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The Quality in Quality of Life in Parkinson's Disease: A Qualitative Meta-Synthesis. 帕金森病患者的生活质量:定性元综合。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-05-07 DOI: 10.1002/mdc3.14063
Marlena van Munster, Anna J Pedrosa, Carolin Künkler, David J Pedrosa

Background: Quality of life (QoL) is known to be impaired in people with Parkinson's disease (PwPD). Not surprisingly, a considerable effort of health interventions is aimed at maintaining or improving QoL. Yet, little is known about its determinants from a PwPD perspective to inform person-centered health care interventions.

Objectives: This systematic review aims to overcome this information gap by synthesizing existing evidence on factors associated with PwPD' self-perceived QoL.

Methods: We searched six electronic databases (MEDLINE, EMBASE, PsycINFO, CINAHL, Web of Science, Cochrane Library) from inception to January 2022 for eligible qualitative studies of QoL in PwPD, supplemented by citation tracking and hand searching. Study quality was assessed using the QualSyst tool. In order to characterize the determinants of QoL in PwPD, we conducted a qualitative meta-synthesis.

Results: Our analysis revealed a wide range of facilitators and barriers to QoL relating to seven overarching themes: Illness experience, health care, everyday life, social life, identity, spirituality/religion, and environment.

Conclusions: Our systematic review reinforces the impact of symptom experience on PwPD's QoL. However, it also highlights the need to consider the non-physical dimensions of PD when assessing patients' QoL. It is therefore essential that health care professionals acknowledge the psychological, social and spiritual repercussions of PD and endeavor to respond to these concerns through a comprehensive and patient-centered strategy. Further research is needed to gain a deeper understanding of these facets of PD and to formulate successful interventions aimed at improving the QoL of PwPD.

背景:众所周知,帕金森病患者的生活质量(QoL)会受到影响。因此,为保持或改善生活质量而采取大量健康干预措施也就不足为奇了。然而,从帕金森病患者的角度来看,人们对其决定因素知之甚少,因此无法为以人为本的医疗保健干预措施提供依据:本系统性综述旨在通过综合与残疾人自我感觉 QoL 相关因素的现有证据来填补这一信息空白:我们检索了六个电子数据库(MEDLINE、EMBASE、PsycINFO、CINAHL、Web of Science、Cochrane Library),从开始到 2022 年 1 月,寻找符合条件的关于残疾人 QoL 的定性研究,并辅以引文追踪和手工检索。研究质量采用 QualSyst 工具进行评估。为了确定残疾人 QoL 的决定因素,我们进行了定性元综合:结果:我们的分析揭示了影响 QoL 的各种因素和障碍,这些因素和障碍与七个重要主题有关:结果:我们的分析揭示了影响患者 QoL 的各种因素和障碍,这些因素和障碍涉及七个总体主题:疾病经历、医疗保健、日常生活、社交生活、身份认同、精神/宗教和环境:我们的系统综述加强了症状体验对残疾人 QoL 的影响。然而,它也强调了在评估病人的 QoL 时考虑帕金森病的非身体层面的必要性。因此,医护人员必须认识到帕金森病对心理、社会和精神的影响,并努力通过以患者为中心的综合策略来应对这些问题。我们需要开展进一步的研究,以便更深入地了解帕金森病的这些方面,并制定成功的干预措施,从而改善帕金森病患者的生活质量。
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引用次数: 0
Serum Neurofilament Light and Clinical Biomarkers for Disease Staging in Huntington's Disease. 用于亨廷顿氏症疾病分期的血清神经丝蛋白光和临床生物标记物
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-03-27 DOI: 10.1002/mdc3.13993
Beatrice Heim, Elias Mandler, Arabella Buchmann, Anna Grossauer, Marina Peball, Dora Valent, Federico Carbone, Katarína Schwarzová, Atbin Djamshidian, Philipp Mahlknecht, Michael Khalil, Florian Krismer, Klaus Seppi
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引用次数: 0
Levodopa-Induced Dyskinesias are Frequent and Impact Quality of Life in Parkinson's Disease: A 5-Year Follow-Up Study. 左旋多巴诱发的运动障碍频繁发生并影响帕金森病患者的生活质量:一项为期 5 年的随访研究
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-05-15 DOI: 10.1002/mdc3.14056
Diego Santos-García, Teresa de Deus, Carlos Cores, Maria J Feal Painceiras, María C Íñiguez Alvarado, Lucía B Samaniego, Antón López Maside, Silvia Jesús, Marina Cosgaya, Juan García Caldentey, Nuria Caballol, Ines Legarda, Jorge Hernández-Vara, Iria Cabo López, Lydia López Manzanares, Isabel González-Aramburu, Asunción Ávila, Víctor Gómez-Mayordomo, Víctor Nogueira, Julio Dotor García-Soto, Carmen Borrué-Fernández, Berta Solano, María Álvarez Sauco, Lydia Vela, Sonia Escalante, Esther Cubo, Zebenzui Mendoza, Isabel Pareés, Pilar Sánchez Alonso, Maria G Alonso Losada, Nuria López-Ariztegui, Itziar Gastón, Jaime Kulisevsky, Manuel Seijo, Caridad Valero, Ruben Alonso Redondo, Maria Teresa Buongiorno, Carlos Ordás, Manuel Menéndez-González, Darrian McAfee, Pablo Martinez-Martin, Pablo Mir

Background: Levodopa-induced dyskinesias (LID) are frequent in Parkinson's disease (PD).

Objective: To analyze the change in the frequency of LID over time, identify LID related factors, and characterize how LID impact on patients' quality of life (QoL).

Patients and methods: PD patients from the 5-year follow-up COPPADIS cohort were included. LID were defined as a non-zero score in the item "Time spent with dyskinesia" of the Unified Parkinson's Disease Rating Scale-part IV (UPDRS-IV). The UPDRS-IV was applied at baseline (V0) and annually for 5 years. The 39-item Parkinson's disease Questionnaire Summary Index (PQ-39SI) was used to asses QoL.

Results: The frequency of LID at V0 in 672 PD patients (62.4 ± 8.9 years old; 60.1% males) with a mean disease duration of 5.5 ± 4.3 years was 18.9% (127/672) and increased progressively to 42.6% (185/434) at 5-year follow-up (V5). The frequency of disabling LID, painful LID, and morning dystonia increased from 6.9%, 3.3%, and 10.6% at V0 to 17.3%, 5.5%, and 24% at V5, respectively. Significant independent factors associated with LID (P < 0.05) were a longer disease duration and time under levodopa treatment, a higher dose of levodopa, a lower weight and dose of dopamine agonist, pain severity and the presence of motor fluctuations. LID at V0 (β = 0.073; P = 0.027; R2 = 0.62) and to develop disabling LID at V5 (β = 0.088; P = 0.009; R2 = 0.73) were independently associated with a higher score on the PDQ-39SI.

Conclusion: LID are frequent in PD patients. A higher dose of levodopa and lower weight were factors associated to LID. LID significantly impact QoL.

背景:左旋多巴诱发的运动障碍(LID)在帕金森病(PD)中很常见:分析左旋多巴诱发运动障碍的频率随时间的变化,确定与左旋多巴诱发运动障碍相关的因素,并描述左旋多巴诱发运动障碍对患者生活质量(QoL)的影响:纳入 COPPADIS 5 年随访队列中的帕金森病患者。LID 的定义是在统一帕金森病评分量表第四部分(UPDRS-IV)的 "运动障碍时间 "项目中得分不为零。UPDRS-IV在基线(V0)时使用,5年内每年使用一次。39项帕金森病问卷总结指数(PQ-39SI)用于评估QoL:在平均病程为 5.5 ± 4.3 年的 672 名帕金森病患者(62.4 ± 8.9 岁;60.1% 为男性)中,V0 阶段的 LID 发生率为 18.9% (127/672),随访 5 年(V5)时,LID 发生率逐渐升高至 42.6% (185/434)。致残性 LID、疼痛性 LID 和晨间肌张力障碍的发生率分别从 V0 时的 6.9%、3.3% 和 10.6% 增加到 V5 时的 17.3%、5.5% 和 24%。与LID相关的重要独立因素(P 2 = 0.62)和在V5时发展为致残性LID的独立因素(β = 0.088; P = 0.009; R2 = 0.73)与PDQ-39SI的较高得分相关:结论:LID在帕金森病患者中很常见。结论:LID在帕金森病患者中很常见,左旋多巴剂量较高和体重较轻是导致LID的相关因素。LID严重影响患者的生活质量。
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引用次数: 0
Long-Term Medication Profiles in Parkinson's Disease under Subthalamic Deep Brain Stimulation: A Controlled Study. 眼下深部脑刺激下帕金森病的长期用药概况:对照研究。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-05-07 DOI: 10.1002/mdc3.14065
Christoph Theyer, Vincent Beliveau, Florian Krismer, Marina Peball, Katherina Mair, Beatrice Heim, Atbin Djamshidian, Stefan Kiechl, Wilhelm Eisner, Sabine Eschlböck, Gregor K Wenning, Peter Willeit, Klaus Seppi, Werner Poewe, Philipp Mahlknecht

Background: Subthalamic deep brain stimulation (STN-DBS) reduces antiparkinsonian medications in Parkinson's disease (PD) compared with the preoperative state. Longitudinal and comparative studies on this effect are lacking.

Objective: To compare longitudinal trajectories of antiparkinsonian medication in STN-DBS treated patients to non-surgically treated control patients.

Methods: We collected retrospective information on antiparkinsonian medication from PD patients that underwent subthalamic DBS between 1999 and 2010 and control PD patients similar in age at onset and baseline, sex-distribution, and comorbidities.

Results: In 74 DBS patients levodopa-equivalent daily dose (LEDD) were reduced by 33.9-56.0% in relation to the preoperative baseline over the 14-year observational period. In 61 control patients LEDDs increased over approximately 10 years, causing a significant divergence between groups. The largest difference amongst single drug-classes was observed for dopamine agonists.

Conclusion: In PD patients, chronic STN-DBS was associated with a lower LEDD compared with control patients over 14 years.

背景:与术前状态相比,眼下丘脑下深部脑刺激(STN-DBS)可减少帕金森病(PD)患者的抗帕金森药物用量。目前还缺乏对这一效果的纵向比较研究:比较 STN-DBS 治疗患者与非手术治疗对照组患者服用抗帕金森药物的纵向轨迹:我们收集了1999年至2010年间接受眼下动眼神经DBS治疗的帕金森病患者与发病年龄、基线、性别分布和合并症相似的对照组帕金森病患者服用抗帕金森药物的回顾性信息:在长达14年的观察期内,74名DBS患者的左旋多巴等效日剂量(LEDD)与术前基线相比减少了33.9%-56.0%。61 名对照组患者的 LEDD 在大约 10 年的时间里有所增加,导致组间差异显著。在单一药物类别中,多巴胺受体激动剂的差异最大:结论:在14年的观察期内,与对照组患者相比,帕金森病患者的慢性STN-DBS与较低的LEDD相关。
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引用次数: 0
Improving Conversations about Parkinson's Dementia. 改善有关帕金森痴呆症的对话。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-05-02 DOI: 10.1002/mdc3.14054
Ivelina Dobreva, Joanne Thomas, Anne Marr, Ruairiadh O'Connell, Moïse Roche, Naomi Hannaway, Charlotte Dore, Sian Rose, Ken Liu, Rohan Bhome, Sion Baldwin-Jones, Janet Roberts, Neil Archibald, Duncan Alston, Khaled Amar, Emma Edwards, Jennifer A Foley, Victoria J Haunton, Emily J Henderson, Ashwani Jha, Fiona Lindop, Cathy Magee, Luke Massey, Eladia Ruiz-Mendoza, Biju Mohamed, Katherine Patterson, Bhanu Ramaswamy, Anette Schrag, Monty Silverdale, Aida Suárez-González, Indu Subramanian, Tom Foltynie, Caroline H Williams-Gray, Alison J Yarnall, Camille Carroll, Claire Bale, Cassandra Hugill, Rimona S Weil

Background: People with Parkinson's disease (PD) have an increased risk of dementia, yet patients and clinicians frequently avoid talking about it due to associated stigma, and the perception that "nothing can be done about it". However, open conversations about PD dementia may allow people with the condition to access treatment and support, and may increase participation in research aimed at understanding PD dementia.

Objectives: To co-produce information resources for patients and healthcare professionals to improve conversations about PD dementia.

Methods: We worked with people with PD, engagement experts, artists, and a PD charity to open up these conversations. 34 participants (16 PD; 6 PD dementia; 1 Parkinsonism, 11 caregivers) attended creative workshops to examine fears about PD dementia and develop information resources. 25 PD experts contributed to the resources.

Results: While most people with PD (70%) and caregivers (81%) shared worries about cognitive changes prior to the workshops, only 38% and 30%, respectively, had raised these concerns with a healthcare professional. 91% of people with PD and 73% of caregivers agreed that PD clinicians should ask about cognitive changes routinely through direct questions and perform cognitive tests at clinic appointments. We used insights from the creative workshops, and input from a network of PD experts to co-develop two open-access resources: one for people with PD and their families, and one for healthcare professionals.

Conclusion: Using artistic and creative workshops, co-learning and striving for diverse voices, we co-produced relevant resources for a wider audience to improve conversations about PD dementia.

背景:帕金森病(PD)患者罹患痴呆症的风险增加,但由于相关的耻辱感和 "无能为力 "的观念,患者和临床医生经常避免谈论这一问题。然而,公开谈论帕金森病痴呆症可使患者获得治疗和支持,并可增加对旨在了解帕金森病痴呆症的研究的参与度:为患者和医护人员共同制作信息资源,以改善有关帕金森氏痴呆症的对话:我们与帕金森氏症患者、参与专家、艺术家和一家帕金森氏症慈善机构合作,以开启这些对话。34 名参与者(16 名帕金森病患者;6 名帕金森痴呆症患者;1 名帕金森病患者;11 名护理人员)参加了创意研讨会,探讨对帕金森痴呆症的恐惧并开发信息资源。25 位帕金森病专家为这些信息资源做出了贡献:虽然大多数帕金森病患者(70%)和护理人员(81%)在参加研讨会之前都对认知能力的改变感到担忧,但只有 38% 和 30% 的人向医疗保健专业人员提出过这些担忧。91%的帕金森病患者和 73% 的照护者同意,帕金森病临床医生应通过直接提问的方式例行询问患者的认知变化,并在门诊预约时进行认知测试。我们利用创意工作坊的见解和帕金森病专家网络的意见,共同开发了两份开放式资源:一份供帕金森病患者及其家人使用,另一份供医疗保健专业人员使用:利用艺术和创意工作坊、共同学习和争取不同的声音,我们为更广泛的受众共同制作了相关资源,以改善有关帕金森病痴呆症的对话。
{"title":"Improving Conversations about Parkinson's Dementia.","authors":"Ivelina Dobreva, Joanne Thomas, Anne Marr, Ruairiadh O'Connell, Moïse Roche, Naomi Hannaway, Charlotte Dore, Sian Rose, Ken Liu, Rohan Bhome, Sion Baldwin-Jones, Janet Roberts, Neil Archibald, Duncan Alston, Khaled Amar, Emma Edwards, Jennifer A Foley, Victoria J Haunton, Emily J Henderson, Ashwani Jha, Fiona Lindop, Cathy Magee, Luke Massey, Eladia Ruiz-Mendoza, Biju Mohamed, Katherine Patterson, Bhanu Ramaswamy, Anette Schrag, Monty Silverdale, Aida Suárez-González, Indu Subramanian, Tom Foltynie, Caroline H Williams-Gray, Alison J Yarnall, Camille Carroll, Claire Bale, Cassandra Hugill, Rimona S Weil","doi":"10.1002/mdc3.14054","DOIUrl":"10.1002/mdc3.14054","url":null,"abstract":"<p><strong>Background: </strong>People with Parkinson's disease (PD) have an increased risk of dementia, yet patients and clinicians frequently avoid talking about it due to associated stigma, and the perception that \"nothing can be done about it\". However, open conversations about PD dementia may allow people with the condition to access treatment and support, and may increase participation in research aimed at understanding PD dementia.</p><p><strong>Objectives: </strong>To co-produce information resources for patients and healthcare professionals to improve conversations about PD dementia.</p><p><strong>Methods: </strong>We worked with people with PD, engagement experts, artists, and a PD charity to open up these conversations. 34 participants (16 PD; 6 PD dementia; 1 Parkinsonism, 11 caregivers) attended creative workshops to examine fears about PD dementia and develop information resources. 25 PD experts contributed to the resources.</p><p><strong>Results: </strong>While most people with PD (70%) and caregivers (81%) shared worries about cognitive changes prior to the workshops, only 38% and 30%, respectively, had raised these concerns with a healthcare professional. 91% of people with PD and 73% of caregivers agreed that PD clinicians should ask about cognitive changes routinely through direct questions and perform cognitive tests at clinic appointments. We used insights from the creative workshops, and input from a network of PD experts to co-develop two open-access resources: one for people with PD and their families, and one for healthcare professionals.</p><p><strong>Conclusion: </strong>Using artistic and creative workshops, co-learning and striving for diverse voices, we co-produced relevant resources for a wider audience to improve conversations about PD dementia.</p>","PeriodicalId":19029,"journal":{"name":"Movement Disorders Clinical Practice","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11233842/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140857222","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Improved Sleep Correlates with Improved Quality of Life and Motor Symptoms with Foslevodopa/Foscarbidopa. 睡眠改善与福斯雷多巴/福斯卡比多巴改善生活质量和运动症状相关。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-03-11 DOI: 10.1002/mdc3.14018
K Ray Chaudhuri, Maurizio F Facheris, Bruno Bergmans, Filip Bergquist, Susan R Criswell, Jia Jia, Pavnit Kukreja, Yohei Mukai, Amy M Spiegel, Resmi Gupta, Lars Bergmann, Rajesh Pahwa

Background: Foslevodopa/foscarbidopa is a subcutaneous infusion of levodopa/carbidopa prodrugs.

Objectives: Assess correlations between sleep and efficacy from interim data of a phase 3 trial of foslevodopa/foscarbidopa (NCT03781167).

Methods: Pearson correlations between sleep (Parkinson's Disease Sleep Scale-2 [PDSS-2]) and quality of life (QoL; Parkinson's Disease Questionnaire-39), motor experiences of daily living (m-EDL; Movement Disorder Society-Unified Parkinson's Disease Scale Part II), and "Off"/"On" times were calculated for baseline and week 26 improvements. Regression analyses were adjusted for baseline PDSS-2 score.

Results: Baseline sleep correlated moderately with QoL (r = 0.44, P < 0.001) and weakly with m-EDL (r = 0.28; P < 0.001). Sleep improvement weakly correlated with improved "Off" time (r = 0.37; P < 0.001) and QoL (r = 0.36; P < 0.001). Regression analyses demonstrated significant positive associations for improved sleep, "Off" time, QoL, and m-EDL.

Conclusions: Improved sleep with foslevodopa/foscarbidopa was associated with improved QoL and "Off" time.

背景福斯莱多巴/福斯卡比多巴是一种左旋多巴/卡比多巴原药的皮下注射剂:根据福斯莱多巴/福斯卡比多巴 3 期试验(NCT03781167)的中期数据,评估睡眠与疗效之间的相关性:计算睡眠(帕金森病睡眠量表-2 [PDSS-2])与生活质量(QoL;帕金森病问卷-39)、日常生活运动体验(m-EDL;运动障碍协会-统一帕金森病量表第二部分)以及 "关"/"开 "时间之间的皮尔逊相关性,以了解基线和第26周的改善情况。回归分析对基线 PDSS-2 评分进行了调整:结果:基线睡眠与 QoL 有中度相关性(r = 0.44,P 结论:基线睡眠与 QoL 有中度相关性:使用磷左多巴/磷卡比多巴改善睡眠与改善 QoL 和 "关闭 "时间有关。
{"title":"Improved Sleep Correlates with Improved Quality of Life and Motor Symptoms with Foslevodopa/Foscarbidopa.","authors":"K Ray Chaudhuri, Maurizio F Facheris, Bruno Bergmans, Filip Bergquist, Susan R Criswell, Jia Jia, Pavnit Kukreja, Yohei Mukai, Amy M Spiegel, Resmi Gupta, Lars Bergmann, Rajesh Pahwa","doi":"10.1002/mdc3.14018","DOIUrl":"10.1002/mdc3.14018","url":null,"abstract":"<p><strong>Background: </strong>Foslevodopa/foscarbidopa is a subcutaneous infusion of levodopa/carbidopa prodrugs.</p><p><strong>Objectives: </strong>Assess correlations between sleep and efficacy from interim data of a phase 3 trial of foslevodopa/foscarbidopa (NCT03781167).</p><p><strong>Methods: </strong>Pearson correlations between sleep (Parkinson's Disease Sleep Scale-2 [PDSS-2]) and quality of life (QoL; Parkinson's Disease Questionnaire-39), motor experiences of daily living (m-EDL; Movement Disorder Society-Unified Parkinson's Disease Scale Part II), and \"Off\"/\"On\" times were calculated for baseline and week 26 improvements. Regression analyses were adjusted for baseline PDSS-2 score.</p><p><strong>Results: </strong>Baseline sleep correlated moderately with QoL (r = 0.44, P < 0.001) and weakly with m-EDL (r = 0.28; P < 0.001). Sleep improvement weakly correlated with improved \"Off\" time (r = 0.37; P < 0.001) and QoL (r = 0.36; P < 0.001). Regression analyses demonstrated significant positive associations for improved sleep, \"Off\" time, QoL, and m-EDL.</p><p><strong>Conclusions: </strong>Improved sleep with foslevodopa/foscarbidopa was associated with improved QoL and \"Off\" time.</p>","PeriodicalId":19029,"journal":{"name":"Movement Disorders Clinical Practice","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11233834/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140094384","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Atypical Presentations of Huntington Disease-like 2 in South African Individuals. 南非人亨廷顿病样 2 的非典型表现
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-05-09 DOI: 10.1002/mdc3.14052
Heena Narotam-Jeena, Mark Guttman, Ludo van Hillegondsberg, Riaan van Coller, Amanda Krause, Jonathan Carr

Background: Huntington disease-like 2 (HDL2) is a neurodegenerative disorder, affecting only individuals of African ancestry. Full penetrance occurs in individuals with 40 repeats or more.

Objective: To describe the phenotypic variability of HDL2 in a group of mixed ancestry individuals from South Africa.

Methods: Eight patients were assessed with analysis of repeat size and magnetic resonance brain imaging. We applied the Unified Huntington's Disease Rating Scale (UHDRS), but in deceased patients (4), this was estimated from video material.

Results: Cognitive domains were more severely affected than motor; UHDRS motor scores were notable for bradykinesia, and to a slightly lesser extent, for rigidity and dystonia; a single patient had marked chorea. Repeat lengths ranged from 45 to 63 (median, 52).

Conclusion: This South African group of mixed ancestry HDL2 individuals presented with severe cognitive and behavioral impairments, with lesser degrees or absence of chorea. This presentation is possibly related to large repeat sizes.

背景:亨廷顿病样 2(HDL2)是一种神经退行性疾病,仅影响非洲血统的个体。具有 40 个或更多重复序列的个体具有完全的渗透性:描述南非一组混血儿 HDL2 的表型变异性:对八名患者进行了重复大小分析和脑磁共振成像评估。我们采用了亨廷顿氏病统一评定量表(UHDRS),但已故患者(4 人)的评定量表是根据视频资料估算的:结果:认知领域受到的影响比运动领域严重;UHDRS运动评分以运动迟缓为主,其次是僵直和肌张力障碍;一名患者有明显的舞蹈症。重复长度从 45 到 63 不等(中位数为 52):结论:这组南非混血 HDL2 患者有严重的认知和行为障碍,但舞蹈症程度较轻或没有舞蹈症。这种表现可能与重复序列大有关。
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引用次数: 0
In Memoriam Gregor K. Wenning (1964-2024). 悼念格雷戈尔-K-温宁(1964-2024)。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-07-04 DOI: 10.1002/mdc3.14134
Werner Poewe, Horacio Kaufmann, Niall Quinn
{"title":"In Memoriam Gregor K. Wenning (1964-2024).","authors":"Werner Poewe, Horacio Kaufmann, Niall Quinn","doi":"10.1002/mdc3.14134","DOIUrl":"10.1002/mdc3.14134","url":null,"abstract":"","PeriodicalId":19029,"journal":{"name":"Movement Disorders Clinical Practice","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11233833/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141498524","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Movement Disorders in Patients with Subacute Sclerosing Panencephalitis: A Systematic Review. 亚急性硬化性泛脑炎患者的运动障碍:系统综述。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-05-15 DOI: 10.1002/mdc3.14062
Divyani Garg, Sahil Patel, Charulata S Sankhla, Vikram V Holla, Vijayashankar Paramanandam, Prashanth L Kukkle, Sanjay Pandey, Susanne A Schneider, Pramod K Pal

Background: Subacute sclerosing panencephalitis (SSPE) is a complication of measles, occurring after a latency of 4-10 years. It continues to occur in developing countries although resurgence is being reported from developed countries. Characteristic features include progressive neuropsychiatric issues, myoclonus, seizures, movement disorders and visual impairment. Electroencephalography (EEG) typically shows periodic generalized discharges, and elevated CSF anti-measles antibodies are diagnostic. Movement disorders are being increasingly recognized as part of the clinical spectrum, and range from hyperkinetic (chorea, dystonia, tremor, tics) to hypokinetic (parkinsonism) disorders and ataxia.

Objectives: This article aims to comprehensively review the spectrum of movement disorders associated with SSPE.

Methods: A literature search was conducted in PubMed and EMBASE databases in December 2023 and articles were identified for review.

Results: Movement disorders reported in SSPE included hyperkinetic (chorea, dystonia, tremor and tics), hypokinetic (parkinsonism), ataxia and extraocular movement disorders. Myoclonus, a core clinical feature, was the most frequent "abnormal movement." Movement disorders were observed in all clinical stages, and could also be a presenting feature, even sans myoclonus. Hyperkinetic movement disorders were more common than hypokinetic movement disorders. An evolution of movement disorders was observed, with ataxia, chorea and dystonia occurring earlier, and parkinsonism later in the disease. Neuroradiological correlates of movement disorders remained unclear.

Conclusion: A wide spectrum of movement disorders was observed throughout the clinical stages of SSPE. Most data were derived from case reports and small case series. Multicentric longitudinal studies are required to better delineate the spectrum and evolution of movement disorders in SSPE.

背景:亚急性硬化性泛脑炎(SSPE)是麻疹的一种并发症,潜伏期为 4-10 年。尽管有报道称这种疾病在发达国家死灰复燃,但在发展中国家仍有发生。其特征包括进行性神经精神问题、肌阵挛、癫痫发作、运动障碍和视力障碍。脑电图(EEG)通常会显示周期性全身放电,脑脊液中抗麻疹抗体升高可作为诊断依据。越来越多的人认识到运动障碍是临床疾病谱的一部分,其范围从运动过度(舞蹈症、肌张力障碍、震颤、抽搐)到运动不足(帕金森氏症)和共济失调:本文旨在全面综述与 SSPE 相关的运动障碍:方法:2023 年 12 月在 PubMed 和 EMBASE 数据库中进行了文献检索,并确定了可供综述的文章:报告的SSPE运动障碍包括运动机能亢进(舞蹈症、肌张力障碍、震颤和抽搐)、运动机能亢进(帕金森病)、共济失调和眼外运动障碍。肌阵挛是一种核心临床特征,也是最常见的 "异常运动"。在所有临床阶段都可观察到运动障碍,即使没有肌阵挛,运动障碍也可能是一种表现特征。运动功能亢进症比运动功能低下症更为常见。患者的运动障碍会发生演变,共济失调、舞蹈症和肌张力障碍出现得较早,而帕金森病则出现得较晚。运动障碍的神经放射学相关性仍不清楚:结论:在 SSPE 的各个临床阶段都可观察到各种运动障碍。大多数数据来自病例报告和小型病例系列。需要进行多中心纵向研究,以更好地界定SSPE运动障碍的谱系和演变。
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引用次数: 0
Deep Brain Stimulation in Patients with Cardiac Implantable Electronic Devices: Pulse Width Pitfalls. 植入心脏电子设备患者的脑深部刺激:脉冲宽度陷阱。
IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-05-23 DOI: 10.1002/mdc3.14066
Jongmok Ha, Dongyeong Lee, Jong Hyeon Ahn, Jin Whan Cho, Jung Il Lee, Alfonso Fasano, Seung Jung Park, Jinyoung Youn
{"title":"Deep Brain Stimulation in Patients with Cardiac Implantable Electronic Devices: Pulse Width Pitfalls.","authors":"Jongmok Ha, Dongyeong Lee, Jong Hyeon Ahn, Jin Whan Cho, Jung Il Lee, Alfonso Fasano, Seung Jung Park, Jinyoung Youn","doi":"10.1002/mdc3.14066","DOIUrl":"10.1002/mdc3.14066","url":null,"abstract":"","PeriodicalId":19029,"journal":{"name":"Movement Disorders Clinical Practice","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11233839/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141081528","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
Movement Disorders Clinical Practice
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