Features of “ET plus” correlate with age and tremor duration: “ET plus” may be a disease stage rather than a subtype of essential tremor

BackgroundEssential tremor (ET) is characterized by considerable clinical heterogeneity. In 2018, the term “ET plus” was introduced to mark a potential stratification point for dividing ET into subtypes – ET vs ET plus (i.e., ET cases with neurological features other than action tremor). However, as ET progresses, patients often develop increasingly severe tremor, spread of tremor, tremor under different activation conditions, and other features. Given this situation, ET plus may represent a disease stage rather than a disease classification or subtype. In theory, if the defining characteristics of a disease subtype fluctuate with age or disease duration, it raises the distinct possibility the “subtype” is a disease stage.MethodsA cohort of 241 prospectively enrolled ET cases underwent a detailed motor and cognitive assessment in which the features of ET plus including cerebellar signs (intention tremor, tandem gait difficulty), rest tremor, dystonia, and cognitive performance were evaluated. We determined whether these features of ET plus correlated with action tremor duration and age.ResultsWe demonstrated that numerous ET plus features were significantly correlated with both age and action tremor duration (numerous p values < 0.05). The same relationships were observed in a series of sensitivity analyses.ConclusionWe observed that the component parts of ET plus are highly age- and stage-dependent. These features are yearly-changing features conditional on a demographic and disease stage variable. These data support the notion that ET plus may represent a disease stage rather than a distinct disease subtype or disease classification.     

Moderate early life stress improves adult zebrafish (Danio rerio) working memory but does not affect social and anxiety‐like responses

Early life stress (ELS) is defined as a short or chronic period of trauma, environmental or social deprivation, which can affect different neurochemical and behavioral patterns during adulthood. Zebrafish (Danio rerio) have been widely used as a model system to understand human neurodevelopmental disorders and display translationally relevant behavioral and stress‐regulating systems. In this study, we aimed to investigate the effects of moderate ELS by exposing young animals (6‐weeks postfertilization), for 3 consecutive days, to three stressors, and analyzing the impact of this on adult zebrafish behavior (16‐week postfertilization). The ELS impact in adults was assessed through analysis of performance on tests of unconditioned memory (free movement pattern Y‐maze test), exploratory and anxiety‐related task (novel tank diving test), and social cohesion (shoaling test). Here, we show for the first time that moderate ELS increases the number of alternations in turn‐direction compared to repetitions in the unconditioned Y‐maze task, suggesting increased working memory, but has no effect on shoal cohesion, locomotor profile, or anxiety‐like behavior. Overall, our data suggest that moderate ELS may be linked to adaptive flexibility which contributes to build “resilience” in adult zebrafish by improving working memory performance.     

MEBO治疗慢性难愈性皮肤溃疡的临床疗效观察

目的观察湿润烧伤膏(Moist Exposed Burn Ointment,MEBO)治疗慢性难愈性皮肤溃疡的临床疗效。方法对2010年1月-2014年12月收治的344例慢性难愈性皮肤溃疡患者创面采用"药刀结合"的方法治疗,药即MEBO,刀即外科清创术、植皮术等。患者初诊时尽量清除创面坏死组织,不能耐受一次性清创者行蚕食疗法;清创后创面均匀涂抹MEBO并外敷MEBO药纱包扎治疗,存在潜腔的创面用MEBO药纱填塞引流,待肉芽组织填满创面后行肉芽创面游离植皮术。观察患者创面愈合时间及愈合情况。结果344例患者中除38例患者好转后自动出院,3例患者死于基础疾病外,其余303例患者创面均完全愈合,愈合率达88.1%,创面平均愈合时间为80.4 d±15.8 d。其中,193例患者经MEBO换药治疗后创面自行愈合(56.1%);48例合并趾骨坏死的糖尿病足患者进行了坏死组织剜除术(14.0%);34例骨外露创面进行了钻孔术(9.9%);54例患者进行了肉芽创面植皮术(包括接受趾骨剜除术及骨钻孔术后的创面)。89例烧伤残余创面愈合较快,最短为7 d,最长为29 d,平均愈合时间为18.8 d±4.9 d。结论MEBO可有效控制创面感染,促进坏死组织液化脱落及肉芽组织生长,缩短创面愈合时间,减轻患者疼痛,适时配合"刀法"还可进一步加速坏死组织脱落,促进肉芽组织生长,及时封闭创面,减少瘢痕增生,是治疗慢性难愈性皮肤溃疡的理想方法。     

Novel B3GALTL mutations in classic Peters plus syndrome and lack of mutations in a large cohort of patients with similar phenotypes

Peters plus syndrome (PPS) is a rare autosomal‐recessive disorder characterized by Peters anomaly of the eye, short stature, brachydactyly, dysmorphic facial features, developmental delay, and variable other systemic abnormalities. In this report, we describe screening of 64 patients affected with PPS, isolated Peters anomaly and PPS‐like phenotypes. Mutations in the coding region of B3GALTL were identified in nine patients; six had a documented phenotype of classic PPS and the remaining three had a clinical diagnosis of PPS with incomplete clinical documentation. A total of nine different pathogenic alleles were identified. Five alleles are novel including one frameshift, c.168dupA, p.(Gly57Argfs*11), one nonsense, c.1234C>T, p.(Arg412*), two missense, c.1045G>A, p.(Asp349Asn) and c.1181G>A, p.(Gly394Glu), and one splicing, c.347+5G>T, mutations. Consistent with previous reports, the c.660+1G>A mutation was the most common mutation identified, seen in eight of the nine patients and accounting for 55% of pathogenic alleles in this study and 69% of all reported pathogenic alleles; while two patients were homozygous for this mutation, the majority had a second rare pathogenic allele. We also report the absence of B3GALTL mutations in 55 cases of PPS‐like phenotypes or isolated Peters anomaly, further establishing the strong association of B3GALTL mutations with classic PPS only.     

静脉溶栓后神经功能恶化的治疗观察

目的 探讨抗凝和抗血小板聚集治疗脑梗死静脉溶栓后神经功能恶化的疗效。方法 收集自2015年1月至2019年12月在石家庄市第一医院神经内科经静脉溶栓后神经功能恶化的62例急性脑梗死患者,行头颅CT除外出血转化后随机分为治疗组和对照组,各31例,治疗组采用单抗加抗凝,即阿加曲班联合氯吡格雷治疗,对照组采用双抗,即阿司匹林联合氯吡格雷治疗,比较两组治疗静脉溶栓后神经功能恶化的临床疗效及不良反应情况。结果 治疗14天后治疗组NIHSS评分较对照组偏低（P<0.05）,治疗组mRS评分亦低于对照组（P<0.05）。治疗组发生消化道出血1例,恶心、呕吐1例,对照组发生消化道出血1例,恶心、呕吐2例,对照组和治疗组不良反应的差异不显著。结论 采用阿加曲班联合氯吡格雷治疗,减轻静脉溶栓后神经功能恶化症状,减轻残疾程度,疗效显著,且不会出现严重不良反应,安全可靠。     

“互联网+”健康教育在体检中心中的应用

目的:探讨"互联网+"健康教育在体检中心中的应用效果。方法:在湖北省十堰市人民医院体检中心2017年9-12月进行体检的患者中抽取510例,按照随机数字表法1:1比例分组为干预组(255例)和对照组(255例),对照组采用常规健康教育,干预组通过体检中心的护士采用微信、App,进行为期6个月的院外健康教育的方式,为患者推送精准、个性化的健康教育内容。评价两组患者饮食、运动、血压、血脂达标情况和护理满意度。结果:干预组在干预后6个月饮食、运动、血压、血脂达标率显著高于对照组,护理满意度高于对照组,差异有统计学意义(P<0.05)。结论:对体检人员实施"互联网+"健康教育模式,可有效改善体检者的生活方式,有助于体检者建立良好的生活行为习惯。     

冲洗式射频消融迷宫手术治疗不同病因永久性心房颤动的疗效比较

目的分析和比较我院冲洗式射频消融迷宫手术治疗不同病因永久性心房颤动（房颤）的疗效。方法：收集我院201 1年1月1日-2012年12月31日外科冲洗式射频消融迷宫手术治疗永久性房颤502例病例资料进行分析。根据病因（手术方式）不同分为两组：瓣膜置换并发冲洗式射频消融迷宫手术（瓣膜病组）406例;非瓣膜置换并发冲洗式射频消融迷宫手术（非瓣膜病组）96例。分别于术中心脏复跳、术后1周、3个月和6个月行超声心动图及心电图检查,观察左房、左室大小及窦性心律维持情况。结果：两组术后各时点心脏转复率均无显著差异,两组左房内径均较术前显著缩小。就全部患者而言,术中心脏转为窦性心律者为358例,转复率为71．3％;术后1周为376例,转复率为74．9％;术后3个月为347例,转复率为69．1％。术后6个月为339例,转复率为67．5％。总体手术死亡3例,2例死于低心排,1例死于恶性心律失常（室颤）,手术死亡率为0．6％。结论：冲洗式射频消融迷宫手术治疗不同病因永久性房颤的疗效没有显著差异,疗效均较好。     

Sex differences in circadian timing systems: Implications for disease

Virtually every eukaryotic cell has an endogenous circadian clock and a biological sex. These cell-based clocks have been conceptualized as oscillators whose phase can be reset by internal signals such as hormones, and external cues such as light. The present review highlights the inter-relationship between circadian clocks and sex differences. In mammals, the suprachiasmatic nucleus (SCN) serves as a master clock synchronizing the phase of clocks throughout the body. Gonadal steroid receptors are expressed in almost every site that receives direct SCN input. Here we review sex differences in the circadian timing system in the hypothalamic–pituitary–gonadal axis (HPG), the hypothalamic–adrenal–pituitary (HPA) axis, and sleep–arousal systems. We also point to ways in which disruption of circadian rhythms within these systems differs in the sexes and is associated with dysfunction and disease. Understanding sex differentiated circadian timing systems can lead to improved treatment strategies for these conditions.