西藏地区低钾型周期性瘫痪15例患者临床分析

目的探讨西藏地区低钾型周期性瘫痪(HPP)患者的发病原因、临床特征、治疗效果,提出西藏地区HPP临床特点。方法分析拉萨市人民医院入院收治的15例HPP患者的临床资料。结果 15例HPP患者均为散发性,男性13例,女性2例,2例合并发生甲亢,2例原发性高血压,明确诱因者仅5例,其他患者除饮食结构简单外无明显诱因。四肢瘫痪对称者9例,不对称者6例,所有患者经补钾治疗后临床症状显著改善。结论西藏地区HPP低钾比较严重,1/3(5/15)患者诱因与饮酒、感冒、腹泻等相关外,其他患者主要与饮食结构简单可能相关;四肢不对称性软瘫是周期性瘫痪的常见类型,心电图(ECG)改变不典型;补钾治疗效果明显,且肌力好转早于血清钾水平改善。     

TTKG在低钾型周期性瘫痪诊断和治疗中的意义

目的 探讨经肾小管尿钾浓度梯度(transtubular potassium concentration gradient, TTKG)测定在低钾型周期性瘫痪(hypokalemic periodic paralysis, HPP)诊断和治疗中的意义.方法 前瞻性选择低钾型周期性瘫痪患者64例,根据病因分为原发组、继发组和甲亢组,健康志愿者25名作为对照组.测定血/尿钾浓度和渗透压,根据其结果计算TTKG,比较其组间TTKG的差异;按TTKG分组,比较组间补钾总量和症状恢复时间的差异,统计补钾治疗后出现反跳性高血钾的情况.结果 原发组TTKG为(1.74±0.44),继发组为(4.59±1.05),甲亢组为(1.74±0.55),正常对照组为(7.21±1.38),比较有统计学意义(P<0.01);原发性HPP患者应少补钾,相反,继发性HPP应多补钾.结论 TTKG可在早期作为鉴别原发性与继发性HPP的指标之一,并指导治疗,避免补钾治疗后出现反跳性高钾血症.     

低钾性周期性麻痹150例临床分析

目的:探讨低钾性周期性麻痹(HPP)的临床特点。方法:分析150HPP例临床表现及辅助检查结果。结果:男女比例14:1,20～40岁为发病高峰年龄,53.3％(80/150)继发于甲亢,肌腱反射正常和活跃者超过56.9％(45/79),65.7％(23/35)患者血清CPK升高。结论:HPP最好发于青年男性,多继发于甲亢;可有肌腱反射正常或活跃以及血清CPK升高。     

Andersen-Tawil综合征1例报告并文献复习

目的探讨Andersen-Tawil综合征(ATS)的发病机制及临床特点。方法回顾性分析1例ATS患者的临床表型。结果本例患者男,17岁,表现为低位耳,反复发作运动、饱食或无明显诱因下非对称性肌无力,入院期间肌无力发作时伴血钾降低;动态ECG提示频发室性期前收缩;EMG检查显示复合肌肉动作电位波幅在运动诱发试验运动后即刻上升6%,30 min下降17%,60 min下降25%,90 min下降35%;头颅MRI Flair示左侧额顶叶交界区脑白质内点状高信号影;基因检测(NGS)提示染色体17q24的KCNJ2基因出现错义突变:c.224C>T(p.Thr75Met),诊断为ATS。予补钾治疗后患者肌力恢复正常,出院后继续服用醋钾唑胺片,随访发现患者周期性麻痹的发作频率、严重程度及持续时间明显改善。结论ATS作为一个多系统累积的离子通道病,其临床表现高度变异,临床上要更加关注患者临床表型,从而有利于表型及机制的完善。     

外来务工人员低钾性周期性麻痹52例临床分析

目的探讨外来务工人员低钾型周期性麻痹（HoPP）的特点。方法回顾性调查结合追踪随访对东莞市桥头医院2004-01-2005-12收治的52例外来务工人员HoPP的临床资料进行分析。结果52例低钾性周期性麻痹患者全部为外来务工人员,经治疗均痊愈。发病诱因主要为剧烈运动或劳累、情绪激动、焦虑。男性发病明显多于女性,男∶女为9∶1。患者以青年为主,占92.3%。伴有甲亢者占23%。结论外来务工人员发病诱因比较明显,劳累、心理状态、社会因素与这一特殊群体的HoPP发病有一定关系。     

低钾性周期性麻痹的神经电生理观察

目的：充分了解低钾性周期性麻痹患者神经传导的特点。方法：对比35例低钾性周期性麻痹患者于补钾治疗前后神经传导的远端潜伏期、波幅、传导速度、F波、H反射的变化。结果：10例发作期存在感觉神经传导速度减慢;31例发作期存在运动神经传导远端潜伏期延长,传导速度慢或波幅低,F波、H反射潜伏期正常或延长;补钾治疗至血钾正常后,神经传导完全恢复正常。结论：低钾性周期性麻痹可以出现感觉神经传导障碍,有感觉异常症状。     

青少年起病的重症肌无力患者临床特点分析

目的分析青少年起病的重症肌无力(MG)患者的临床特点。方法回顾性分析2006-2013年作者医院神经内科住院的351例起病年龄≤18岁MG患者的临床资料,包括人口统计学(性别、年龄),临床特点(临床分型、起病症状、疾病转归、胸腺情况)和合并疾病(自身免疫性疾病)。结果青少年起病的MG患者发病年龄小于5岁者占45.9%,女性多见占53.6%。根据美国重症肌无力协会(myasthenia gravis foundation of America,MGFA)分型,Ⅰ型占80.3%,Ⅱa型占8.5%,Ⅱb型占9.1%,Ⅲa型占0.3%,Ⅲb型占1.4%,Ⅳb型占0.3%。由眼肌型MG(OMG)转化为全身型MG(GMG)患者39例(13.8%),主要诱发转化的因素为不规律用药或停药。诱发病情复发或加重的因素中以受凉多见,占63.6%。合并胸腺异常患者有60例,其中28例行胸腺切除术,术后病理分型:胸腺增生20例,胸腺瘤4例。合并有其他自身免疫性疾病共20例,其中甲状腺功能亢进占4.6%,甲状腺功能减低占0.6%,甲状腺囊肿占0.6%。共有6例患者发生过危象,其中5例发生肌无力危象者为由OMG转化为GMG患者。结论青少年MG患者有自己独特的临床特点:5岁之前发病更多见;诱发病情复发或加重的因素中以受凉多见;合并胸腺增生者多于合并胸腺瘤者;最常见的甲状腺功能异常为甲状腺功能亢进。     

低钾性周期性瘫痪102例临床分析

目的 :观察低钾性周期性瘫痪的临床特点 ,探讨其治疗方法。方法 :分析 10 2例病人的临床表现、辅助检查结果及其不同补钾方法。结果 :本组病人均四肢软瘫、肌张力均减低、腱反射呈多样性 ,均无病理反射。 18%为继发性周期性瘫痪 ,90 %心电图异常 ,口服补钾明显优于静脉补钾。结论 :根据低钾性周期性瘫痪的临床特点 ,不难作出正确的诊断和恰当处理 ,口服补钾明显优于其他补钾方法     

早期就诊的急性缺血性卒中病人未溶栓原因分析

目的:研究6小时内到达医院就诊的急性缺血性脑卒中未进行溶栓治疗的原因。方法:通过对实施急性脑血管病急诊绿色通道1年期间,发病6小时内就诊的患者未进行溶栓治疗的原因进行分析。结果:166例缺血性脑卒中患者在发病6小时内经急诊绿色通道就诊,81例符合溶栓条件的患者中47例接受溶栓治疗,占符合溶栓条件患者28.31%。溶栓患者平均发病时间(211.70±86.10)min,NIHSS评分10(范围5～22)。静脉溶栓25例,动脉溶栓22例。从发病至静脉溶栓平均开始时间为(55.48±26.01)min,明显短于动脉溶栓平均开始时间(86.59±40.40)min(P=0.003)。119例未进行溶栓治疗患者中不符合条件85例,符合条件而未溶34例(占20.48%)。发病6小时内就诊的患者未溶栓的原因有神经功能障碍轻或明显改善、早期显示病灶、脑栓塞以及家属或患者拒绝。结论:发病6小时内就诊的患者未进行溶栓的可调整原因主要是家属或患者拒绝。加强公众对脑卒中的了解及接受程度有助于提高溶栓比例。     

糖尿病性非酮症偏侧舞蹈症16例临床分析

目的分析糖尿病性非酮症偏侧舞蹈症患者临床表现与预后。方法收集在本院收治的16例非酮症糖尿病并伴有偏侧舞蹈症的一般资料、入院情况、临床症状和体征、实验室检查、影像学检查结果、治疗和预后等资料,总结其临床特点和预后。结果 16例患者急性起病12例,占75.0%;临床症状主要为肢体一侧不自主、无规律的类似舞蹈样的动作12例,占75.0%;发病时血糖13.8~26.3mmol·L~(-1),平均发病时血糖(21.3±4.6)mmol·L~(-1)。16例患者CT显示:壳核高密度6例(37.50%),尾状核高密度4例(25.0%),双侧纹状体高密度3例(17.58%),CT未见异常者3例(18.75%);MRI显示:壳核不均匀高信号7例(43.75%),尾状核不均匀高信号6例(37.50%),右颞叶不均匀高信号3例(18.75%);所有患者给予常规胰岛素进行血糖控制在7.7~11.0mmol·L~(-1),给予氟哌啶醇、奋乃静等药物对症治疗,治疗时间22~41d,平均治疗时间28.61±5.14d;出院时患者症状消失14例(87.50%),在出院时仍然有不同程度的偏侧舞蹈症状2例(13.50%)。结论糖尿病性非酮症偏侧舞蹈症患者主要表现为一侧不自主、无规律的类似舞蹈样的动作,控制血糖后给予氟哌啶醇、奋乃静等药物对症治疗预后良好。     

Neuroimaging in epilepsy in tropics

Epilepsy is a major public health problem in many tropical countries. Also, some of the tropical diseases are major contributors to the higher prevalence of epilepsy in these countries. The etiologic factors responsible for epilepsy in these countries are quite different from those in the developed world. This article discusses the etiologic factors and neuroimaging of epilepsy in light of the conditions in these tropical countries.     

癫痫与抑郁关系的研究进展

抑郁是癫痫患者中常见的精神障碍,严重地影响了患者的生活质量。传统的观点认为癫痫患者因为存在着诸多社会学问题易出现抑郁倾向,癫痫和抑郁是单向的联系,但大量的研究已经证明癫痫和抑郁之间存在双向的联系,一种异常状态的存在可能易转化为另一种异常状态的发展。癫痫和抑郁存在着共同的发病机制。本文主要就癫痫和抑郁的双向联系以及抗抑郁药物在癫痫患者中的应用进行阐述。     

Sudanophilic lipid accumulation in astrocytes in periventricular leukomalacia in monkeys

Summary Sudanophilic lipid accumulation is a characteristic feature of periventricular leukomalacia (PVL) of infants. At least two types of lipid-containing cells have been identified, one being the macrophage, the other the pre-myelin glial cell. A third type of lipid-containing cell has been seen in two monkeys with spontaneous PVL. Electron microscopically this cell appears to be an astrocyte. This probably represents a reaction of the astrocyte to hypoxia and may be the equivalent of the hypertrophic astrocytes found in human infants.Supported in part by NIH grant HDO 8633 and the Regional Primate Research Center Grant RR-00166     

Increase in cathepsin D activity in rat brain in aging

Cathepsin D-like activity in homogenates of five brain areas of 3-month-old and 24-month-old Fischer 344 rats was measured. With hemoglobin as substrate at pH 3.2, more than 90% of the activity was inhibited by pepstatin. In each area studied, activity was more than twice as high in the old rat brain: 140-160% higher in the cortex, cerebellum, pons-medulla, and striatum and 90-100% higher in the hippocampus and spinal cord. The greatly increased metabolic capacity in the absence of an increase in protein turnover may have a role in age-related pathological degeneration in the brain.     

Characteristics of nociceptors in the periodontium--an in vitro study in rats

Recent studies have indicated that nociceptors can be classified into various types according to their physiological properties. These studies have clarified that the frequency distribution of various nociceptor types is different among body sites and animal species. In the present study, we investigated the physiological properties of rat's periodontal nociceptors in an in vitro jaw-nerve preparation. Responses were recorded from functional single filaments in the inferior alveolar nerve. To determine the nociceptor type, calibrated von Frey filaments, heat, and bradykinin (BK) stimuli were used. We found five subtypes of nociceptors in the periodontal ligaments of the lower incisor: Adelta-high threshold mechanonociceptors (Adelta-HTM, n=28), Adelta-mechanoheat nociceptors (Adelta-MH, n=6), Adelta-polymodal nociceptors (Adelta-POLY, n=26), C-high threshold mechanonociceptors (C-HTM, n=3) and C-polymodal nociceptors (C-POLY, n=4). Most nociceptors were Adelta-innervated, while only a small number of C-innervated nociceptors were found. The present results suggest that periodontal nociceptors transmit mainly fast pain, and may thus play a role in rapid detection of injure-related stimuli during mastication.     

Gender in Voice Perception in Autism

Deficits in the perception of social stimuli may contribute to the characteristic impairments in social interaction in high functioning autism (HFA). Although the cortical processing of voice is abnormal in HFA, it is unclear whether this gives rise to impairments in the perception of voice gender. About 20 children with HFA and 20 matched controls were presented with voice fragments that were parametrically morphed in gender. No differences were found in the perception of gender between the two groups of participants, but response times differed significantly. The results suggest that the perception of voice gender is not impaired in HFA, which is consistent with behavioral findings of an unimpaired voice-based identification of age and identity by individuals with autism. The differences in response times suggest that individuals with HFA use different perceptual approaches from those used by typically developing individuals.     

Defects in Bioenergetic Coupling in Schizophrenia

Synaptic neurotransmission relies on maintenance of the synapse and meeting the energy demands of neurons. Defects in excitatory and inhibitory synapses have been implicated in schizophrenia, likely contributing to positive and negative symptoms as well as impaired cognition. Recently, accumulating evidence has suggested that bioenergetic systems, important in both synaptic function and cognition, are abnormal in psychiatric illnesses such as schizophrenia. Animal models of synaptic dysfunction demonstrated endophenotypes of schizophrenia as well as bioenergetic abnormalities. We report findings on the bioenergetic interplay of astrocytes and neurons and discuss how dysregulation of these pathways may contribute to the pathogenesis of schizophrenia, highlighting metabolic systems as important therapeutic targets.     

Ethnic disparities in problem behaviour in adolescence contribute to ethnic disparities in social class in adulthood

BACKGROUND: It is important for prevention of social class disparities to know how ethnic disparities in social class arise among migrant children. We contribute to this understanding by examining the role of problem behaviour in adolescence. METHODS: Prospective observational study with 753 Dutch native and 217 Turkish migrant adolescents (11-18 year) followed for 10 years. Internalising and externalising problems were assessed in adolescence and employment status and occupational level were assessed in adulthood. The difference in odds ratios (OR) before and after adjustment for internalising and externalising problems was an indication of the predictive value of disparities in internalising and externalising problems for the development of social class disparities. RESULTS: A total of 135 (62%) of the Turkish and 602 (80%) of the Dutch adults were employed. Internalising and externalising problems were not associated with employment status. Of the employed, 65 (48%) Turkish and 179 (30%) Dutch adults worked in low-level occupations (p < 0.0001). Internalising and externalising problems were associated with both ethnicity and occupation. The OR for low-level occupation for Turkish adults was 1.78 (1.19-2.65), indicating ethnic disparities. Adjustment for internalising problems lowered the OR with 36% to 1.50 (0.97-2.31), and adjustment for externalising problems lowered it with 8% to 1.72 (1.15-2.57). Findings were similar for men and women and did not vary by age. CONCLUSIONS: Ethnic disparities in occupational level in adulthood could partly be attributed to disparities in mental health between Turkish migrants and Dutch natives in adolescence. Prevention of ethnic disparities in mental health at young age may therefore also contribute to the prevention of occupational differences in adulthood.     

Progress in neuroprotection in Parkinson's disease

Slowing or aborting the progress of neurodegeneration in Parkinson's disease (PD) remains the most important unmet need of this disorder. There are several recent developments in trial design and also in drugs under investigation for possible neuroprotective effect. Emphasis has been placed on clinical as opposed to imaging end-points and these include change in a clinical rating scale, e.g. United Parkinson's disease Rating Scale (UPDRS), or time to additional therapy. The introduction of the delayed-start, or wash-in, trial design adds an additional dimension to drug evaluation for neuroprotection. Compounds that have been recently tested in clinical trial include the monoamine oxidase-B inhibitor rasagiline, the anti-apoptotic agents TCH346 and CEP1347, and the promitochondrial agent creatine. The dopamine agonists have been evaluated for a neuroprotective effect using imaging end-points. Perhaps the most important and simplest concept for neuroprotection has been the theory that early dopaminergic support for the degenerating dopaminergic system per se provides significant long-term clinical benefit for PD patients.     

Changes in parasympathetic system in medulla oblongata in male pigs in the course of tachycardia-induced cardiomyopathy

Background

Autonomic imbalance constituting a fundamental feature of heart failure (HF) has been assessed mainly at the periphery. Changes in the functioning of autonomic centers in the brain remain unclear. We investigated the molecular elements of parasympathetic system, i.e. α7 nicotinic acetylcholine receptor (α7nAChR) and enzymes metabolizing acetylcholine (acetylcholinesterase, AChE, choline acetyltransferase, ChAT) in medulla oblongata (MO) of male pigs with chronic tachycardia-induced cardiomyopathy.

Methods

The mRNA levels of AChE, ChAT, α7nAChR and X-box binding protein 1 (spliced form, XBP1s) in MO were analyzed using qPCR, AChE and ChAT activities using spectrophotometry, proteasome activity using fluorometry, and the protein level of α7nAChR using Western blotting.

Results

The development of systolic HF was accompanied by an increase in circulating catecholamines, a decrease in the AChE and α7nAChR mRNA in MO, an increase in AChE activity (all p < 0.05), and no change in either the mRNA or activity of ChAT. Both circulating catecholamine levels and AChE activity were inversely related to systolic function of left myocardial ventricle (p < 0.05). The level of α7nAChR protein in MO and its cytoplasmatic fraction were higher in pigs with moderate and severe HF as compared to the other animals (p < 0.01). There was no difference in proteasome activity in MO between diseased and healthy animals, whereas the XBP1s mRNA decreased during HF progression (p < 0.05).

Conclusions

Molecular elements of parasympathetic system are changed within the medulla oblongata during the progression of systolic non-ischemic heart failure in male pigs, indicating a functional link between MO and heart in HF.