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Percutaneous Venous Angioplasty in Patients with Multiple Sclerosis and Chronic Cerebrospinal Venous Insufficiency: A Randomized Wait List Control Study.
经皮静脉血管成形术治疗多发性硬化和慢性脑脊髓静脉功能不全患者: 一项随机等待列表对照研究。
- 影响因子:1.14
- DOI:10.1016/j.avsg.2019.05.018
- 作者列表:"Napoli V","Berchiolli R","Carboncini MC","Sartucci F","Marconi M","Bocci T","Perrone O","Mannoni N","Congestrì C","Benedetti R","Morganti R","Caramella D","Cioni R","Ferrari M
- 发表时间:2020-01-01
Abstract
BACKGROUND:Venous percutaneous transluminal angioplasty (vPTA) in patients with multiple sclerosis (MS) and chronic cerebrospinal venous insufficiency (CCSVI) have shown contradictory results. The aim of the study is to evaluate the efficacy of the procedure in a randomized wait list control study. METHODS:66 adults with neurologist-confirmed diagnosis of MS and sonographic diagnosis of CCSVI were allocated into vPTA-yes group (n = 31) or vPTA-not group (n = 35, control group). vPTA was performed immediately 15 days after randomization in the PTA-yes group and 6 months later in the control group. Evoked potentials (EPs), clinical-functional measures (CFMs), and upper limb kinematic measures (ULKMs) were measured at baseline (T0) and six months after in both groups, just before the venous angioplasty in the vPTA-not group (T1). RESULTS:Comparing the vPTA-yes and vPTA-not group, the CFM-derived composite functional outcome showed 11 (37%) versus 7 (20%) improved, 1 (3%) versus 3 (8%) stable, 0 versus 7 (20%) worsened, and 19 (61%) versus 18 (51%) mixed patients (χ2 = 8.71, df = 3, P = 0.03). Unadjusted and adjusted (for baseline confounding variables) odds ratio at 95% confidence interval were, respectively, 1.93 (1.3-2.8), P value 0.0007, and 1.85 (1.2-1.7), P value 0.002. EP- and ULKM-derived composite functional outcome showed no significant difference between the two groups. CONCLUSIONS:Venous angioplasty can positively impact a few CFMs especially for the quality of life but achieving disability improvement is unlikely.
摘要
背景: 多发性硬化 (MS) 和慢性脑脊髓静脉功能不全 (CCSVI) 患者的静脉经皮腔内血管成形术 (vsta) 显示出矛盾的结果。本研究的目的是在随机等待列表对照研究中评价该程序的疗效。 方法: 将 66 例经神经科医生确诊为MS且超声诊断为CCSVI的成人患者分配到vsta-yes组 (n = 31) 或vsta-not组 (n = 35,对照组)。在随机分组后 15 天,PTA-yes组和对照组 6 个月后立即进行vsta。两组均在基线 (T0) 和 6 个月后测量诱发电位 (EPs) 、临床功能指标 (CFMs) 和上肢运动学指标 (ULKMs)。就在vsta-not组 (T1) 静脉血管成形术前。 结果: 比较vsta-yes和vsta-not组,CFM衍生的复合功能结果显示 11 (37%) 对 7 (20%) 改善,1 (3%) 与 3 例 (8%) 稳定相比,0 例与 7 例 (20%) 恶化,19 例 (61%) 与 18 例 (51%) 混合患者 (χ 2 = 8.71,Df = 3,P = 0.03)。未校正和校正 (基线混杂变量) 在 95% 置信区间下的比值比分别为 1.93 (1.3-2.8),p值为 0.0007 和 1.85 (1.2-1.7),p值 0.002。EP和ULKM来源的复合功能结局在两组间无显著差异。 结论: 静脉血管成形术可对少数CFMs产生积极影响,特别是对生活质量的影响,但不太可能实现残疾改善。
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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.
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.
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。