[2]发作性一侧肢体舞蹈样徐动(上)

患者男性,18岁,黑龙江绥化人.10年来发作四肢交替性、舞蹈样异常运动,每天发作20～30次,每次持续10余秒;每次发作均在注意力不集中,而且拟运动时(如上课时老师叫起发言),从坐位突然站立起来时,出现发作.     

非酮症高血糖性舞蹈病一例

1病例报告患者女,68岁,因四肢及面部不自主运动10d,于2010-11-8入院。患者于2010-10-29无明显诱因下出现发作性的四肢不自主舞动,不能自控,同时伴有面部肌肉不自主抽动,清醒时出现,睡眠时消失。既往患糖尿病10余年,近半年未监测血糖。体格检查:生命体征平稳,一     

中枢神经系统黏膜相关淋巴瘤一例

临床资料患者男性,49岁,因"发作性四肢抽搐,头痛、视物模糊3年"于2007年11月9日入院.患者3年前出现发作性四肢抽搐,伴意识丧失,每次持续10 min,每月发作1次,感冒后易诱发.     

复方妥英麻黄茶碱片致发作性锥体外系疾病1例

<正>复方妥英麻黄茶碱为呼吸科常用药物,因其经济便宜、效果显著,已成为哮喘、慢阻肺等患者的首选药物。但临床偶有因用法用量不当导致药物中毒现象,表现为共济失调、肌张力障碍、烦躁、甚至癫痫等。现报道1例因长期不规律服用复方妥英麻黄茶碱导致发作性锥体外系症状的病例。1病例患者,女,53岁,离退休人员。因“发作性不自主运动2年,加重5 d”于2021年8月31日入院。患者于2年前无明显诱因出现发作性四肢及躯干不自主运动,意识清楚,伴头晕、视物模糊,每次发作持续约0.5 h。查头颅CT、MRI未见异常。近1年来发作频繁,每次持续10 min左右,发作间期走路不稳。既往哮喘病史30年,一直口服“复方妥英麻黄茶碱片”,哮喘控制不理想;失眠3年;高血压病史2年,口服“吲达帕胺”片,血压控制可。查体：体温36℃,心率68次/min,呼吸17次/min,血压127/87 mmHg(1 mmHg=0.133 kPa)。     

托吡酯治疗癫癎出现自身免疫性溶血性贫血1例报告

服用托吡酯(TPM)治疗癫(疒间)出现自身免疫性溶血性贫血(AIHA),国内外尚未见报道,现报告1例如下。1 病例 男,67岁。于2000年9月1日凌晨2时左右,在睡眠中突然意识丧失,四肢抽搐,两眼上翻,口吐白沫,牙关紧闭,口唇青紫,历时3～5分钟后自行缓解。一月内发作3次,每次发作症状、持续时间相似。脑电图显示癫癎样放电。诊     

疑难病例会诊专栏[2] 发作性一侧肢体舞蹈样徐动(上)

患者男性,18岁,黑龙江绥化人。10年来发作四肢交替性、舞蹈样异常运动,每天发作20～30次,每次持续10余秒;每次发作均在注意力不集中,而已拟运动时(如上课时老师叫起发言),从坐位突然站立起来时,出现发作。或突然要跑步时(如追车等),易出现发作,跑步过程中无发作;发作常为一侧肢体(左侧),轻时手脚快速舞动样动作,重时也可出现躯干的肌紧张性异常扭动。静止和持续运动时无发作。发     

交替性瘫痪并癫癎发作一例报告

患儿女,4岁,因发作性、交替性肢体瘫痪4年,发作性四肢抽搐10+次,于2002年9月4日入院.患儿自出生后3个月起出现偏侧肢体无力,持续3～7 d后自行缓解.相隔1周后又可再次出现另一侧肢体无力,如此反复至今.当双侧肢体同时出现无力时,即出现突发四肢抽搐,双眼上翻,伴意识障碍,持续约1～2 min.病程中有数次突然惊恐发作,不伴肢体抽搐.     

发作性运动障碍1例报告

发作性运动障碍是一类罕见的神经系统疾病 ,其临床特点是反复发作的不自主运动 ,不伴意识障碍。根据诱因、发作持续时间及病因等将本病分为四型 :发作性运动诱发的异常运动(PKD) ;发作性非运动诱发的异常运动 (PNKD) ;发作性持续运动诱发的肌张力障碍 (PED ) ;夜间发作性肌张力障碍(HPD)。现将笔者遇到的 1例PKD报告如下。1　临床资料　　患者女性 ,3 5岁。 17年前无明显诱因下出现右侧上下肢不自主扭动 ,在惊吓或紧张时易发 ,每次持续 1～ 2min ,无意识丧失 ,缓解时肢体活动正常 ,睡眠时不发作 ,但惊醒后易发作 ,患者开始时每天发作…     

脊髓动静脉畸形所致脊髓性肌阵孪

脊髓性肌阵挛是指受累的脊髓节段所支配的肌肉出现突然的、无意识障碍、脑电图正常的不自主运动.其原因包括:感染、髓内胶质瘤、硬脊膜外脊髓压迫症、外伤和变性疾病等.作者报告一例由脊髓动静脉畸形(AVM)所引起的肌阵挛.病例报告:患者男性,57岁.三年前开始胸部和上腹部出现"颤抖样"不自主运动.最初,每周发作二次至三次.每次持续5～10秒钟.入院前半年,发作次数逐渐增加至每天100次.睡眠时亦可发生.精神紧张时加重.无不自主运动疾病的家族史.血压140/90mmHg.神经系统检查:神志清楚,胸部和上腹部可见节段性肌阵挛,呈无节律的类似"颤     

动态脑电图监测卡马西平引起肌阵挛1例报告

患者蒋某某，女，18岁。患者就诊前1月余不明原因突然意识丧失，倒地，四肢抽搐，面色青紫，口吐白沫，持续数分钟后自行缓解。后就诊于当地县医院，普通脑电图见痫样放电，即诊断为癫痫，服用卡马西平，0．1，tid。此后无类似发作。数日前病人频繁出现左侧上下肢或眼肌阵挛或不自主运动于我院就诊，病人述每日发作数十次，每次持续2～3s，发作时“心里明白”，间隔期最短仅为1～2min。     

The effect of age of onset of PD on risk of dementia

Background Dementia occurs in the majority of patients with Parkinson’s disease (PD). Late onset of PD has been reported to be associated with a higher risk for dementia. However, age at onset (AAO) and age at baseline assessment are often correlated. The aim of this study was to explore whether AAO of PD symptoms is a risk factor for dementia independent of the general effect of age. Methods Two community-based studies of PD in New York (n = 281) and Rogaland county, Norway (n = 227) and two population-based groups of healthy elderly from New York (n = 180) and Odense, Denmark (n = 2414) were followed prospectively for 3–4 years and assessed for dementia according to DSM-IIIR. All PD and control cases underwent neurological examination and were followed with neurological and neuropsychological assessments. We used Cox proportional hazards regression based on three different time scales to explore the effect of AAO of PD on risk of dementia, adjusting for age at baseline and other demographic and clinical variables. Findings In both PD groups and in the pooled analyses, there was a significant effect of age at baseline assessment on the time to develop dementia, but there was no effect of AAO independent of age itself. Consistent with these results, there was no increased relative effect of age on the time to develop dementia in PD cases compared with controls. Interpretation This study shows that it is the general effect of age, rather than AAO that is associated with incident dementia in subjects with PD. Received in revised form: 22 December 2005     

腺垂体功能减退症临床特征变化分析

目的探讨腺垂体功能减退症患者的病因结构变化及临床表现。方法回顾性分析我院2013-01—2016-12住院及门诊78例腺垂体功能减退症患者的临床资料。结果男32例(41.03%),女46例(58.97%);诊断时年龄11~89岁,平均62.5岁;鞍区占位(包括术前及术后)52例(66.67%),席汉综合征8例(10.26%),空泡蝶鞍9例(11.65%),病因不明8例(10.26%),垂体-下丘脑发育不良1例(1.28%)。首次就诊科室:纳差厌食、恶心呕吐就诊于消化内科36例(46.15%)最常见。ACTH+TSH+Gn+G激素缺乏为19例最多,占24.36%,ACTH+TSH+Gn缺乏15例,占19.23%。结论腺垂体功能减退症病因结构发生变化,发病人群、首发症状及受累激素也不同,患者女性多于男性,发病年龄偏高,症状不典型,分布于临床多个科室,其中以低钠血症为首发临床表现就诊消化内科最多。     

Standards of instrumentation of EMG

Standardization of Electromyography (EMG) instrumentation is of particular importance to ensure high quality recordings. This consensus report on “Standards of Instrumentation of EMG” is an update and extension of the earlier IFCN Guidelines published in 1999. First, a panel of experts in different fields from different geographical distributions was invited to submit a section on their particular interest and expertise. Then, the merged document was circulated for comments and edits until a consensus emerged.The first sections in this document cover technical aspects such as instrumentation, EMG hardware and software including amplifiers and filters, digital signal analysis and instrumentation settings. Other sections cover the topics such as temporary storage, trigger and delay line, averaging, electrode types, stimulation techniques for optimal and standardised EMG examinations, and the artefacts electromyographers may face and safety rules they should follow. Finally, storage of data and databases, report generators and external communication are summarized.     

Onset of action of antipsychotics in the treatment of mania

Introduction: An important consideration in treating acute mania is the promptness with which a chosen therapy can bring symptom amelioration. This article reviews the available published data from controlled, blinded studies regarding the latency of responses to antipsychotics in patients with acute mania.

Methods: Articles for this review were obtained from a search of the Medline database (1966–1999), using the following keywords and phrases: antipsychotic, atypical, bipolar disorder, mania, neuroleptic, typical. The bibliographic sections of articles gleaned from this search were used to direct further inquiries.

Results: Although information regarding the onset of action of antipsychotics is limited, we discovered data for four typical and three atypical antipsychotics. Drugs with the fastest onsets include haloperidol, risperidone, and olanzapine, with onsets appearing in 2–6 days. Chlorpromazine and thiothixene were at the slowest end of the continuum, with onsets of 2 weeks or longer. Data regarding pimozide are mixed, with some studies showing an onset equivalent to that of the 'fast' compounds and others showing one similar to that of the 'slow' compounds.

Conclusions: Choice of therapy should consider not only efficacy and safety, but also onset speed. Atypical antipsychotics appear to offer safer, faster, and more effective therapies.     

Function of circle of Willis

Nearly 400 years ago, Thomas Willis described the arterial ring at the base of the brain (the circle of Willis, CW) and recognized it as a compensatory system in the case of arterial occlusion. This theory is still accepted. We present several arguments that via negativa should discard the compensatory theory. (1) Current theory is anthropocentric; it ignores other species and their analog structures. (2) Arterial pathologies are diseases of old age, appearing after gene propagation. (3) According to the current theory, evolution has foresight. (4) Its commonness among animals indicates that it is probably a convergent evolutionary structure. (5) It was observed that communicating arteries are too small for effective blood flow, and (6) missing or hypoplastic in the majority of the population. We infer that CW, under physiologic conditions, serves as a passive pressure dissipating system; without considerable blood flow, pressure is transferred from the high to low pressure end, the latter being another arterial component of CW. Pressure gradient exists because pulse wave and blood flow arrive into the skull through different cerebral arteries asynchronously, due to arterial tree asymmetry. Therefore, CW and its communicating arteries protect cerebral artery and blood–brain barrier from hemodynamic stress.     

肌萎缩侧索硬化三例误诊分析

目的掌握肌萎缩侧索硬化(ALS)的诊断标准,以便早期准确诊断,避免误诊。方法分析3例ALS患者早期被误诊的临床资料。结果 3例患者均以下肢无力发病,逐渐波及上肢或对侧肢体,脊柱MR I示颈部或腰部椎间盘突出压迫硬膜囊,手术治疗后,症状无缓解,病情仍进行性加重,经肌电图检查证实为ALS。结论临床医师应熟知ALS的诊断标准,对患者详细询问病史、认真查体和电生理检查是减少ALS误诊的关键。     

失匹配负波在意识障碍评估中的临床价值探讨

自失匹配负波(MMN)于20世纪70年代被发现以来,我们对规律性声音被打破后所诱发的前注意检测有了进一步认识,而MMN成为了开启认知大门的钥匙。至今为止,MMN的研究范围从产生机制发展到神经精神疾病相关的临床试验,特别是对于急性脑损伤(ABI)昏迷以及进展后的慢性意识障碍(DoC)患者,MMN被认为是一个可靠的预后预测指标。然而,由于MMN难以用于个体评估,目前在临床实践中的应用仍十分有限,广大临床医师对MMN的了解甚少。因此,本文就MMN的产生机制、在意识障碍中的临床意义、判读方法及其影响因素做一综述。     

Characteristics of neuronal release of ATP

1. Recent studies have described a transmitter-like release of ATP in brain. Once released, extraneuronal ATP is rapidly metabolized to adenosine by ecto-ATPase and nucleotidase.

2. Adenosine, through actions at specific receptors, Inhibits neuronal firing in the brain. ATP shares these inhibitory actions, presumably by forming adenosine extraneuronally. Caffeine and theophylline probably exert CNS stimulation by antagonizing adenosine's inhibitory actions in the brain.

3. Extracellular ATP occasionally excites quiescent neurons in the cortex. A possible role for ATP as a sensory neurotransmitter is suggested by its excitatory actions on a subpopulation of dorsal horn cells.

4. ATP release has also been described from sensory nerves in the periphery, motor nerves, nerves of the myenteric plexus, bladder, vas deferens, and from adrenal chromaffin ceils and platelets. The possibility that ATP might function as a transmitter, cotransmitter or modulator in the peripheral nervous system is discussed.     

Frequency of accumulation of filaments in adaxonal cytoplasm of Schwann cells of myelinated fibers of rat spinal roots

Summary The frequency of accumulation of 6-nm filaments in the adaxonal cytoplasm of Schwann cells in the 6th lumbar dorsal and ventral roots was evaluated in 4-, 8-, 26- and 45-week-old Sprague-Dawley rats. The frequency was higher in 4- and 8-week-old (growing) rats than in 26- and 45-week old (mature) rats, and also higher in ventral than in dorsal roots in 4-, 8- and 26-week old rats. There were no clusters on certain groups of myelinated fibers according to the size of transverse axonal area, in both the ventral and dorsal roots. Therefore, this accumulation may reflect certain functions of the adaxonal cytoplasm of Schwann cell during natural growth and maturation of the axon and myelin sheath.     

The Institute of Epileptology of King's College, University of London

Summary: The Institute of Epileptology of King's College, London has arisen from need and from opportunity. The need is due to the relative neglect nationally and internationally of the most common serious brain disorder with important physical, psychological, and social complications. The relative neglect is reflected in services, research, charitable donations, public profile, and stigma and in a serious lack of professional education. The opportunity arose because of the existence in several medical institutions at Denmark Hill, London, of a group of medical and related colleagues with a special interest covering almost every aspect of this multidisciplinary disorder who agreed to combine their expertise in this initiative. The idea was born and developed in 1991–1992 and was supported by all the parent institutions: The Maudsley and King's College Hospitals, St Piers Lingfield, The Institute of Psychiatry, King's College School of Medicine and Dentistry, and the School of Life, Basic Medical and Health Sciences, all under the umbrella of King's College, University of London. Further stimulus and help came from a group of dedicated supporters in private and public life. There are three strands to this initiative: (a) a charity, The Fund for Epilepsy; (b) the clinical Centre for Epilepsy, which was formally opened at the Maudsley Hospital in July 1994; and (c) the academic Institute of Epileptology for research and teaching, which was launched on November 15, 1994.