低钾型周期性麻痹50例临床分析

目的探讨低钾型周期性麻痹的临床特点和治疗方法及疗效。方法回顾性分析本院收治的低钾型周期性麻痹50例患者的临床资料。结果 50例患者中,大部分病例有较明显诱因,原发性36例,其中有家族史者6例,继发于甲亢者14例。经过口服和静脉注射钾后,全部病例均痊愈出院。结论血清钾及心电图的检查有利于早期诊断及治疗低钾型周期性麻痹。避免各种诱因是防止复发的关键,继发性低钾型周期性麻痹患者要加强原发病治疗。     

低钾型周期性瘫痪患者血钾浓度与肌力、心电图及肌酶水平的关系

目的 探讨低钾型周期性瘫痪(HoPP)患者血钾浓度与肌力、心电图及肌酶水平的关系.方法 对126例HoPP患者进行血钾浓度、肌力、心电图及肌酶水平检测并分析.结果 126例HoPP患者中,轻度低钾组15例,中度低钾组36例,重度低钾组75例;3组肌力梯度下降(P<0.05).HoPP患者入院时心电图检查113例有异常,补钾治疗后复查心电图明显好转(P<0.05).3组患者血清肌酶水平均有不同程度增高,每两组间的差异统计学意义(均P<0.05);血钾浓度与肌酸激酶水平呈负相关(r=-0.672,P<0.01).结论 HoPP的血钾浓度与肌力、肌酶水平、心电图改变密切相关,血钾、肌力、肌酶及心电图检查对HoPP的诊断、治疗及预后具有重要意义.     

重症低钾性周期性麻痹的救治(附10例临床分析)

低钾性周期性麻痹为临床常见病，多数预后较好，但重症低钾性周期性麻痹若救治不及时，常可因为呼吸肌麻痹或严重心律失常而死亡。本文就１０例重症低钾性周期性麻痹的救治体会总结报道如下： 临床资料：１０例均为男性，年龄在１５～５６岁，均为首次发作。起病至就诊时间在２～４２小时。发病诱因，过度劳累２例，上感２例，出大汗２例，饱餐后１例，输注葡萄糖液后３例。除３例发生于白天外，其余７例均发生于夜间睡眠中或凌晨醒后。 临床表现：均急性起病，自下而上，由近端到远端发展的四肢对称性驰缓性瘫痪为主要表现。多数下肢重于上…     

周期性麻痹56例临床分析

本文总结了我院1991年至1997年以周期性麻痹入院的56例患者。结果发现：①周期性麻痹以散发多见,男性发病明显多于女性,男：女为13：1,以青年组最多,占69％;②继发性周期性麻痹中甲亢占73／;③低血钾可以出现心脏受抑制表现;④肢体瘫痪程度与血钾下降程度无平行关系;⑤低血钾可影响平滑肌;⑥周期性麻痹可伴血象升高。     

散发性低钾性周期性麻痹KCNJ18 KCNJ12基因分析

目的通过对散发性低钾性周期性麻痹KCNJ12、KCNJ18基因测序分析,探讨基因突变的相关性。方法取我院住院的52例散发性低钾性周期性麻痹患者和10例健康对照者血样,应用PCR和测序技术,进行KCNJ12、KCNJ18基因编码区PCR、测序比对。结果通过对52例患者和10例健康对照者KCNJ12、KCNJ18基因扩增测序比对,均未发现明显异常。结论未发现散发性低钾性周期性麻痹患者KCNJ12、KCNJ18基因编码区序列存在突变。     

低钾性周期性麻痹的血清肌酶学改变

低钾性周期性麻痹(hypokalemic periodic paralysis, HoPP)是以反复发作的骨骼肌弛缓性瘫痪为特征的一种疾病,发作时伴有血钾降低,有研究证实,本病与染色体1q31-32连锁DHPR的als亚单位突变引起的骨骼肌钙通道异常有关,在我国以散发者多见。以往对本病发生后血清肌酶的研究不多,认为血清肌酶升高是肌炎的重要特征,在HoPP不会出现血清肌酶的改变。为探讨两者之间的关系,我们1999-2003年对我科住院的38例HoPP病人进行血清酶学检查发现32 例病人血清肌酶升高,现报道如下。     

低钾型周期性麻痹36例临床分析

低钾型周期性麻痹是临床上常见的表现为四肢突发无力的疾病之一,发病突然,容易误诊,且治疗风险大。为提高对本病的全面认识,现将我科5a来收治的36例患者的临床资料分析如下。1资料和方法1.1一般资料本组36例患者中男33例,女3例;年龄17-42岁,平均28岁;首次发病20例,反复发病16例;发病诱因：剧烈运动并大汗10例,感冒8例,饱餐6例,腹泻5例,饮酒4例,无明显诱因者3例。     

周期性麻痹50例临床分析

周期性麻痹是神经科常见疾病之一,现将我院自1978年—1987年共收治了成年人周期性麻痹50例分析如下: 临床资料性别:男44例占88％,女6例占12％。年龄,最小16岁,最大56岁。其中22岁以下4例占8％,21—50岁43例占86％,50岁以上3例占6％,发病诱因:着凉感冒后发病13例占26％,早晨起床或安静时发病11例占22％,劳累或活动中发病8例占16％,腹泻后发病1例占2％,饱餐后发病2例占     

低钾型周期性瘫痪与高血糖的关系

目的 探讨低钾型周期性瘫痪病因和发病机制。方法 对2002-01-2005-04确诊的15例低钾型周期性瘫痪患者入院时予血糖检测，并在血钾正常后检测空腹血糖，与同期入院无糖尿病患者15例血糖进行对比。结果 15例低钾型周期性瘫痪患者入院时血糖均高于正常水平，并同对照组入院时血糖有显著性差异。血钾正常后血糖恢复正常，与对照组相比无显著性差异。结论 低钾型周期性瘫痪患者在发病时血糖增高，血钾正常后血糖恢复正常，血糖增高可能与低钾型周期性瘫痪发病机制有关。     

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。     

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     

Effects of prevention of afferentation of the development of the chick optic lobe

BONDY, S. C., M. E. HARRINGTON AND C. L. ANDERSON. Effects of prevention of afferentation on the developmentof the chick optic lobe. BRAIN RES. BULL. 3(5) 411–413, 1978.—The effects of unilateral extirpation of the right optic cup of the three-day incubated chick embryo upon the rate of synthesis and the stability of DNA in the non-innervated optic lobe, have been studied. This surgical procedure prevents innervation of the optic lobe contralateral to the removed eye, while the other optic lobe is normally innervated by retinal ganglion cells of the remaining eye. At the 20th day of incubation, the DNA content of the non-innervated lobe was below that of the paired lobe receiving normal innervation. This deficiency of cell number was caused by two events; death of an excess number of neurons formed early in embryogenesis and a reduced rate of glial proliferation in the later stages of incubation.     

帕金森病运动症状进展分析

目的分析帕金森病(PD)患者运动症状进展特点。方法采用PD统一评分量表(UPDRS)Ⅲ对912例PD患者进行评估。结果与病程1年的患者比较,除病程1~2年的患者外,其他病程患者的UPDRSⅢ评分、强直分、姿势或步态异常分、轴性症状总分、言语分、步态分显著升高(均P0.05),病程5~6年及14年患者的震颤分,病程5~6年、7~8年、9~13年、14年患者的运动迟缓分、姿势分显著升高(P0.05~0.01)。轴性症状进展速度高于UPDRSⅢ评分。结论 PD患者病程早期UPDRSⅢ评分进展快,震颤症状进展独立于其他症状,轴性症状评分较UPDRSⅢ更敏感地反映疾病加重趋势。     

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.     

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.     

广西农村壮族妇女精神分裂症患者生活质量状况调查

目的研究农村壮族妇女精神分裂症患者的生活质量及影响因素。方法前瞻性的队列研究。采用随机分层抽样法分为农村壮族妇女精神分裂症组、农村汉族妇女精神分裂症组、农村正常妇女对照组,应用“世界卫生组织生存质量测定报告”(WHOQOL-100)及PANSS量表调查其生活质量和疾病的严重程度。结果农村壮族妇女精神分裂症患者生活质量明显低于农村汉族妇女精神分裂症患者,影响其生活质量的相关因素是生活环境及精神支柱/个人信仰。结论经济贫困、环境条件、缺乏有效的医疗服务和社会保障是农村壮族妇女精神分裂症患者生活质量低的关键。因此,建立农村壮族社区精神卫生服务网络势在必行。     

Impact of our understanding of the genetic aetiology of epilepsy

A genetic contribution to aetiology is estimated to be present in up to 40% of patients with epilepsy. It is useful to categorise genetic epilepsies according to the mechanisms of inheritance into Mendelian disorders, non-mendelian or ‘complex’ disorders, and chromosomal disorders. Over 200 Mendelian diseases include epilepsy as part of the phenotype, and the genes for a number of these have been identified recently. These include autosomal recessive progressive myoclonic epilepsies such as Unverricht-Lundborg disease, Lafora disease and the neuronal ceroid lipofuscinoses, and three autosomal dominant idiopathic epilepsies. The last named have been shown to arise from mutations in ion channel genes. Autosomal dominant nocturnal frontal lobe epilepsy is caused by mutations in CHRNA4, benign familial neonatal convulsions by mutations in KCNQ2 and KCNQ3, and generalised epilepsy with febrile seizures plus by mutations in SCN1B. ‘Complex’, familial epilepsies are more difficult to analyse, but evidence has been obtained for loci predisposing to juvenile myoclonic epilepsy on chromosome 6p and 15q. Lastly, the genes underlying several spike-wave epilepsies in mice have been cloned, and three of these encode sub-units of voltage-gated calcium channels. Received: 29 September 1999/Accepted: 7 December 1999     

他汀类药物的使用和颅内动脉瘤破裂相关性分析

目的 探讨他汀类药物对颅内动脉瘤破裂的影响。方法 2010年3月至2014年3月收治颅内囊状动脉瘤67例,其中破裂者32例,未破裂者35例。采用多变量Logistic回归评估他汀类药物的使用和颅内动脉瘤破裂的关系。结果 破裂组术前使用他汀类药物4例（12.5%,4/32）,未破裂组16例（45.7%,16/35）。破裂组服用他汀类药物的百分比显著低于未破裂组（P<0.01）。纠正潜在的混杂干扰后（or值: 0.30,95%可信空间:0.12~="" 0.64）显示,颅内动脉瘤破裂与他汀类药物的使用呈显著负相关,也与高血清总胆固醇浓度有关。结论 本结果提示他汀类药物对颅内动脉瘤破裂有一定的预防效果。     

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。