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A cello concert in Brazilian lands: the life, art, and disease of Jacqueline du Pré. 巴西土地上的大提琴音乐会:杰奎琳·杜·普莱尔的生活、艺术和疾病。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-09-01 Epub Date: 2025-09-19 DOI: 10.1055/s-0045-1811726
Diogo Haddad-Santos, Filipi Fim Andreão, Filipe Virgilio Ribeiro, Karlos Daniell Araújo Dos Santos, Fernanda Herculano, Guilherme Sciascia do Olival, Rafael Paterno Dias Carneiro

The present paper explores the extraordinary life of cellist Jacqueline du Pré, her remarkable contribution to music, and her battle with multiple sclerosis (MS). Beyond her artistic impact, we discuss how her story influenced the creation of the Brazilian Multiple Sclerosis Association (Associação Brasileira de Esclerose Múltipla, ABEM, in Portuguese) and its cultural significance in Brazil, particularly through the play Duet for One, by Tom Kempinski. The study reflects on the role of art as a powerful tool to raise awareness and contribute to the understanding of diseases.

本文探讨了大提琴家杰奎琳·杜普莱尔非凡的一生,她对音乐的卓越贡献,以及她与多发性硬化症(MS)的斗争。除了她的艺术影响之外,我们还讨论了她的故事如何影响巴西多发性硬化症协会(associa o Brasileira de Esclerose Múltipla, ABEM,葡萄牙语)的成立,以及该协会在巴西的文化意义,特别是通过汤姆·凯宾斯基的戏剧《一个人的双人舞》。这项研究反映了艺术作为提高认识和促进了解疾病的有力工具的作用。
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引用次数: 0
Cerebellar syndromes: clinical observations leading to the recognition of the three types. 小脑综合征:辨证论治的临床观察。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-09-01 Epub Date: 2025-09-19 DOI: 10.1055/s-0045-1811727
Mario Manto, Hiroshi Mitoma

Cerebellar syndrome is traditionally categorized into three primary types: cerebellar motor syndrome (CMS), vestibulocerebellar syndrome (VCS), and cerebellar cognitive affective syndrome (CCAS) or Schmahmann syndrome (SS). The first type is subdivided into five elemental features: dysmetria, kinetic tremor, asynergia, adiadochokinesis and dyschronometria. The second is characterized by dysmetria of saccades and jerky pursuit, as well as downbeat nystagmus and gaze-evoked nystagmus. And the third type is associated with a broader spectrum of cognitive and affective symptoms, including impairments in executive function, spatial cognition, language processing and emotional regulation. In its extreme form, cerebellar mutism can also develop during childhood following cerebellar vermis surgery. Recent physiological studies have shed light on the underlying neural mechanisms of these syndromes by identifying a common link of dysfunction within the cerebellum's internal forward model. This is essential to the prediction of the outcomes of motor and cognitive actions and underlines dysmetria as the core common element. Despite the diversity in clinical presentation, cerebellar syndromes can be understood as disruptions of a unified neural mechanism, providing a new framework for better understanding of cerebellar deficits.

小脑综合征传统上分为三种主要类型:小脑运动综合征(CMS)、前庭小脑综合征(VCS)和小脑认知情感综合征(CCAS)或Schmahmann综合征(SS)。第一种类型可细分为五个基本特征:节律障碍、运动性震颤、失能、运动障碍和计时障碍。第二阶段的特征是扫视和快速追逐的不对称,以及轻拍性眼震和凝视诱发性眼震。第三种类型与更广泛的认知和情感症状有关,包括执行功能、空间认知、语言处理和情绪调节方面的障碍。在极端的情况下,小脑性缄默症也可以在小脑蚓手术后的儿童时期发展。最近的生理学研究通过确定小脑内部正向模型中功能障碍的共同联系,揭示了这些综合征的潜在神经机制。这对于预测运动和认知行为的结果至关重要,并强调了不对称是核心的共同因素。尽管临床表现多样,但小脑综合征可以理解为统一神经机制的破坏,为更好地理解小脑缺陷提供了新的框架。
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引用次数: 0
Social and occupational functioning scale for epilepsy: performance in Brazilian adult patients with epilepsy. 癫痫的社会和职业功能量表:巴西成年癫痫患者的表现。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-09-01 Epub Date: 2025-10-31 DOI: 10.1055/s-0045-1811721
Gloria M A S Tedrus, Danilo Wingeter Ramalho, Elisa Dal Rio Teixeira

Epilepsy is a condition that can lead to social difficulties and restrictions.To evaluate social and occupational functioning and relationships with clinical findings of Brazilian adult patients with epilepsy.The scores of the Social and Occupational Functioning Scale for Epilepsy (SOFSE) were related to the clinical data of adult patients with epilepsy.Among the 79 cases included, with a mean age of 44.3 years old, 47 were employed and 32 were unemployed. It was observed that there was a predominance of males among the employed patients (32 [68.1%] versus 15 [31.9]; p = 0.008), most patients had a companion (27 [57.4%] versus 20 [42.6%]; p = 0.005), and most patients had no anxiety symptoms on the Hospital Anxiety and Depression Scale (HADS) (30 [63.8%] versus 17 [36.2%]; p = 0.011). The total score in the SOFSE was 64.7, with a higher total score among the employed patients (56.6 ± 12.5 versus 70.3 ± 14.3; p < 0.001). There was a correlation between the total SOFSE score with formal education (r = 0.30) and with HADS-anxiety (r = - 0.56) and HADS-depression (r = - 0.36). Lower scores in the communication domain were associated with a high frequency of seizures. There were lower scores in the domain leisure activity in TLE (2.0 ± 2.0 versus. 2.2 ± 1.5; p = 0.014).The unemployment rate was high in adults with epilepsy. Employment was associated with male gender, having a companion, and absence of anxiety symptoms. Better functional adjustment was associated with schooling. Social and occupational functional impairment was associated with a high frequency of seizures, TLE, and anxious and depressive symptoms.

癫痫是一种可能导致社交困难和限制的疾病。评估巴西成年癫痫患者的社会和职业功能及其与临床表现的关系。成人癫痫患者的社会与职业功能量表(SOFSE)得分与临床资料相关。在79例中,平均年龄为44.3岁,就业47例,失业32例。结果发现,受雇患者中男性占主导地位(32人[68.1%]对15人[31.9],p = 0.008),大多数患者有伴侣(27人[57.4%]对20人[42.6%],p = 0.005),大多数患者在医院焦虑抑郁量表(HADS)中无焦虑症状(30人[63.8%]对17人[36.2%],p = 0.011)。SOFSE总分为64.7分,在职组患者总分更高(56.6±12.5比70.3±14.3,p = 0.014)。成人癫痫患者的失业率很高。就业与男性、有伴侣和没有焦虑症状有关。更好的功能调整与上学有关。社交和职业功能障碍与高频率的癫痫发作、TLE、焦虑和抑郁症状相关。
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引用次数: 0
Patient journey and treatment pattern in myasthenia gravis: real-world data from the Brazilian public health system. 重症肌无力的患者旅程和治疗模式:来自巴西公共卫生系统的真实数据。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-09-01 Epub Date: 2025-10-27 DOI: 10.1055/s-0045-1811720
Renata Andrade, Wilson Marques Junior, Luiza Vasconcelos, Paula Pungartnik, Thaís Zamboni Berra, Lucas Vieira Cortez, Daiane Beneduzzi

Myasthenia gravis (MG) is a rare neurological disease and the most common autoimmune disorder, characterized by muscle weakness. In Brazil, there is limited data on these patients within the Brazilian public healthcare system (SUS, from Sistema Único de Saúde, in Portuguese).To assess patients with MG in the Brazilian public healthcare system to understand their characteristics, patient journey, and treatment patterns.A retrospective observational study using real-world data from SUS to analyze patients with MG from January 2010 to December 2023. Data were extracted from the DATASUS, focusing on the Outpatient Procedure (SIA) and Hospital Admissions (SIH) information systems. Probabilistic record linkage compiled longitudinal patient data, assessing epidemiology, demographics, clinical characteristics, and treatment patterns, including medications, doses, and lines of treatment (LOT).A total of 13,476 patients with MG were identified. Admissions in healthcare units increased over the years, with 30.4% experiencing exacerbations and 9% crises. The mean age was 45.6 years, with a majority being female (65.4%) and white (53.4%). The case-fatality rate rose from 0.76% in 2011 to 1.90% in 2023. Treatment patterns showed frequent transitions between LOT, indicating constant instability and inadequate symptom control, with azathioprine and pyridostigmine being the most used medications. Some patients started treatment with intravenous immunoglobulin (IVIg), used in combination with other medications, and continued it continuously.The study highlights the increasing burden of MG on SUS, emphasizing the challenges of disease management and the need for continuous advancements in diagnostic and therapeutic strategies to improve patient outcomes.

重症肌无力(MG)是一种罕见的神经系统疾病和最常见的自身免疫性疾病,其特征是肌肉无力。在巴西,巴西公共卫生系统(SUS,来自Sistema Único de Saúde,葡萄牙语)中关于这些患者的数据有限。评估巴西公共医疗保健系统中MG患者,了解他们的特点、患者旅程和治疗模式。一项回顾性观察性研究,使用SUS的真实数据分析2010年1月至2023年12月MG患者。数据从DATASUS中提取,重点是门诊程序(SIA)和住院(SIH)信息系统。概率记录链接汇编了纵向患者数据,评估流行病学、人口统计学、临床特征和治疗模式,包括药物、剂量和治疗线(LOT)。共有13476例MG患者被确定。多年来,到医疗机构就诊的人数有所增加,其中30.4%出现病情恶化,9%出现危机。平均年龄45.6岁,以女性(65.4%)和白人(53.4%)居多。病死率从2011年的0.76%上升到2023年的1.90%。治疗模式显示LOT之间的频繁转换,表明持续不稳定和症状控制不足,其中硫唑嘌呤和吡哆斯的明是最常用的药物。一些患者开始使用静脉注射免疫球蛋白(IVIg)治疗,与其他药物联合使用,并持续使用。该研究强调了MG对SUS的负担日益增加,强调了疾病管理的挑战以及不断改进诊断和治疗策略以改善患者预后的必要性。
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引用次数: 0
Measures of clinical effect in vestibular rehabilitation. 前庭康复的临床效果评价。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-09-01 Epub Date: 2025-10-31 DOI: 10.1055/s-0045-1806831
Gabriela Ramos Ventura, Maristela Linhares Dos Santos, Joubert Vitor de Souto Barbosa, Emannuel Alcides Bezerra Rocha, Gerônimo Bouza Sanchis, Rafael Limeira Cavalcanti, Marcello Barbosa Ottoni Gonçalves Guedes, Johnnatas Mikael Lopes
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引用次数: 0
The effect of proprioceptive vestibular rehabilitation on sensory-motor symptoms and quality of life. 本体感觉前庭康复对感觉运动症状和生活质量的影响。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-09-01 Epub Date: 2026-01-28 DOI: 10.1055/s-0045-1806832
Partiksha, Sakshi Sadhu
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引用次数: 0
Postictal status and clinical variables in adults with epilepsy. 成人癫痫患者的术后状态和临床变量。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-09-01 Epub Date: 2025-09-08 DOI: 10.1055/s-0045-1811625
Cecília Fusco Agostinho, Glória M A S Tedrus

Symptoms in the postictal period are often neglected by professionals and patients/family members.To relate the characteristics of the postictal period with the clinical variables of adult patients with epilepsy.Prospectively, the clinical characteristics of the postictal period were related to the clinical variables and the scores on the Hospital Anxiety and Depression Scale (HADS) and Mini-Mental State Examination (MMSE) of 70 patients with epilepsy.The mean age was 47.6 years old, and 34 (48.6%) patients were male, the mean age at onset of epilepsy was 20.3 years old, the epileptic syndrome was structural in 48 cases, the mean score in the MMSE was 23.6, the mean score in the HADS-anxiety was 7.3, and in the HADS-depression it was 5.6. Postictal manifestation occurred in 59 (84.2%) cases, with prolonged duration in 22 (37.2%) cases. There was a difference in the duration and presence of postictal manifestation according to age at the onset of epilepsy. Higher HADS-anxiety scores were associated with the presence of confusion in the postictal period (t-test; 9.3 ± 6.8 versus 5.8 ± 4.8; p = 0.015). Lower MMSE scores were related to postictal anxiety and sleepiness/headache/confusion. Left-sided epileptiform activity (EA) was associated with postictal sleepiness/headache/confusion. There was a difference in postictal manifestation according to epileptic syndrome.The characteristics of the postictal period differed according to the age of onset, the type of seizure, and the epileptic syndrome. Different symptoms in the postictal period were associated with the scores on HADS anxiety, MMSE, and left-sided EA on the electroencephalogram.

感染后的症状往往被专业人员和患者/家属所忽视。目的探讨成人癫痫患者后发期特征与临床变量的关系。前瞻性分析70例癫痫患者的临床特征与临床变量及医院焦虑抑郁量表(HADS)和简易精神状态检查(MMSE)得分相关。平均年龄47.6岁,男性34例(48.6%),平均癫痫发病年龄20.3岁,癫痫综合征结构性48例,MMSE平均得分23.6分,hads -焦虑平均得分7.3分,hads -抑郁平均得分5.6分。术后表现59例(84.2%),持续时间延长22例(37.2%)。在持续时间和存在的阳性表现,根据年龄在癫痫发作的差异。较高的hads焦虑评分与术后出现精神错乱相关(t检验;9.3±6.8比5.8±4.8;p = 0.015)。较低的MMSE得分与术后焦虑、嗜睡/头痛/意识混乱有关。左侧癫痫样活动(EA)与术后嗜睡/头痛/意识不清有关。根据癫痫综合征的不同,阳性表现有差异。发病年龄、发作类型和癫痫综合征不同,其发病后期特征也不同。术后不同症状与HADS焦虑、MMSE和左侧EA的脑电图得分相关。
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引用次数: 0
Erdheim-Chester manifesting purely as a neurological disease. 厄德海姆-切斯特症候群纯粹表现为神经系统疾病
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-09-01 Epub Date: 2025-06-21 DOI: 10.1055/s-0045-1809403
Lídia Laura Salvador Ramos, Iago Resende Carvalho, Diogo Fernandes Dos Santos, Andrea de Martino Luppi, Bruno de Carvalho Dornelas
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引用次数: 0
Assessment of possible small fiber Neuropathy in early-stage vitamin B12 deficiency using electrophysiological methods. 利用电生理方法评估早期维生素B12缺乏症可能的小纤维神经病变。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-09-01 Epub Date: 2025-09-19 DOI: 10.1055/s-0045-1811722
Elif Simin Issi

Small fiber neuropathy (SFN) affects thinly myelinated and unmyelinated fibers, often presenting with subtle clinical signs that are undetectable in routine nerve conduction studies. Vitamin B12 deficiency is a known risk factor for SFN, yet early-stage cases frequently remain undiagnosed. Sympathetic skin response (SSR) and cutaneous silent period (CSP) are noninvasive electrophysiological techniques used to assess autonomic and somatic small fiber function.The present study aimed to evaluate the diagnostic utility of SSR and CSP in detecting possible subclinical small-fiber neuropathy (pSFN) in individuals with early-stage vitamin B12 deficiency.The present observational study included 28 patients with vitamin B12 deficiency who had nonspecific complaints, Douleur Neuropathique en 4 Questions (DN4) scores < 4, and normal nerve conduction studies, along with 25 healthy controls. Electrophysiological testing involved SSR recordings from all extremities and CSP measurements from the right median and sural nerves.In the patient group, Median Nerve Cutaneous Silent Period (MN-CSP) and Tibialis Anterior -Sural Cutaneous Silent Period (TA-sural CSP) durations were significantly shorter, while termination and onset latencies were prolonged compared with controls. MN-CSP and TA-sural CSP durations demonstrated high diagnostic accuracy. Sympathetic skin response latencies were significantly prolonged in both hands and feet, indicating autonomic dysfunction. No significant differences were observed in SSR amplitudes.Sympathetic skin response and CSP are valuable tools for detecting possible subclinical SFN in vitamin B12 deficiency. Sympathetic skin response effectively identified autonomic dysfunction, while CSP provided additional diagnostic value for somatic small fiber impairment. Combining SSR and CSP may enhance early detection of pSFN in vitamin B12 deficiency and allow timely intervention.

小纤维神经病(SFN)影响薄髓鞘和无髓鞘纤维,通常表现出常规神经传导检查无法检测到的微妙临床体征。维生素B12缺乏是SFN的一个已知危险因素,但早期病例经常未被诊断出来。交感皮肤反应(SSR)和皮肤沉默期(CSP)是用于评估自主神经和躯体小纤维功能的无创电生理技术。本研究旨在评价SSR和CSP检测早期维生素B12缺乏症患者可能的亚临床小纤维神经病变(pSFN)的诊断价值。目前的观察性研究包括28例维生素B12缺乏症患者,他们有非特异性的主诉,双重神经病变4个问题(DN4)评分
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引用次数: 0
Calcitonin gene-related peptide monoclonal antibodies and medication-overuse headache: stopping excessive pain medication is still necessary. 降钙素基因相关肽单克隆抗体与药物过度使用头痛:停止过度止痛药仍然是必要的。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-09-01 Epub Date: 2025-07-28 DOI: 10.1055/s-0045-1809332
Pedro Augusto Sampaio Rocha-Filho

Medication-overuse headache affects 1 to 2% of the global population and is often associated with chronic migraine. This condition significantly impacts the lives of patients, as well as their families, and it poses a major economic burden due to lost productivity and medical costs. The present narrative review is part of a controversy session. We argue that reversing the behavior of overusing symptomatic pain medications is important for the treatment of this type of headache. To support this argument, the article reviews and critically analyzes the relevant literature on the subject.

药物过度使用头痛影响全球人口的1%至2%,通常与慢性偏头痛有关。这种情况严重影响患者及其家属的生活,并由于生产力损失和医疗费用造成重大经济负担。目前的叙述性回顾是一个有争议的会议的一部分。我们认为,扭转过度使用对症止痛药的行为对治疗这种类型的头痛很重要。为了支持这一论点,本文回顾并批判性地分析了有关这一主题的相关文献。
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引用次数: 0
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Arquivos de neuro-psiquiatria
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