脂质沉积性肌病合并周围神经病的临床和神经电？…

研究脂质沉积性肌病合并周围神经损害和不伴周围神经损害的临床及神经电生理特点。方法 对１９例病理证实的脂质沉积性肌病中７例合并周围神经损害和１２例不伴周围神经损害的患者进行了常规肌电图，正中神经和胫后神经感觉传导速度及运动末端潜伏期测定。结果 发现临床上两组不同点是前者产后者长；肌电图提示运动单位动作电位时限第一组较第二宽，去除多相波后更明显，部分合并神经源性损害；     

伴神经源性损害的非炎性肌病临床与电生理特点

目的探究伴神经源性损害的非炎性肌病患者临床及电生理特点。方法回顾性收集自2015至2017年我院明确诊断为肌肉疾病且在我院肌电图室完成常规肌电图检查的所有患者,分析伴神经源性损害且诊断为非炎性肌病患者的临床及电生理特点。结果共收集经基因检测或肌肉活检明确诊断为肌肉疾病患者110例,肌电图出现神经源性损害者为10例,其中出现神经源性损害且为非炎性肌病者4例。上述4例患者分别为1例脂质沉积性肌病、1例中央轴空病、1例包涵体肌病及1例Welander型远端型肌病;肌电图均合并神经源性损害,同时伴或不伴周围神经损害。结论少数非炎性肌病患者肌电图可出现神经源性损害,肌电图不能作为诊断肌肉疾病的单独标准。     

炎性肌病 脂质沉积性肌病肌电图结果对比分析

目的分析炎性肌病与脂质沉积性肌病(LSM)的肌电图(EMG)特点。方法选取2003-08—2012-08我院确诊的25例炎性肌病患者和25例脂质沉积性肌病患者为研究对象,回顾性分析其临床与肌电图资料。结果炎性肌病患者出现自发电位的几率为51.02%,脂质沉积性肌病患者出现自发电位的几率为22%。炎性肌病和脂质沉积性肌病均可合并神经源性损害,炎性肌病运动单位动作电位(MUAP)神经源性损害出现几率4%;脂质沉积性肌病合并神经源性损害时,MUAP神经源性损害占24%。结论炎性肌病和脂质沉积性肌病肌电图表现有差异,有助于两者的诊断与鉴别诊断。     

脂质沉积性肌病临床与神经电生理分析

目的 分析脂质沉积性肌病(LSM)的临床及神经电生理特点,旨为早期诊断提供帮助.方法 回顾性分析18例经肌肉活检病理确诊的LSM患者的临床和神经电生理资料,神经电生理资料包括双侧股四头肌、三角肌的同心圆针电极检查资料以及-侧上肢正中神经及尺神经感觉、运动神经传导速度和-侧正中神经F波检测.结果 肌电图表现正常占38.9％;神经源性损害占38.9％;肌源性损害占16.7％;既有神经源性又有为肌源性损害占5.6％;检测72条神经纤维表现为波幅下降占37.5％,表现为传导速度和或潜伏期延长占12.5％,F波异常27.8％.结论 LSM肌电图表现并非如肌炎及其他类型肌病以肌源性损害多见,而多表现为正常或神经源性损害.     

慢性酒精中毒患者周围神经损害的临床与神经电生理研究

目的：探讨总结慢性酒精中毒患者周围神经损害的临床与神经电生理改变特点。方法对55例慢性酒精中毒患者的神经电生理（肌电图、运动传导速度、感觉传导速度）检测结果进行分析,并同期选择55例健康受试者作为对照,对比观察2组检测指标的差异。结果55例酒精中毒患者主要表现为对称性肢带肌萎缩、肌力减退和肢体麻木等,与健康受试者相比,慢性酒精中毒患者肌电图在静息状态、轻收缩相、重收缩相各相异常发生率较高,2组肌电图异常发生率比较差异均有统计学意义（P＜0.05）;神经传导速度比较,慢性酒精中毒患者组无论是MCV还是SCV传导速度减慢率均高于对照组,差异有统计学意义（ P＜0.05）。结论慢性酒精重度患者常出现周围神经损害,以神经末端损害为主,表现为对称性肢带肌萎缩、肌力减退和感觉障碍等,神经电生理改变以肌电图异常、感觉神经、运动神经的传导速度降低最为典型,可作为早期诊断的重要手段。     

脂质沉积性肌病的临床、神经电生理和病理学特征

目的研究脂质沉积性肌病(LSM)的临床、神经电生理及病理学特点。方法回顾性分析5例LSM患者的临床、神经电生理及病理学资料。结果本组发病年龄平均为25.6岁;均为亚急性或慢性起病,病情缓慢进展或出现缓解复发;2例有家族史;主要表现为运动耐受差和不同程度的肌无力(5/5)、腱反射减弱或消失(5/5)、肌痛或肌压痛(4/5)、肌萎缩(3/5)、末梢型感觉障碍(2/5);肌酶轻至中度升高(4/5),肌电图表现周围神经源性损害合并肌源性损害(2/5),或者单纯肌源性损害(1/5);肌肉病理学显示纤维间、肌膜下、胞核周围大量排列成串的或成团的脂滴,无炎性细胞浸润。高肉碱、低脂肪饮食、以及糖皮质激素治疗有效。结论LSM临床以肌无力和运动不耐受为主要症状,神经电生理表现为肌源性损害和/或周围神经损害,肌肉活检为诊断脂质沉积性肌病所必需。     

呈急性轴索性运动感觉神经病样表现的线粒体肌病二例:临床病理和基因突变分析

研究背景线粒体肌病主要表现为慢性四肢近端肌无力伴肌肉酸痛,可合并亚临床周围神经损害,而急性轴索性运动感觉神经病样表现伴乳酸酸中毒极为罕见。本研究对2例急性轴索性运动感觉神经病样表现伴乳酸中毒患者的临床特点进行分析,探索其病理和基因突变特点。方法通过周围神经和肌肉肌电图检查,分析神经损害特点;改良Gomori三色、琥珀酸脱氢酶等肌肉组织酶学染色明确肌肉病变性质;电子显微镜观察肌肉组织超微结构改变;24对重叠引物测序法行线粒体基因全序列测定。结果肌电图提示神经源性和肌源性损害并存,运动神经波幅显著降低、传导速度正常,感觉神经波幅轻至中度降低、传导速度正常。病理检查呈慢性肌肉病改变,改良Gomori三色染色可见较多破碎红纤维;电子显微镜观察线粒体数目和形态显著异常,并可见典型的"结晶"样包涵体。线粒体基因全序列测定明确为3243AG(例1)和8344AG(例2)位点突变,均为已知致病突变。结论线粒体肌病可以表现为急性轴索性运动感觉神经病样表现和代谢危象,为急症型,值得重视。     

慢性酒精中毒性肌病的临床与神经电生理学研究

目的　探讨慢性酒精中毒性肌病的临床和电生理改变特点。方法　对 2 6例慢性酒精中毒性肌病、13例慢性酒精中毒性周围神经病、2 1例慢性酒精中毒性神经和肌肉混合损害患者 ,以及 2 0例正常受试者进行详细询问病史、查体 ,并记录酒精摄入量和测定相关神经电生理指标 ,包括肌电图、单纤维肌电图、肌纤维传导速度、周围神经传导速度和诱发电位。结果　 (1)酒精中毒性肌病的主要临床表现为肢带肌的无力、肌肉疼痛和萎缩 ,肌病的症状和体征往往先于周围神经的损害。 (2 )与正常对照受试者比较 ,各组患者神经肌肉颤抖值均增大 (t检验 ,P<0 .0 5 ) ,纤维密度增加 (t检验 ,P<0 .0 5 ) ,周围神经病组患者的神经肌肉颤抖值和纤维密度改变尤为显著 (t检验 ,P<0 .0 1)。 (3)肌病组患者肌纤维传导速度明显减慢 (t检验 ,P<0 .0 5 ) ,其余两组患者无显著变化 (t检验 ,P>0 .0 5 )。结论　 (1)酒精中毒性肌病临床主要表现为对称性肢带肌萎缩、肌力减退和肌肉疼痛。 (2 )肌电图提示肌源性改变 ,特别是肌纤维传导速度减慢 ,是诊断酒精中毒性肌肉病变的客观指征。     

平山病的神经电生理学研究

目的 探讨平山病的神经电生理学特点及其与肌萎缩侧索硬化、多灶性运动神经病的鉴别诊断.方法 分别对平山病(26例)、肌萎缩侧索硬化(30例)和多灶性运动神经病(16例)患者进行运动和感觉传导速度、肌电图及交感皮肤反应等神经电生理学检查.运动传导速度采用由远端至近端分段刺激,记录复合肌肉动作电位的波幅、时限、面积及波形的变化,并判断是否存在神经传导阻滞;肌电图检查记录脑区肌肉(双侧胸锁乳突肌),颈区肌肉(拇短展肌、小指展肌、第一骨问肌、肱二头肌),胸区肌肉(T10椎旁肌、腹直肌)和腰骶区肌肉(胫骨前肌)的肌电活动.比较3组患者神经电生理学特点的差异性.结果 平山病组患者均无神经传导阻滞,肌电图检查显示颈区肌肉呈神经源性损害;肌萎缩侧索硬化组患者亦无神经传导阻滞,肌电图检查显示脑区、颈区、胸区和腰骶区肌肉均呈神经源性损害;多灶性运动神经病组患者均存在神经传导阻滞,肌电图检查颈区和腰骶区肌肉呈神经源性损害.平山病组患者神经传导阻滞的发生率与多灶性运动神经病组比较,差异有统计学意义(x2=42.000,P=0.000);平山病组患者神经源性损害的发生率与肌萎缩侧索硬化组比较,差异亦有统计学意义(x2=56.000,P=0.000).结论 平山病组患者运动和感觉传导速度均无异常,无神经传导阻滞,但肌电图检查显示颈区肌肉呈神经源性损害.     

化疗药物对末梢神经的影响(附17例临床报告)

目的：观察了17例患者化疗前及化疗后肌电图的变化。结果：其中有9／17患者正中神经及胫神经运动神经远瑞潜伏期（ML）延长26～62％,4／17胫神经运动神经传导速度（MW）下降13～20％,12／17正中神经感觉神经传导速度（SCV）下降19～35％,11／17腓肠神经SCV下降18～48％,波幅均正常。3例化疗前、1例化疗停后一年肌电图ML、MCV、SCV均恢复正常。提示化疗药物可引起周围神经的亚临床改变,而肌电脉现为典型的神经源性损害。停药后复查肌电图可恢复正常时限,认为化疗药物对周围神经的毒性为可逆性。而Ara-c累积剂量大于48g／m2,神经源性损害不可逆。结论：在血液病患者的治疗过程中应适当应用神经营养药物,以减轻神经源性损害。     

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.     

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.     

Clobazam for Treatment of Intractable Epilepsy: A Critical Assessment

Summary: Clobazam (CLB), a 1,5-benzodiazepine, is a remarkably effective add-on drug for individual patients with refractory partial epilepsy. CLB has an excellent safety record. As with all benzodiazepines used for treating epilepsy, sedation and withdrawal effects, together with the development of tolerance, limit its usefulness. Recent efforts to prevent or reverse tolerance with intermittent administration of CLB or periodic injection of a benzodiazepine antagonist, flumazenil, are encouraging and justify further investigations.     

La passion de Torquemada et le terrorisme islamique : quelques points de comparaison

This original research compares the doctrinal, psychopathological and operational standpoints of the 15th century Spanish Inquisition (Torquemada) with those of radical Islamism from 1988 to 2005 (Al-Qaeda). The following are reviewed: (a) the main texts codifying the procedure for conducting the criminal investigation of a Holy Office trial (Directorium inquisitorum); (b) the life and work of the grand inquisitor Tomás de Torquemada (1420–1498); (c) the psychopathological relations between passion (passionate psychoses, passionate idealism, paranoid personality) and fanaticism; (d) “the madmen, the enlightened and the criminals” of Islamic terrorism; (e) the cognitive and emotional motives for engagement in the jihadist radicalization of young people; (f) the common principles of monotheistic fanaticism (Inquisition, Al-Qaeda) and the particular dogmas of Islamic terrorism in our time; (g) the operating modes of the Inquisition and the Jihadist holy war. The author concludes that the rigour and seriousness of the inquisitorial judicial procedure, which was precise, individual and personalized, contrasts with the revolutionary pamphlets of Al-Qaeda, which only provide broad guidelines for the modus operandi of the fight against infidels, who are usually random victims.     

Claustration et collectionnisme : réflexions à propos d’un cas de syndrome de Diogène avec vols kleptomaniaques et vengeurs

Social withdrawal is a pathognomonic behaviour that is consistently associated with mental illnesses. Compulsive hoarding can also be interpreted as a pathological behaviour, even when it does not involve kleptomania. Diogenes syndrome (DS) was first described in 1975, and is characterized by both behaviours - social withdrawal and compulsive hoarding. Even though it is often the manifestation of a psychiatric condition, its aetiology is diverse. The most frequent ones are however: dementia, schizophrenia and mental retardation. In this study, we describe an atypical case presenting with DS. Il consists of a young man, seen in a forensic setting, who had been diagnosed with kleptomania in the past, presents with compulsive hoarding, and whose recent thefts were fuelled by revenge. Finally, to our knowledge, the way social withdrawal is viewed is seldom taken into account. We analyse its implication on social withdrawal.     

Neurological soft signs and schizophrenia: a review of current knowledge

Schizophrenia is a frequent and disabling disorder emerging during adolescence or early adulthood. The identification of underlying processes has been hampered by the complex clinical expression and the probable etiological heterogeneity. The frequency of neurological soft signs (NSS) in patients with schizophrenia and their presence early in life (during the first two years) in high risk subjects support the hypothesis that schizophrenia is a "brain disease" reflecting pre- or perinatal insults during development. The growing interest for NSS has lead to multiple studies that are often difficult to compare. The objective of this review is to summarize the current knowledge on NSS, methodological issues and the future perspectives.     

Lacan, lecteur de Minkowski : l'approche structurale

The notion of structure occupies a predominant place in the theory of Lacan. He indicates that was developed from the work of Minkowski. In fact, through his phenomeno-structural approach, Minkowski does not limit himself to purely observable phenomena, but attempts to determine the underlying structure. He refers to the comprehensive phenomenology and psychopathology, and this method provides him with clinical finesse and another means of determining a diagnosis. Thus Lacan has used this as a basis for his approach to structure to develop a theory regarding the individual. This implies that the structure of the individual is based on his relation to language. From this concept, he then develops the clinical structures of neurosis, psychosis, and perversion. These structural landmarks also have an effect on the course of treatment.