进行性四肢麻木 无力4年

正病例摘要患者男性,42岁,主因四肢麻木、无力4年,于2016年3月24日入院。患者4年前(2012年初)无明显诱因出现全身乏力,剧烈运动耐力下降,易疲劳,症状无日间波动;此后逐渐出现双侧手指、足趾麻木,伴双手握力下降,日常生活活动能力无明显降低,无肌肉疼痛、视物模糊等,未予重视。约3年前(2013年6月)出现双下肢麻木、无力,下山、上楼费力,有时蹲起困难,伴脚踏不实,行走不稳、呈醉酒步态;此后逐渐出现双手骨间肌萎缩,尤以虎口显著,双足略下垂,穿衣、     

滥用笑气中毒致神经系统损害一例

正患者女性,20岁,主因四肢麻木、无力并进行性加重2周,排尿困难4 d,于2015年10月30日收入中日友好医院。患者于2周前在美国上学途中无明显诱因突然出现黑蒙、意识丧失伴跌倒,数分钟后自行恢复意识,能部分回忆发作时情景,无抽搐发作,此后,自觉行走时双下肢乏力、双足麻木,逐渐进展至双侧小腿麻木,行走不稳伴跌倒多次;病情进行性加重,1周前出现站立不能,双上肢活动不灵     

脊髓髓周动静脉瘘1例报告

患者,男,57岁,已婚工人,冬泳爱好者.因渐进性双下肢无力、麻木伴二便障碍8个月,于2009年10月9日入院.患者于8个月前无明显诱因出现双下肢无力、麻木,渐进性加重.     

行走不稳4个月双下肢麻木无力发作性抖动3个月

正患者男性,69岁,主因行走不稳4个月,双下肢麻木无力、发作性抖动3个月,于2015年11月10日入我院神经科。患者4个月前(2015年7月)无明显诱因出现行走不稳,不自主向左侧歪斜;约1个月后(2015年8月)出现双下肢麻木、无力,行走时前屈、前倾,伴静止时双下肢发作性抖动,动作幅度较大,持续数分钟,2~3次/d,夜间加重、活动或自行按摩后缓解,无头晕、头痛,无意识障碍等。外院头部MRI(2015年9月5日)显示,左侧侧脑室后角和周围占位性病     

腺垂体机能减退症合并Guillain-Barre综合征1例报告

腺垂体机能减退症合并Guillain-Barre综合征(GBS)临床少见,现报告1例如下. 1 病例 男,48岁.因"双下肢无力、麻木3个月"于2012年9月12日入院.患者3个月前无明显原因出现双下肢无力、酸痛、麻木,伴头昏、心慌、活动后气促,渐出现行走无力.无明显吞咽困难、饮水呛咳及呼吸困难,大小便正常.查体:神志清楚,精神萎靡,全身皮肤苍白,颜面浮肿,双上肢肌力V级,双下肢肌力Ⅳ级,四肢肌张力减低、腱反射消失,病理征(-),腓肠肌压痛,四肢手套-袜套样痛觉减退.     

以多发性周围神经病为突出表现的Churge-Strauss综合征1例报告

患者,女,61岁.因"双下肢疼痛、麻木、无力伴双手指麻木14d"于2007年4月20日入院.25d前出现双小腿内侧面对称性皮肤紫癜,自认为药物过敏,未治自愈,现留有褐色色素沉着;14d前开始出现双下肢疼痛、麻木、无力伴双手指麻木,并逐渐加重,在某医院诊断为"周围动脉闭塞".     

进行性四肢无力伴皮肤损害的病例讨论

病例资料患者住院号:634×××,女性,64岁.因"进行性四肢无力、麻木伴皮损1个月"于2010年1月8日入住复旦大学附属华山医院(我院)神经科. 现病史患者2009年12月初无明显诱因下出现双下肢麻木、无力,逐渐感行走费力,需他人扶持.12月12日出现周身红色皮疹,大小不一,直径0.5～4 cm,无破溃、瘙痒和疼痛;停药后皮疹未消退.12月16日出现四肢无力,麻木感明显,不能自行行走,双上肢持物不稳.12月30日无明显诱因下出现高热,体温最高达39.6℃,呈不规则发热,予抗生素治疗后(具体不详)体温仍在37.5℃左右波动;伴左眼疼痛,左眼视力进行性下降,同时有饮水呛咳、进食稀饭尚可.病程中无明显头疼,恶心,呕吐,咳嗽、咳痰、胸闷、气促、呼吸困难和大小便失禁.     

前列腺癌及嗜酸性粒细胞增多症合并多发性神经病变一例报告

前列腺癌及嗜酸性粒细胞增多症合并多发性神经病变一例报告乔学壮李华患者男性，６６岁。因双下肢麻木、无力３个月，加重伴双上肢无力１个月，于１９９５年８月１２日入院。患者３个月前始渐感双下肢麻木、无力，有时伴有刺痛。近１个月自觉上述症状加重，且出现双上肢麻...     

中枢神经系统表面铁沉积症1例报道

<正>1病例介绍患者女性,74岁,主因“走路不稳、双下肢麻木4年,双上肢麻木疼痛1年”于2021年11月1日入院。患者4年前无明显诱因逐渐出现走路不稳、行走蹒跚、双下肢麻木,伴双手持物时轻微不自主抖动,言语停顿,伴头晕、情绪低落,无头部、肢体静止时抖动,无肢体僵硬、表情僵硬,无肢体无力、言语不清、口角流涎,无精神行为异常、睡眠中行为异常、记忆力减退,无嗅觉减退、便秘、二便失禁,无吞咽困难、饮水呛咳、视物成双。     

貌似多发性硬化的髓内肿瘤1例报告

患者 女性,18岁,因双下肢渐进性麻木无力伴大小便障碍1年余入院。患者于1990年底觉左下腹搔痒,左下肢麻木,走路易掉鞋,经用地巴唑后好转;1991年7月左下肢又出现麻木,一周后自行缓解;同年9月左下肢麻木伴无力明显,且右下肢亦觉无力,行走要扶持,当地医院诊断不明,用激素治疗有所缓解。11月双下肢无力加重,且件大小便困难在当地住院。腰穿脑脊液未发现异常,椎管碘油造影无梗阻,视诱发电位双P_(100)潜伏期延长,病初主诉有一过性视蒙,拟多发性硬化再以激     

Efficacy and Effectiveness of Child and Adolescent Psychotherapy and Pharmacotherapy

Decades of intervention research have produced a rich body of evidence on the effects of psychotherapies and pharmacotherapies with children and adolescents. Here we summarize and critique that evidence. We review findings bearing on the efficacy of psychosocial treatments and medications under controlled experimental conditions. We also report evidence, where available, on the effectiveness of both classes of treatment with clinically referred youth treated in real-world clinical contexts. In general, the large body of evidence on efficacy contrasts sharply with the small base of evidence on effectiveness. Addressing this gap through an enriched research agenda could contribute importantly to linking scientific inquiry and clinical practice—to the benefit of both ventures. This is one element of a multifaceted agenda for future research and for synthesis of research, which will require the interplay of multiple disciplines related to child and adolescent mental health.     

Satellite cells and myonuclei in young and elderly women and men

The overall aim of this study was to assess the effects of aging on the satellite cell population. Muscle biopsies were taken from the tibialis anterior muscle of healthy, moderately active young (age range, 20-32 years; n = 31) and elderly (age range, 70-83 years; n = 27) women and men with comparable physical activity pattern. Satellite cells and myonuclei were visualized using a monoclonal antibody against neural cell adhesion molecule and counterstained with Mayer's hematoxylin. An average of 211 (range, 192-241) muscle fibers were examined for each individual. Compared with the young women and men, the elderly subjects had a significantly lower (P < 0.011) number of satellite cells per muscle fiber but a significantly higher (P < 0.004) number of myonuclei per muscle fiber. The number of satellite cells relative to the total number of nuclei [satellite cells/(myonuclei + satellite cells)] was significantly lower in the elderly than in the young women and men. These results imply that a reduction in the satellite cell population occurs as a result of increasing age in healthy men and women.     

Necdin蛋白与神经元的生长分化

在神经系统 ,Necdin只在成熟神经元的细胞核中表达 ,可能与成熟神经元分裂静止状态的保持有关。近年的研究表明 ,Necdin是一种生长抑制蛋白 ,能与多种因子如SV4 0大T抗原 ,腺病毒E1A ,转录因子E2F1以及肿瘤抑制蛋白p5 3等结合 ,在功能上类似于成视网膜瘤蛋白Rb。necdin基因缺陷时 ,会引起脑内 ,特别是下丘脑神经元分化障碍。人类necdin基因位于PWS综合征的基因缺失区 ,可能与PWS的一些症状有关。本文从Necdin蛋白的基本概况 ,生物功能以及Necdin与疾病三个方面进行了综述     

Retrospective and prospective design and data

The study of the presentation, symptomatology and family characteristics of an exclusively adolescent sample of patients with borderline personality disorder (BPD) was undertaken. Twenty-four cases of borderline personality disorder, 20 females, 4 males, identified using chart review and meeting the criteria of the Diagnostic Interview for Borderlines (DIB) and DSM III-R, were matched with psychiatric controls. Adolescents with borderline personality disorder were found to have high rates of affective symptomatology with Axis I diagnosis of major depressive disorder - MDD (DSM-III-R), and high rates of interpersonal psychopathology, i.e., manipulation, devaluation, and a pervasive sense of boredom. The latter seem to be characteristic as for adults with borderline personality disorder. The families were particularly angry and volatile.     

Cortisol levels and depression in men and women using heroin and cocaine

Abnormalities in the hypothalamic-pituitary-adrenal (HPA) axis are well documented in men using illicit drugs and/or infected with HIV; however, less is known about HPA function, or the health consequence of HPA dysfunction, in their female counterparts. People with depression exhibit hypercortisolemia, and depression is common in people with HIV or substance use problems. The current study investigated cortisol secretion in 209 demographically matched men and women, stratified by their HIV and drug use status. Self-reported depressive symptoms were evaluated using a standardized, validated questionnaire (CES-D). Women reported more depressive symptoms than men (p=.01). Male and female drug users exhibited higher cortisol concentrations (p=.03), and were more likely to report depressive symptoms (p=.04), than non-users. Depression was related to elevated cortisol concentrations for the study population (p=.03), and women with elevated cortisol concentrations were significantly more depressed than all other participants (p=.05). While it is unknown whether high cortisol concentrations precede depressive symptoms or vice versa, these data indicate that higher cortisol concentrations are associated with depressive symptoms in heroin and cocaine users, and that this association is more pronounced in women than men. HIV status did not act in an additive or synergistic way with drug use for either cortisol or CES-D measures in the current study. Unique therapies to treat the endocrine and mental health consequences of illicit drug use in men and women deserve consideration as depressive symptoms, and high cortisol concentrations associated with depressive symptoms, differ by gender.     

Opiate and digitalis receptor assays distinguish between neuroexcitant and inactive organochlorines and between anesthetics and convulsants

Specific binding of [3H]naloxone to rat brain tissue in vitro was inhibited by the excitant organochlorinated insecticides (OCI), by ether (E) and octanol (OCT), and by the convulsant indoklon (IND) and its anesthetic isomer, isoindoklon (ISO). In the presence of 100 mM NaCl the inhibition of naloxone binding by E, OCT and ISO was greatly potentiated, whereas that by OCI and IND was attenuated. KCl (100 mM) was equally effective as NaCl on the action of anesthetics, but the effect of the excitant drugs was, in contrast to NaCl, unaffected by KCl. Specific binding of [3H]ouabain in the absence of Na, was depressed by anesthetics and enhanced by neuroexcitants. In the presence of NaCl, which by itself inhibits ouabain binding to brain, both anesthetics and excitants enhanced ouabain binding. DDE, a non-insecticidal analog of DDT, and the dimethyl derivative of the OCI, lindane, were inactive in the receptor assays. These observations point to a unique isolated system which responds consistently to anesthetic agents as a class and, in a different way, to neuroexcitant compounds.     

Learning and forgetting new names and objects in MCI and AD

We studied how subjects with mild cognitive impairment (MCI), early Alzheimer's disease (AD) and age-matched controls learned and maintained the names of unfamiliar objects that were trained with or without semantic support (object definitions). Naming performance, phonological cueing, incidental learning of the definitions and recognition of the objects were tested during follow-up. We found that word learning was significantly impaired in MCI and AD patients, whereas forgetting patterns were similar across groups. Semantic support showed a beneficial effect on object name retrieval in the MCI group 8 weeks after training, suggesting that the MCI patients’ preserved semantic memory can compensate for impaired episodic memory. The MCI group performed equally well as the controls in the tasks measuring incidental learning and recognition memory, whereas the AD group showed impairment in this respect. Both the MCI and the AD group benefited less from phonological cueing than the controls. Our findings indicate that word learning is compromised in both MCI and AD, whereas long-term retention of newly learned words is not affected to the same extent. Incidental learning and recognition memory seem to be well preserved in MCI.     

相关性和一致性：这对相仿概念和测量方法的回顾与阐明

概述：一致性(agreement)和相关性(correlation)是两个广泛使用的概念,用来评估变量之间的关联。虽然二者相似且相关,但是它们代表关联完全不同的概念。评估变量之间的一致性假设变量测量的是相同的结构,而在变量测量完全不同的结构时也可以评估它们之间的相关性。这种概念上的差异就要求使用不同的统计方法,并且当评估一致性或相关性时,统计方法根据数据的分布和研究者的兴趣可能会有所不同。例如, Pearson相关性,作为评估连续变量之间相关性的一种普遍测量方法,只有用于符合线性关系的变量时才能提供有用的信息;当用于不符合线性关系的变量时就无法提供准确信息甚至会产生误导。同样地,内部相关性,作为一种评估连续变量之间一致性的常用方法,如果一致性不好的实质正好是研究兴趣所在,那么该测量就不能为研究者提供充分的信息。本报告回顾了一致性和相关性的概念,并讨论了几种常用方法在应用中的差异。     

Necdin蛋白与神经元的生长分化

在神经系统,Necdin只在成熟神经元的细胞核中表达,可能与成熟神经元分裂静止状态的保持有关.近年的研究表明,Necdin是一种生长抑制蛋白,能与多种因子如SV40大T抗原,腺病毒E1A,转录因子E2F1以及肿瘤抑制蛋白p53等结合,在功能上类似于成视网膜瘤蛋白Rb.necdin基因缺陷时,会引起脑内,特别是下丘脑神经元分化障碍.人类necdin基因位于PWS综合征的基因缺失区,可能与PWS的一些症状有关.本文从Necdin蛋白的基本概况,生物功能以及Necdin与疾病三个方面进行了综述.     

Oxytocin and vasopressin agonists and antagonists as research tools and potential therapeutics

We recently reviewed the status of peptide and nonpeptide agonists and antagonists for the V(1a), V(1b) and V(2) receptors for arginine vasopressin (AVP) and the oxytocin receptor for oxytocin (OT). In the present review, we update the status of peptides and nonpeptides as: (i) research tools and (ii) therapeutic agents. We also present our recent findings on the design of fluorescent ligands for V(1b) receptor localisation and for OT receptor dimerisation. We note the exciting discoveries regarding two novel naturally occurring analogues of OT. Recent reports of a selective VP V(1a) agonist and a selective OT agonist point to the continued therapeutic potential of peptides in this field. To date, only two nonpeptides, the V(2) /V(1a) antagonist, conivaptan and the V(2) antagonist tolvaptan have received Food and Drug Administration approval for clinical use. The development of nonpeptide AVP V(1a), V(1b) and V(2) antagonists and OT agonists and antagonists has recently been abandoned by Merck, Sanofi and Pfizer. A promising OT antagonist, Retosiban, developed at Glaxo SmithKline is currently in a Phase II clinical trial for the prevention of premature labour. A number of the nonpeptide ligands that were not successful in clinical trials are proving to be valuable as research tools. Peptide agonists and antagonists continue to be very widely used as research tools in this field. In this regard, we present receptor data on some of the most widely used peptide and nonpeptide ligands, as a guide for their use, especially with regard to receptor selectivity and species differences.