drużdż, Katarzyna Hojan

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.

鉴于这些症状对患者生活质量的深远影响,肉毒毒素A (BoNTA)对颈肌张力障碍患者非运动症状(NMS)的影响仍然是一个重要的临床研究领域。虽然BoNTA在缓解颈肌张力障碍运动症状方面的疗效已得到证实,但其对抑郁、焦虑障碍、睡眠障碍等NMS的影响有待进一步研究。这篇系统综述综合了BoNTA对这些选定的非运动症状的影响的最新证据。对PubMed、Web of Science和Scopus数据库的全面搜索确定了266篇文章,其中8项研究符合我们严格的纳入标准。在总共280名接受BoNTA治疗的成年颈张力障碍患者中,评估了干预前和干预后抑郁、焦虑和睡眠障碍的变化。结果表明BoNTA对抑郁症状有积极作用,大多数研究显示治疗后有统计学上显著的改善。同样,研究也报告了BoNTA治疗后焦虑评分的显著降低。然而,这种治疗方法对睡眠障碍的影响不太确定,没有一项研究显示睡眠质量或白天嗜睡有显著改善。结果强调BoNTA对颈肌张力障碍患者的非运动症状,特别是抑郁和焦虑有积极影响的潜力,尽管其对睡眠的影响尚不清楚。这些发现强调了进一步研究的必要性,以充分了解BoNTA非运动效应的潜在机制,并制定综合治疗策略。
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引用次数: 0
Biomarkers of functional movement disorders - a systematic review. 功能性运动障碍的生物标志物——系统综述。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-01-01 Epub Date: 2025-05-26 DOI: 10.5603/pjnns.103052
Anna Dunalska, Kamila Saramak, Katarzyna Śmiłowska, Vanessa Carvalho, Rachael Nicholls, Ali Abusrair, Jarosław Sławek, Simon Schmitt, Carolin Klages, Kirsten R Müller-Vahl, Natalia Szejko

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.

功能性运动障碍(FMD)是指在解剖区域缺乏相应病理的运动改变的不同表型,通常以神经学检查结果不一致为特征。现状:虽然有一些提示口蹄疫的临床特征,但仍然缺乏客观的生物标志物。我们对文献进行了系统综述,重点是2019年2月以后发表的文献,旨在总结口蹄疫生物标志物的当前知识。我们将我们的发现分为四大类:遗传、生物流体、神经成像和电生理生物标志物。对于功能性震颤、功能性抽搐样行为(FTLB)和功能性肌阵挛的鉴别诊断,先前的研究支持使用电生理生物标志物。来自神经影像学研究的证据支持口蹄疫的多网络模型,认为口蹄疫是一种影响注意力、感觉运动、自我代理/多模式整合和边缘/突出回路的疾病。生物标志物如神经丝轻链、炎症和自身免疫因子仍应被认为是实验性的,因为结果是基于小样本量。一项遗传学研究的初步证据表明,在口蹄疫中,涉及血清素能途径的个体易感风险基因之间存在复杂的相互作用。临床意义:尽管口蹄疫的诊断仍然具有挑战性,主要取决于临床判断,但研究正在进行中,以确定潜在的生物标志物,以提高诊断的信心。以往的研究表明,口蹄疫患者除了心理症状外,还可以检测到生物学变化。与压力反应和情绪调节相关的不同神经传递模式证明了这一点。未来方向:我们认为,为了找到更可靠的口蹄疫生物标志物,在世界不同地区的不同人群中进行更大规模的试验至关重要。
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引用次数: 0
Improving Quality of Life in Polish patients with multiple sclerosis: a multicentre analysis. 改善波兰多发性硬化症患者的生活质量:一项多中心分析
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-01-01 Epub Date: 2025-06-20 DOI: 10.5603/pjnns.105052
Joanna Tarasiuk, Katarzyna Kapica-Topczewska, Agata Czarnowska, Monika Chorąży, Katarzyna K Snarska, Jan Kochanowicz, Halina Bartosik-Psujek, Iwona Rościszewska-Żukowska, Jolanta Czarnota, Alicja Kalinowska, Karolina Kania, Wojciech Ambrosius, Joanna Siuda, Weronika Galus, Anna Walawska-Hrycek, Aleksandra Kaczmarczyk, Martyna Odzimek, Piotr Dubiński, Anna Jamroz-Wiśniewska, Konrad Rejdak, Marta Milewska-Jędrzejczak, Andrzej Głąbiński, Izabela Domitrz, Patryk Sochań, Natalia Bednarczyk, Katarzyna Kubicka-Bączyk, Robert Kwinta, Monika Adamczyk-Sowa, Ewa Krzystanek, Anetta Lasek-Bal, Katarzyna Maciejowska, Jacek Zaborski, Aleksandra Podulka, Marek Klus, Małgorzata Dorobek, Justyna Zielińska-Turek, Witold Palasik, Anna Pokryszko-Dragan, Justyna Chojdak-Łukasiewicz, Monika Nojszewska, Aleksandra Podlecka-Piętowska, Kinga Sujkowska, Beata Zakrzewska-Pniewska, Jacek Zwiernik, Anna Litwin, Aleksandra Chmielewska, Ihor Kazmierchuk, Magdalena Piwowarska, Beata Szajnoga, Natalia Kliszczak, Sonia Wiercimok, Monika Poziombka-Kaźmierczak, Beata Zwiernik, Marcin Mycko, Tomasz Siwek, Waldemar Brola, Alina Kułakowska

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.

简介:多发性硬化症(MS)是一种导致残疾的进行性疾病,主要影响青壮年。本研究旨在评估社会人口学和临床因素,包括疾病改善疗法(dmt)可及性的变化,对波兰多发性硬化症患者生活质量(QoL)的影响,并将研究结果与早期的国家研究进行比较。材料和方法:2024年2月至4月期间,从波兰19个MS治疗中心的MS患者中收集数据。生活质量采用EuroQol五维五水平(EQ- 5d - 5l)指数和EQ视觉模拟量表(EQ- vas)进行评估。通过标准化问卷调查获得社会人口学和临床数据。结果:纳入3165例MS患者(平均年龄42.03岁;病程:10.6±7.85年;男女比例2.2:1)。EQ-5D-5L平均评分为0.89(±0.15)分,EQ-VAS平均评分为71.58(±18.67)分,表明研究队列总体生活质量为中等至良好。疼痛/不适(80.7%)和焦虑/抑郁(79.6%)是最常见的问题。较高的生活质量评分与较年轻的年龄、复发缓解型MS、较低的残疾率(EDSS≤3.5)、较短的病程、无合并症、就业、城市居住、无子女、DMT使用和不频繁切换DMT相关(p < 0.001)。与波兰早期的研究相比,这些结果表明,在过去十年中,生活质量有了有意义的改善。结论:与一般人群相比,波兰MS患者的生活质量仍然降低。然而,与以前的国家数据相比,已经有了显著的改善。这一趋势可能反映了MS护理的进步,包括更广泛地获得新型dmt和优化的治疗策略。
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引用次数: 0
Use of the serratus anterior muscle flap for reconstruction of an anterior cranial fossa after a gunshot wound. 应用前锯肌瓣重建枪伤后的前颅窝。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-01-01 Epub Date: 2025-10-16 DOI: 10.5603/pjnns.105904
Kristýna Procházková, Vladimír Mařík, Jiří Fiedler
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引用次数: 0
Distinct local brain atrophy pattern in clinically stable multiple sclerosis patients during the peri-pregnancy period: A follow-up study. 妊娠期临床稳定型多发性硬化症患者明显的局部脑萎缩模式:一项随访研究
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-01-01 Epub Date: 2025-10-30 DOI: 10.5603/pjnns.106787
Małgorzata Siger, Mariusz Stasiołek, Halina Bartosik-Psujek, Iwona Rościszewska-Żukowska

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.

研究目的:本研究的目的是评估同一组多发性硬化症(MS)女性在短时间和长时间随访期间的局部脑容量,这是我们之前的研究中在妊娠期(PPP)评估的。研究的临床理由:在我们之前的研究中,我们观察到临床稳定的ms患者在PPP期间丘脑体积(TV)显著减少。在这次随访研究中,我们确定PPP期间观察到的区域脑体积变化是否也是一种长期影响。材料和方法:我们纳入了11例MS患者,并收集了4个时间点的临床和磁共振成像(MRI)数据:时间点1 (TP1)至妊娠前6个月;时间点2 (TP2)至分娩后6个月;时间点3 (TP3) > TP2后12个月,“短期随访”;时间点4 (TP4): TP2后24个月,“长随访”。MRI分析包括T2病变体积、T2LV评估;T1病变体积,T1LV;钆增强病灶数,T1GD+;在每个时间点进行TV和胼胝体体积(CCV)测定。结果:TP3时CCV明显低于TP2时(p = 0.02)。我们还发现TP3时T1LV明显高于TP2时(p = 0.02)。在短暂的随访中,我们观察到CCV的变化略大于初始研究(p = 0.06)。TP4时CCV显著低于TP2, T1LV显著高于TP2 (p = 0.02, p = 0.02)。我们观察到T1LV在从TP1开始的所有时间点显著增加。结论:我们的研究表明,在临床稳定的妊娠期多发性硬化症患者中,尽管重新开始相同的治疗,局部脑萎缩和局灶性病变负担在短期和长期内仍有进展。这些观察结果表明,妊娠对MS病理过程的保护作用是有限的。临床意义:我们的研究结果提供了相关的临床信息。产后早期对MS患者进行治疗时,应考虑局部脑萎缩和局灶性病变负担增加作为决策依据,以防止疾病进展。
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引用次数: 0
Modified Unified Wilson's Disease Rating Scale - scale presentation and pilot clinimetric testing. 改良的统一威尔逊氏病评定量表-量表介绍和临床试验。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-01-01 Epub Date: 2025-11-18 DOI: 10.5603/pjnns.101652
Anna Członkowska, Marta Skowrońska, Petr Dusek, Aurelia Poujois, Chloe Laurencin, Tomasz Litwin, Iwona Kurkowska-Jastrzębska, Grzegorz Chabik

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.

研究目的:统一威尔逊氏病评定量表(UWDRS)于2007年首次引入,用于评估威尔逊氏病(WD)患者的神经功能状态和损害。然而,主要问题已经提出,包括缺乏对精神和精神状态的评估,缺乏对某些运动功能评估的指导,步态和姿势评估的困难,以及跨尺度问题的异质反应选项。研究的临床依据:提出了一种改良版的UWDRS (mUWDRS)。材料和方法:创建了一个新版本的UWDRS,修改了措辞和选项,纳入了运动障碍学会统一帕金森病评定量表(MDS-UPDRS)、统一运动障碍评定量表和泛酸激酶相关神经退行性疾病评定量表(PKAN-DRS)的项目。和原文一样,mUWDRS的第一部分评估了意识的水平。第二部分现在分为两个部分自我报告的子量表:IIa部分评估10项日常生活活动,新的IIb部分评估认知功能、精神和行为状态。第三部分像以前一样评估神经系统状态,但对步态和姿势项目进行了修改。经过2轮认知预测试后,8名神经科医生对7名患者的mUWDRS进行了判据间一致性(Fleiss kappa或Kuder Richardson系数)和内部一致性(Cronbach’s alpha)的验证。结果:在先导测试中,通译者与mUWDRS的一致性几乎是完美或相当的,第一部分的指数值为1,第二部分为0.90,第二部分为0.93,行为状态为0.86,第三部分为0.826。观察到高水平的内部一致性,第二部分的Cronbach's α为0.82,第三部分为0.84。结论和临床意义:mUWDRS旨在评估WD的运动和非运动症状以及功能障碍,在中试中表现良好。由于修改后的WD评定量表具有良好的量表间一致性,故拟进行适当的效度研究。该秤可在https://www.movementdisorders.org/MDS/MDS-Clinical-Outcome-Assessment.htm上获得。
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引用次数: 0
期刊
Neurologia i neurochirurgia polska
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