多发性肌炎合并间质性肺病一例

多发性肌炎（PM）是一种肌肉炎性免疫性疾病，除累及肌肉外，还可引起肺脏、心脏及胃肠道等多系统损害。间质性肺病（ILD）是PM患者最常见、最严重的并发症，大部分病例抗Jo-1抗体阳性。     

影响多发性肌炎、皮肌炎患者预后的相关因素研究

目的 观察影响多发性肌炎(polymyositis，PM)和皮肌炎(dematomyosids，DM)患者预后的相关因素，并探讨抗Jo-1抗体在判断预后中的价值。方法 对52例PM(27例)和DM(25例)患者进行1～27年随访，并对所有患者进行抗Jo-1抗体测定，临床肌力评定应用4级功能障碍评级法，统计学处理应用科克斯比例危险模式、四格表确切概率法及χ^2分析影响PM和DM患者预后相关因素。结果 52例患者病死率21．2％，1年生存率86．2％，3年生存率81．4％，5年生存率78．4％，9年生存率74．6％．未发现与生存时间密切相关的因素。伴有恶性肿瘤(P=0．003)、间质性肺炎(P=0．006)，吞咽困难(P=0．004)患者病死率高于不伴上述疾病患者，痊愈和基本治愈患者占生存患者的60．7％。发病时肌肉功能情况与预后无关。52例PM和DM患者抗Jo-1抗体阳性9例(17．3％)，其中PM阳性者6例(22．2％)，DM阳性者3例(12．0％)，抗Jo-1抗体阳性患者66．7％伴间质性肺炎，33．3％伴吞咽困难。结论 PM和DM患者长期预后较好，存活者中大部分患者可基本痊愈．伴有恶性肿瘤、间质性肺炎、吞咽困难患者病死率高。抗Jo-1抗体阳性患者易伴间质性肺炎和吞咽困难。     

血清抗Jo-1抗体和抗核抗体与多发性肌炎患者心、肺损害的关系

目的 探讨血清抗Jo-1抗体和抗核抗体(ANA)与多发性肌炎(PM)患者心、肺损害的关系.方法 将52例PM患者根据是否伴心肺损害分为PM伴肺部损害组(14例)、伴心脏损害组(21例)和无心肺损害组(17例),分别检测血清抗Jo-1抗体和ANA,并进行比较.结果 PM伴肺部损害组血清抗Jo-1抗体阳性率(42.9%)明显高于无心肺损害组(5.9%)(P＜0.01);血清ANA阳性率PM伴心脏损害组(76.2%)及PM伴肺部损害组(71.4%)明显高于无心肺损害组(35.2%)(P＜0.01,P＜0.05).结论 血清抗Jo-1抗体可能与PM伴肺部损害及血清ANA可能与PM伴心、肺损害有关.     

特发性炎性肌病进展

正特发性炎性肌病(IIM)是一组具有不同临床表现和病理学特点的骨骼肌免疫性疾病,通常包括多发性肌炎(PM)、皮肌炎(DM)、免疫性坏死性肌病(NAM)、包涵体肌炎(IBM)、结缔组织病相关性免疫性坏死性肌病和罕见类型的炎性肌病。炎性肌病不同类型构成比随疾病研究的深入显著改变,其中95%曾诊断的多发性肌炎重新经病理学和免疫学评估而改变诊断。多发性肌炎除名称外可能已不复存在[1-2],更多可能是伴特殊抗体的炎性肌病、重叠     

黏病毒抗性蛋白A在皮肌炎病理诊断中的应用

目的旨在了解黏病毒抗性蛋白A(MxA)在不同炎性肌病肌纤维中的表达情况,探讨MxA在皮肌炎病理诊断中的应用价值。方法本研究采用回顾性研究方法。收集2013至2017年所做的炎性肌病肌活检标本和临床资料,其中皮肌炎18例为皮肌炎组,其他炎性肌病22例(免疫介导坏死性肌病7例,包涵体肌炎6例,抗合成酶抗体综合征5例,重叠性肌炎4例)为对照组。采用免疫组化学法评估MxA在不同炎性肌病肌纤维中的表达情况,并与皮肌炎病理特征(1)束周萎缩;(2)主要组织相容性复合体-Ⅰ(MHC-Ⅰ)束周表达上调;(3)毛细血管膜攻击复合物(C5b-9)沉积进行比较。结果皮肌炎组15例患者的肌纤维MxA表达上调,以束周肌纤维上调最为明显;对照组未见表达上调。MxA对皮肌炎诊断的敏感度和特异度分别为83%和100%,束周萎缩的敏感度和特异度分别为72%和95%。MHC-Ⅰ束周表达上调的敏感度和特异度分别为50%和86%。毛细血管C5b-9沉积的敏感度和特异度分别为39%和81%。将束周萎缩或MxA阳性(合集)作为一个指标,其敏感度和特异度分别高达94%和100%。结论 MxA对皮肌炎诊断的敏感度和特异度较经典的皮肌炎病理标志束周萎缩更高,也优于MHC-Ⅰ的束周表达上调及毛细血管C5b-9沉积。MxA染色对炎性肌病的诊断和鉴别诊断具有较高的参考价值。     

多发性肌炎、皮肌炎酶组织化学、组织化学诊断探讨

多发性肌炎、皮肌炎 (PM/DM)是一组与自身免疫有关的炎症性肌病。我们采用先进的组织化学和酶组织化学技术对 82例 PM、 DM患者的肌肉进行回顾性研究 ,以探讨组织化学和酶组织化学技术在病理诊断中的意义。材　料　和　方　法1.多发性肌炎 (PM) :女 34例 ,男 10例 ;最小17岁 ,最大 6 3岁 ,平均 30 .5岁 ,以中年为最多见。平均就诊时间 1年 4个月 ,最长病程 7年 ,最短 7天。近端肌力 、 级为最多见 ,偶见肌力为 级。其中吞咽困难占 31% ,肌痛占 5 0 % ,血沉快占 18% ,肌无力 10 0 % ,发热 35 % ,关节痛 13% ,雷诺氏征 4 .3% ,心肌酶升…     

自身免疫性多发性肌炎

多发性肌炎(PM)是指肌肉广泛炎性病变,临床主要表现为肌无力等症状的一类肌肉疾病,病因未明,可能与感染、中毒、恶性肿瘤、胶原病和药物等有关。本文综述一组与自身免疫有关的PM,称为自身免疫性多发性肌炎(AIPM)。     

多发性肌炎/皮肌炎临床和病理研究(附305例分析)

目的:研究多发性肌炎/皮肌炎(PM/DM)的临床、病理特征。方法:回顾性总结305例PM/DM患者的临床资料,根据Bohan标准分为5型,研究其临床表现、血清肌酶学、肌电图、肌肉病理的特点。结果:本病临床上主要有肌无力、肌痛或肌捏痛,CK等血清肌酶增高,肌电图呈肌原性损害。各型肌肉病理均显示免疫炎性改变,以单纯多发性肌炎组织损害程度较重,主要表现为肌纤维散在萎缩、肌肉膜炎;皮肌炎组多为肌束周萎缩、血管炎性病变;肌炎合并恶性肿瘤(CAM)、儿童型肌炎(JPM/DM)、肌炎合并其它结缔组织疾病(CTM)三组血管炎性改变亦较明显。结论:各型肌炎的临床和病理有所不同,发病的免疫病理机制亦有所区别。     

强直性肌营养不良的肌电图特点分析

目的分析强直性肌营养不良(DM)的肌电图特点。方法对2014至2017年11例诊断为DM1型患者(DM组)和随机选取11例多发性肌炎(PM)患者(对照组)的针极肌电图、神经传导进行比较分析,总结DM患者的肌电图特点。结果 DM组5例行基因检查确诊。(1)DM组复合肌肉动作电位(CMAPs)下降4条(占所有神经传导5%);(2)DM组肌强直电位出现例数(11例)较PM组(1例)多(χ2=18.333,P0.05);(3)DM组近、远端肌肉均受累(18块近端肌肉和19块远端肌肉出现肌源性损害),PM组(21块近端肌肉和2块远端肌肉出现肌源性损害)更易出现近端肌源性损害(χ2=11.344,P=0.001);(4)DM组(8块近端肌肉和20块远端肌肉)出现肌强直放电与PM组(1块近端肌肉和0块远端肌肉)比较,强直性放电的近远端肌肉受累比较,差异无显著性(P=0.310)。结论 DM 1型患者肌电图改变的主要特点为肌强直电位放电伴肌源性损害,且以远端、近端肌肉均受累显著;PM肌电图改变的主要特点为肌源性损害,以近端肌肉受累明显。肌电图特点可以鉴别DM和PM。     

皮肌炎和多发性肌炎175例神经传导检测

目的 研究皮肌炎(dermatomyositis,DM)和多发性肌炎(polymyositis,PM)合并神经传导检测(NCS)异常患者的临床和电生理特点以及病因探讨.方法 收集2005年1月到2008年9月中国医学科学院北京协和医院病房收治的DM或PM确诊病例175例,对其临床和NCS结果进行回顾性分析.结果 175例患者中,NCS异常者66例,其中明确的周围神经病32例(48.5%),不能肯定为周围神经损害者34例(51.5%).合并周围神经损害的DM或PM患者恶性肿瘤(3/32,9.3%)、其他免疫病(6/32,18.8%)的发生率较无周围神经损害的DM或PM患者(4/109,3.7%;7/109,6.4%)有明显增加(X2=13.653,P=0.003).结论 合并周围神经损害的DM或PM患者恶性肿瘤、其他免疫病的发生率明显增加,NCS可以为临床早期诊断提供更多的提示和帮助.     

Epilepsy and anomalies of neuronal migration: MRI and clinical aspects

Neuronal migration disorders are the result of disturbed brain development. In such disorders, neurons are abnormally located. In diagnosing these conditions, magnetic resonance imaging is superior to any other imaging technique. This enables us to improve our knowledge of the clinical correlates of neuronal migration. With reference to migrational disorder, a retrospective study of all 303 patients with epileptic seizures referred for magnetic resonance imaging during a 3-year period was performed, 13 patients (aged 12-41, mean age 27) were identified. They represent 4.3% of the entire study group. Of the patients with known epilepsy, 6.7% and of the mentally retarded, 13.7% had migrational disorders. Four patients had schizencephaly as the dominant finding, one was classified as hemimegalencephaly, 2 had isolated heterotopias, and 6 had localized pachy- and/or poly-microgyria. The clinical pictures are complex. Ectopias of grey matter are recognised foci of epilepsy, but from an epileptological and a clinical viewpoint little attention has been given to these disorders. The present study shows that malmigration is not rare in epilepsy patients, especially not in the mentally retarded.     

Hepatic Considerations in the Use of Antiepileptic Drugs

Summary: Virtually all of the major antiepileptic drugs (AEDs) can cause hepatotoxicity, although fatal hepatic reactions are rare. The mechanisms, incidences, and risk profiles for such reactions differ from drug to drug. With carbamazepine and phenytoin, hepatotoxicity may be due to drug hypersensitivity. Although the profiles of patients at risk have not been well-defined for these two antiepileptic drugs, it would appear from reports in the literature that older adolescents and adults are at higher risk than children of developing serious or fatal hepatotoxicity. Once hepatotoxicity develops, mortality rates are 10–38% with phenytoin and 25% for carbamazepine. The risk profile for valproate fatal hepatotoxicity has been more clearly defined. Those at primary risk of fatal hepatic dysfunction are children under the age of 2 years who are receiving multiple anticonvulsants and also have significant medical problems in addition to severe epilepsy. The risk is considerably lower for patients over the age of 2 years on valproate monotherapy. In contrast to the risk profile with other AEDs, adults receiving valproate as monotherapy have the lowest risk of hepatotoxicity. Fatal hepatic dysfunction coincident with valproate may be the result of aberrant drug metabolism. Concomitant use of AEDs that induce microsomal P450 enzymes (e.g., phenytoin and phenobarbital) may enhance the production of a toxic metabolite, and hence the greater risk of hepatotoxicity with polypharmacy.     

Vascular Malformations and Epilepsy: Clinical Considerations and Basic Mechanisms

Summary: Vascular malformations (VMs) are associated with epilepsy. The natural history of the various VMs, clinical presentation, and tendency to provoke epilepsy determine treatment strategies. Investigations have probed the mechanisms of epileptogenesis associated with these lesions. Electrophysiologic changes are associated with epileptogenic cortex adjacent to VMs. Putative pathophysiologic mechanisms of epileptogenesis include neuronal cell loss, glial proliferation and abnormal glial physiology, altered neurotransmitter levels, free radical formation, and aberrant second messenger physiology.     

Classification of methods in transcranial Electrical Stimulation (tES) and evolving strategy from historical approaches to contemporary innovations

Transcranial Electrical Stimulation (tES) encompasses all methods of non-invasive current application to the brain used in research and clinical practice. We present the first comprehensive and technical review, explaining the evolution of tES in both terminology and dosage over the past 100 years of research to present day. Current transcranial Pulsed Current Stimulation (tPCS) approaches such as Cranial Electrotherapy Stimulation (CES) descended from Electrosleep (ES) through Cranial Electro-stimulation Therapy (CET), Transcerebral Electrotherapy (TCET), and NeuroElectric Therapy (NET) while others like Transcutaneous Cranial Electrical Stimulation (TCES) descended from Electroanesthesia (EA) through Limoge, and Interferential Stimulation. Prior to a contemporary resurgence in interest, variations of transcranial Direct Current Stimulation were explored intermittently, including Polarizing current, Galvanic Vestibular Stimulation (GVS), and Transcranial Micropolarization. The development of these approaches alongside Electroconvulsive Therapy (ECT) and pharmacological developments are considered. Both the roots and unique features of contemporary approaches such as transcranial Alternating Current Stimulation (tACS) and transcranial Random Noise Stimulation (tRNS) are discussed. Trends and incremental developments in electrode montage and waveform spanning decades are presented leading to the present day. Commercial devices, seminal conferences, and regulatory decisions are noted. We conclude with six rules on how increasing medical and technological sophistication may now be leveraged for broader success and adoption of tES.     

Carbamazepine Efficacy and Utilization in Children

Summary: Carbamazepine is effective for preventing partial and generalized tonic-clonic seizures in children. Although absence epilepsies are more common in children than adults, an estimated 80% of children with epilepsy have seizure types or epilepsies that are potentially responsive to carbamazepine. The differential diagnosis of ictal staring is an especially important issue in children because absence and atypical absence seizures are more prevalent in children than adults. Age-related pharmacokinetic differences and drug interactions are major considerations in children. On average, children have higher clearance rates of carbamazepine, shorter half-lives, and higher ratios of carbamazepine-10, 11-epoxide to carbamazepine than adults. In addition, children with severe epilepsy are more likely to require multiple-drug therapy, which can lead to complex drug interactions. When carbamazepine is administered along with valproate, drug protein binding interactions can cause intermittent side effects.     

Neonatal Seizures: Problems in Diagnosis and Classification

Summary: The clinical identification of neonatal seizures is critical for the recognition of brain dysfunction; however, diagnosis is often difficult because of the poorly organized and varied nature of these behaviors. Current classification systems are limited in their ability to communicate motor, autonomic, and electroencephalo-graphic features of seizures precisely and to provide a basis for uniform effective diagnosis, therapy, and determination of prognosis. Recent investigations of neonates, utilizing bedside electroencephalographic/polygraphic/ video monitoring techniques, have provided the basis for improved diagnosis and classification of seizures in the newborn. These studies have demonstrated that not all clinical phenomena currently considered to be seizures require electrocortical epileptiform activity for their initiation or elaboration. In addition, the specific clinical character of the phenomena considered to be seizures, the clinical state of the infant, and the character of the EEG indicate the probable pathophysiological mechanisms involved and suggest probable etiologies, prognosis, and therapy. Similarities between animal models that demonstrate reflex physiology and neonates with motor automatisms and tonic posturing suggest that these clinical behaviors may not be epileptic in origin but, rather, primitive movements of progression and posture mediated by brainstem mechanisms. Although not all clinical behaviors currently considered to be neonatal seizures may have similar pathophysiological mechanisms, they are clinically significant because they all indicate brain dysfunction.     

Valproate Monotherapy in the Management of Generalized and Partial Seizures

Summary: For decades, therapeutic tradition has promoted the concept of polypharmacy in the management of epilepsy. In recent years, however, studies have shown that, for most patients, monotherapy can provide comparable or better seizure control than administration of multiple anticonvulsants, while diminishing the potential for adverse reactions, drug interactions, and poor compliance. Valproate is an important monotherapeutic agent that is highly effective in the control of idiopathic primary and secondarily generalized epilepsies, and partial seizures that do not generalize. Comparative studies have found that valproate is at least as effective as phenytoin and carbamazepine in the treatment of generalized and partial seizures. Given the similar efficacy, other factors such as pharmacokinetics and side effects may therefore determine anticonvulsant selection for monotherapy.     

Un nouveau cadre conceptuel de travail pour la psychiatrie

In an attempt to place psychiatric thinking and the training of future psychiatrists more centrally into the context of modern biology, the author outlines the beginnings of a new intellectual framework for psychiatry that derives from current biological thinking about the relationship of mind to brain. The purpose of this framework is twofold. First, it is designed to emphasize that the professional requirements for future psychiatrists will demand a greater knowledge of the structure and functioning of the brain than is currently available in most training programs. Second, it is designed to illustrate that the unique domain which psychiatry occupies within academic medicine, the analysis of the interaction between social and biological determinants of behavior, can best be studied by also having a full understanding of the biological components of behavior.