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Video-Based Pairwise Comparison: Enabling the Development of Automated Rating of Motor Dysfunction in Multiple Sclerosis.

基于视频的成对比较: 能够开发多发性硬化运动功能障碍的自动评级。

  • 影响因子:3.03
  • DOI:10.1016/j.apmr.2019.07.016
  • 作者列表:"Burggraaff J","Dorn J","D'Souza M","Morrison C","Kamm CP","Kontschieder P","Tewarie P","Steinheimer S","Sellen A","Dahlke F","Kappos L","Uitdehaag B
  • 发表时间:2020-02-01
Abstract

OBJECTIVES:To examine the feasibility, reliability, granularity, and convergent validity of a video-based pairwise comparison technique that uses algorithmic support to enable automated rating of motor dysfunction in patients with multiple sclerosis (MS). DESIGN:Feasibility and larger cross-sectional cohort study. SETTING:The outpatient clinic of 2 specialist university medical centers. PARTICIPANTS:Selected sample from a cohort of patients with MS participating in the Assess MS study (N=42). Videos were randomly drawn from each strata of the ataxia severity-degrees as defined in the Expanded Disability Status Scale (EDSS). In Basel: 19 videos of 17 patients (mean age, 43.4±11.6y; 10 women). In Amsterdam: 50 videos of 25 patients (mean age, 50.0±10.0y; 15 women). INTERVENTIONS:Not applicable. MAIN OUTCOME MEASURES:In each center, neurologists (n=13; n=10) viewed pairs of videos of patients performing standardized movements (eg, finger-to-nose test) to assess relative performance. A comparative assessment score was calculated for each video using the TrueSkill algorithm and analyzed for intrarater (test-retest; ratio of agreement) and interrater reliability (intraclass correlation coefficient [ICC] for absolute agreement) and convergent validity (Spearman ρ). Granularity was estimated from the average difference in comparative assessment scores at which 80% of neurologists considered performance to be different. RESULTS:Intrarater reliability was excellent (median ratio of agreement≥0.87). The comparative assessment scores calculated from individual neurologists demonstrated good-excellent ICCs for interrater reliability (0.89; 0.71). The comparative assessment scores correlated (very) highly with their Neurostatus-EDSS equivalent (ρ=0.78, P<.001; ρ=0.91, P<.05), suggesting a more fine-grained rating. CONCLUSIONS:Video-based pairwise comparison of motor dysfunction allows for reliable and fine-grained capturing of clinical judgment about neurologic performance, which can contribute to the development of a consistent quantified metric of motor ability in MS.

摘要

目的: 检查的可行性,可靠性,粒度,和基于视频的成对比较技术的收敛效度,该技术使用算法支持实现多发性硬化 (MS) 患者运动功能障碍的自动评级。 设计: 可行性和更大的横断面队列研究。 单位: 2 个专科大学医疗中心的门诊。 参与者: 从参与评估MS研究的MS患者队列中选择样本 (N = 42)。从扩展残疾状态量表 (EDSS) 中定义的共济失调严重程度的每个分层随机抽取视频。巴塞尔: 17 例患者的 19 个视频 (平均年龄,43.4 ± 11.6y; 10 例女性)。阿姆斯特丹: 25 例患者的 50 个视频 (平均年龄,50.0 ± 10.0y; 15 例女性)。 干预措施: 不适用。 主要观察指标: 在每个中心,神经科医生 (n = 13; n = 10) 观看了对患者进行标准化运动 (例如,手指对鼻子测试) 的视频评估相对性能。使用TrueSkill算法计算每个视频的比较评估分数,并分析内部评分 (测试-重测; 协议比率) 和评定者间信度 (绝对协议的组内相关系数 [ICC]) 和会聚效度 (Spearman ρ)。根据比较评估评分的平均差异估计粒度,80% 的神经科医生认为表现不同。 结果: 量表内信度为优良 (中位数比值 ≥ 0.87)。由个别神经科医生计算的比较评估得分显示出良好的优秀ICCs的评定者间信度 (0.89; 0.71)。比较评估分数与其神经状态-EDSS当量 (ρ = 0.78,P <) 高度相关。001; Ρ = 0.91,P<.05),提示更细粒度的评级。 结论: 基于视频的运动功能障碍成对比较可以可靠和细粒度地捕获关于神经功能表现的临床判断,这有助于开发一致的MS运动能力量化指标。

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影响因子:1.52
发表时间:2020-04-02
DOI:10.1080/09273948.2019.1597896
作者列表:["Apivatthakakul A","Kunavisarut P","Rothova A","Pathanapitoon K"]

METHODS::Purpose: To report on ocular Vogt-Koyanagi-Harada (VKH)-like syndrome under vemurafenib treatment for metastatic melanoma.Design: A case report.Method: Description of clinical and imaging manifestations including fundus photography, fluorescein, and indocyanine green angiography.Results: A 46-year-old Thai female was diagnosed with metastatic melanoma of the skin and had been treated with multiple surgical excisions, radiotherapy, and vemurafenib (initial dose 480 mg orally twice daily, subsequently increased to maximum dose of 960 mg twice daily). After 6 months of vemurafenib use, she complained of bilateral redness and photophobia and was diagnosed with bilateral anterior uveitis, which was topically treated. Two weeks later, her visual acuity (VA) sharply deteriorated to 20/80 and counting fingers. Ocular examination at that stage stronly resembled acute VKH disease. She exhibited intraocular inflammation, and her fundus examination revealed bilateral optic disc swelling and serous retinal detachment. Fluorescein angiogram showed disc leakage and multiple pinpoint hyperfluorescence leakage spots and indocyanine green demonstrated multiple hypofluorescent spots. Oral prednisolone 30 mg/day was commenced while vemurafenib medication was ceased. Three weeks later, her vision improved, and serous retinal detachment subsided. However, her cutaneous melanoma recurred.Conclusions: Vemurafenib, a potential adjunct treatment for metastatic melanoma, was complicated by the development of panuveitis, papillitis, and multiple serous detachments. These ocular symptoms were similar to the presentation of acute VKH syndrome.

翻译标题与摘要 下载文献
影响因子:2.19
发表时间:2020-01-01
DOI:10.1111/dmcn.14268
作者列表:["Crow YJ","Shetty J","Livingston JH"]

METHODS::Comprehensive reviews of the clinical characteristics and pathogenesis of Aicardi-Goutières syndrome (AGS), particularly its contextualization within a putative type I interferonopathy framework, already exist. However, recent reports of attempts at treatment suggest that an assessment of the field from a therapeutic perspective is warranted at this time. Here, we briefly summarize the neurological phenotypes associated with mutations in the seven genes so far associated with AGS, rehearse current knowledge of the pathology as it relates to possible treatment approaches, critically appraise the potential utility of therapies, and discuss the challenges in assessing clinical efficacy. WHAT THIS PAPER ADDS: Progress in understanding AGS disease pathogenesis has led to the first attempts at targeted treatment. Further rational therapies are expected to become available in the short- to medium-term.

关键词: 暂无
翻译标题与摘要 下载文献
影响因子:1.52
发表时间:2020-04-02
DOI:10.1080/09273948.2019.1603312
作者列表:["Takayama K","Obata H","Takeuchi M"]

METHODS::Purpose: To report the efficacy of adalimumab in a case of chronic Vogt-Koyanagi-Harada (VKH) disease refractory to conventional corticosteroids and immunosuppressive therapy and complicated by central serous chorioretinopathy (CSC).Case report: A 66-year-old woman diagnosed with VKH was treated with intravenous corticosteroids followed by oral corticosteroids and cyclosporine. However, systemic corticosteroids could not be tapered because of recurrent ocular inflammation and systemic complications (diabetes mellitus, moon face, bone weakness), while CSC appeared in both eyes. A diagnosis of chronic VKH resistant to medications complicated by corticosteroid-induced CSC was made. Systemic corticosteroids and cyclosporine were tapered and adalimumab initiated. Bilateral ocular inflammation and CSC were gradually reduced and visual acuity improved without any adverse effect. Twelve months after starting adalimumab monotherapy, no signs of active VKH and CSC were present.Conclusions: Adalimumab is one of the effective therapeutic options for refractory VKH disease complicated with corticosteroid-induced adverse effects.

神经系统自身免疫性疾病方向

神经系统自身免疫性疾病是以自身免疫细胞、免疫分子等攻击神经系统为主要致病机制的自身免疫性疾病。在免疫反应中,作用于神经系统自身抗原的致病抗体统称为神经系统自身抗体。

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