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[Inflammatory myopathies]. [炎症性肌病]。
Pub Date : 2019-11-13 DOI: 10.32388/340890
C. Scoppetta, C. Casali, S. D'Agostini, F. Pauri, G. Amabile
COMMON TREATMENTS SIGNS/ SYMPTOMS The most common sign of inflammatory myopathies is weakness in the large muscles of the shoulders, neck or hips. Inflammation damages tissue so you lose strength in these muscles. Inflammatory myopathies may cause problems like these: • Trouble climbing stairs, lifting objects over your head or getting out of a seat • Choking while eating or intake of food into the lungs • Shortness of breath • Cough Dermatomyositis causes skin rashes that look like red or purple spots on the eyelids, or scaly, red bumps on the elbows, knuckles or knees. Children may also have white spots on their skin called calcinosis or vasculitis, a blood vessel inflammation that causes skin lesions.
常见治疗体征/症状炎性肌病最常见的体征是肩部、颈部或臀部的大肌肉无力。炎症会损伤组织,使这些肌肉失去力量。炎症性肌病可能会导致以下问题:•爬楼梯、将物体举过头顶或从座位上下来时遇到困难•进食时窒息或将食物吸入肺部•呼吸急促•咳嗽性皮肌炎会导致眼睑上出现红色或紫色斑点的皮疹,或肘部、指关节或膝盖上出现鳞状、红色肿块。儿童的皮肤上也可能有白色斑点,称为钙化或血管炎,这是一种导致皮肤损伤的血管炎症。
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引用次数: 0
Refsum's disease. 遗传性共济失调性神经病。
Pub Date : 2008-02-25 DOI: 10.1002/9780470690369.CH19
J. Brown
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引用次数: 0
Striated sphincter and genital dysfunctions in multiple sclerosis patients: an electrophysiological approach 多发性硬化症患者的横纹括约肌和生殖功能障碍:电生理方法
Pub Date : 1993-08-01 DOI: 10.1016/0013-4694(93)91245-V
F. Sartucci, A. Pellegrinetti, L. Voci, L. Sagliocco, L. Bonfiglio, L. Murri
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引用次数: 1
[Dexamethasone therapy in Huntington chorea: preliminary results]. [地塞米松治疗亨廷顿舞蹈病:初步结果]。
Pub Date : 1991-11-01
A Nuti, C Maremmani, R Ceravolo, N Pavese, U Bonuccelli, A Muratorio

Neuroleptic drugs represent the current therapy for Huntington's chorea (HC). However neuroleptics can improve involuntary movements, but not functional performance and disease progression. Several clinical and experimental data suggest the existence of functional relationship between corticosteroids and extrapyramidal system. We administered dexamethasone to six choreics, all female. Dexamethasone was given i.m. at dose of 4 mg/die for 20 days and 8 mg/die for 20 days more. Dexamethasone at both the doses used, determined significant improvement (p less than 0.05) of dyskinesia, evaluated by AIMS, and manual dexterity, evaluated by Tapping test. Although at present it is not clear which mechanism are responsible for this of dexamethasone favourable effect, it might open new perspectives in HC therapy.

抗精神病药物是目前治疗亨廷顿舞蹈病(HC)的常用药物。然而,抗精神病药可以改善不自主运动,但不能改善功能表现和疾病进展。一些临床和实验数据表明,皮质类固醇与锥体外系系统存在功能关系。我们给六名舞蹈家注射了地塞米松,都是女性。地塞米松按4mg /d静脉滴注20 d, 8mg /d静脉滴注20 d。使用两种剂量的地塞米松均可显著改善运动障碍(用AIMS评估)和手灵巧性(用Tapping测试评估)(p < 0.05)。虽然目前尚不清楚地塞米松有利作用的机制,但它可能为HC治疗开辟新的视角。
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引用次数: 0
[Changes in the nigrostriatal dopamine receptor compartment after continuous dopaminergic infusions in Parkinson disease]. [帕金森病患者持续多巴胺能输注后黑质纹状体多巴胺受体室的变化]。
Pub Date : 1991-11-01
F Baronti, S Ruggieri, M M Mouradian, A Bonamartini, P Bocciarelli, M F De Pandis, T N Chase, A Agnoli

Motor fluctuations often complicate chronic levodopa treatment of Parkinson's disease. Pharmacologically, these phenomena are characterized by a progressive shortening of the duration of action of levodopa and a gradual narrowing of the range of "optimally effective" doses, able to improve parkinsonian akinesia without inducing abnormal involuntary movements. The effects of a continuous intravenous infusion of levodopa lasting 9 +/- 0.3 days on these clinical-pharmacological indices have been studied in 12 parkinsonian patients. Continuous infusion therapy gradually ameliorated motor fluctuations by more than 40%, and this improvement lasted for at least 6 days after resuming standard oral therapy. Moreover, levodopa duration of action was prolonged by about 30%, and the range of "optimally effective" dose was widened by about 50%. The above data suggest the possibility of plastic modifications of the pathogenetic mechanisms underlying motor fluctuations in Parkinson's disease, and a potential deleterious effect of intermittent oral therapy. Consequently, continuous dopaminergic stimulation, when used in the early stages of the disease, might theoretically have a prophylactic role on the development or worsening of motor fluctuations.

运动波动常使慢性左旋多巴治疗帕金森病复杂化。药理学上,这些现象的特点是左旋多巴作用持续时间逐渐缩短,“最佳有效”剂量范围逐渐缩小,能够改善帕金森运动障碍,而不会引起异常的不自主运动。在12例帕金森病患者中研究了持续静脉输注左旋多巴9 +/- 0.3天对这些临床药理学指标的影响。持续输注治疗逐渐改善运动波动超过40%,并且这种改善在恢复标准口服治疗后至少持续6天。此外,左旋多巴的作用时间延长了约30%,“最佳有效”剂量范围扩大了约50%。上述数据提示帕金森病运动波动的发病机制可能存在可塑性改变,以及间歇性口服治疗的潜在有害影响。因此,在疾病的早期阶段使用持续的多巴胺能刺激,理论上可能对运动波动的发展或恶化具有预防作用。
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引用次数: 0
[Cerebrovascular manifestations while taking combined estrogens and progestins for contraceptive purposes. Clinical cases]. 服用雌激素和黄体酮联合避孕时脑血管的表现。临床病例)。
Pub Date : 1991-11-01
L Sinibaldi, C Gerace, A Meccia

This study concerns 20 patients with cerebrovascular disease in the course of oral contraception with estrogens/progestins. The assumption of oral contraceptives appears to be related to the cerebrovascular manifestations, which could be caused by alterations of the blood vessel walls or of the coagulative process induced by estrogens/progestins. The thrombogenic action of these substances could be enhanced by preexisting conditions such as protein C or protein S deficiency.

本研究涉及20例服用雌激素/黄体酮口服避孕药过程中的脑血管疾病患者。口服避孕药的假设似乎与脑血管症状有关,脑血管症状可能是由雌激素/黄体酮引起的血管壁改变或凝固过程引起的。这些物质的血栓形成作用可以通过先前存在的条件如蛋白C或蛋白S缺乏而增强。
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引用次数: 0
[Status epilepticus in chronically dialyzed patients treated with erythropoietin]. [促红细胞生成素治疗慢性透析患者的癫痫持续状态]。
Pub Date : 1991-11-01
R Massetani, R Galli, R Calabrese, F Sartucci, P Rindi, L Severino Brandi, L Murri

This paper describes the clinical features of two patients with chronic renal failure and uremic anaemia treated with recombinant human erythropoietin (9000 I.U. subcutaneously subdivided in 3 times weekly at the end of haemodialysis treatment) who developed seizures and status epilepticus. This treatment has unequivocal benefits but in some patients has been accompanied by elevated blood pressure leading to hypertensive encephalopathy with seizures. In fact, the correction of the anaemia results in a rise in packed cell volume with a consequent increase in blood viscosity, predisposing to increased vascular resistance and the development of hypertension.

本文报道2例慢性肾功能衰竭合并尿毒症贫血患者在血液透析结束时接受重组人促红细胞生成素(9000 iu,每周3次皮下注射)治疗后出现癫痫持续状态的临床特点。这种治疗有明确的益处,但在一些患者中伴随血压升高导致高血压性脑病发作。事实上,贫血的纠正会导致堆积细胞体积的增加,从而导致血液粘度的增加,易导致血管阻力的增加和高血压的发展。
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引用次数: 0
[Diurnal worsening in Parkinson patients treated with levodopa]. [左旋多巴治疗帕金森患者的日恶化情况]。
Pub Date : 1991-11-01
P Piccini, P Del Dotto, C Pardini, P D'Antonio, G Rossi, U Bonuccelli

Parkinson's disease (PD) patients show a good response to levodopa in the morning, and reduced duration or complete failure of response later in the day, but the pathophysiology of this phenomenon remains unclear. We evaluated motor performance hourly over a twelve-hour period in patients treated with levodopa/carbidopa (group A), with bromocriptine (group B), and in "de novo" patients (group C). At 8 am, 12 and 4 pm, group A patients received standard doses of levodopa/carbidopa, whereas patients of group B and C took, respectively, 5 mg bromocriptine and placebo. In "de novo" patients and in patients under bromocriptine we did not observe significant diurnal changes in motor score, whereas in patients under levodopa a progressive daytime worsening, which significantly correlated with progressive increase in 3-O-methyldopa plasma levels, was visible. These data seem to indicate a contributory role of pharmacokinetic or pharmacodynamic factors related to levodopa assumption, rather than to the underlying disease, in the afternoon worsening in PD.

帕金森病(PD)患者在早晨对左旋多巴有良好的反应,在当天晚些时候反应持续时间缩短或完全失效,但这一现象的病理生理学尚不清楚。我们评估了左旋多巴/卡比多巴(a组)、溴隐亭(B组)和“新生”患者(C组)在12小时内每小时的运动表现。在上午8点、12点和下午4点,a组患者接受标准剂量的左旋多巴/卡比多巴,而B组和C组患者分别服用5毫克溴隐亭和安慰剂。在“新生”患者和服用溴隐亭的患者中,我们没有观察到明显的运动评分昼夜变化,而在左旋多巴的患者中,可以看到白天进行性恶化,这与3- o -甲基多巴血浆水平的进行性增加显著相关。这些数据似乎表明,与左旋多巴假设相关的药代动力学或药效学因素在PD患者下午恶化中的作用,而不是与潜在疾病有关。
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引用次数: 0
[Antiphospholipid antibodies and cerebral ischemia]. [抗磷脂抗体与脑缺血]。
Pub Date : 1991-11-01
S Severi, M Ghezzi, P Zolo, A Bianchi

Lupus anticoagulants (LAs) and anticardiolipin antibodies (ACAs) are acquired circulating immunoglobulins that cross-react with anionic and neural phospholipids. These factors may display anticoagulant properties in vitro by interfering with phospholipid-dependent coagulation tests. These antibodies are usually not associated with a bleeding tendency. In fact, paradoxically to their name, since the initial recognition they have been related to systemic and cerebral thromboembolisms, despite their in vitro "anticoagulant" properties. We report the clinical and laboratory findings in 4 LAs and ACAs positive patients with brain ischemia.

狼疮抗凝血剂(LAs)和抗心磷脂抗体(ACAs)是获得性循环免疫球蛋白,与阴离子和神经磷脂交叉反应。这些因子可能通过干扰磷脂依赖性凝血试验在体外显示抗凝特性。这些抗体通常与出血倾向无关。事实上,与它们的名字相矛盾的是,尽管它们具有体外“抗凝”特性,但自最初被发现以来,它们一直与系统性和脑血栓栓塞有关。我们报告4例LAs和ACAs阳性脑缺血患者的临床和实验室结果。
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引用次数: 0
[Maps of somatosensory evoked potentials from the arm in patients with neoplasms and post-traumatic brain lesions]. [肿瘤和创伤后脑损伤患者手臂的体感诱发电位图]。
Pub Date : 1991-11-01
C Paradiso, V Caruso, S Rossi, R Cioni, S Passero, F Giannini, R Ravenni, N Battistini

Short-latency somatosensory evoked potentials by the stimulation of the median nerve at the wrist, were recorded in six patients (four with cerebral tumors and two with post-trauma lesions). The electrodes were placed on the scalp following the 10-20 International System. A reference electrode was placed on earlobe contralateral to the site of the stimulation. Eleven normal subjects were used as control (mean age 64.4 +/- 12.05). We used the Brain-Surveyor-Basis Trade system which allowed us to elaborate the results by coloured mapping through linear interpolation of signal amplitudes. The following parameters were investigated: peak latencies of the N13, N20, P22, N30 waves; amplitudes of the post-rolandic P14-N20, N20-P25, pre-rolandic P22-N30 components and the central conduction time N13-N20 (CCT). The evaluation of latencies was not significant in determining the lesion site. On the contrary, the evaluation of amplitudes revealed expressive asymmetry, though it did not define the nature of alteration (increase or decrease due to lesion), and the correlation between these variations and the site of the lesion. The authors discussed the possibility that amplitude abnormalities in patients with tumors were related either to the tumors and/or drug effects.

6例患者(4例脑肿瘤患者和2例创伤后病变患者)通过刺激腕部正中神经记录了短潜伏期体感诱发电位。电极按照10-20国际系统放置在头皮上。参考电极放置在刺激部位对侧的耳垂上。11名正常受试者作为对照(平均年龄64.4±12.05)。我们使用脑-测量师-基础贸易系统,这使我们能够通过信号幅度的线性插值,通过彩色映射来详细说明结果。研究了以下参数:N13、N20、P22、N30波的峰值潜伏期;P14-N20、N20-P25、P22-N30各组件的幅值和中心导通时间N13-N20 (CCT)。潜伏期的评估在确定病变部位方面没有意义。相反,振幅的评估显示了表达的不对称性,尽管它没有定义改变的性质(由于病变增加或减少),以及这些变化与病变部位之间的相关性。作者讨论了肿瘤患者振幅异常与肿瘤和/或药物作用有关的可能性。
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Rivista di neurologia
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