可逆性后部白质脑病综合征临床及影像学特点分析

目的 探讨可逆性后部白质脑病综合征(RPLS)的临床和影像学特点.方法 回顾性分析9例RPLS患者的临床及影像学资料.结果 9例患者临床表现以头痛、意识障碍、癫发作、视觉异常为主;头颅MRI检查有大脑半球后部白质为主的T1WI低信号,T2WI、Fair像呈高信号的病灶,且大多双侧对称.结论 头痛、意识及精神障碍、癫发作、视觉异常、是可逆性后部白质脑病综合征主要临床表现,影像学特征主要为大脑后部对称性、可逆性脑白质损害.     

小鼠羊水来源干细胞原代培养及向神经元的诱导分化

目的 研究小鼠羊水来源干细胞(mAFSC)的原代培养及向神经元的诱导分化. 方法 机械手段抽取孕中期小鼠羊水,体外培养扩增,观察细胞生长特性;流式细胞仪检测mAPSC表面抗原表达;锥虫蓝检测P3、P8、P13代mAFSC细胞活性.体外用神经元诱导液诱导mAFSC分化为神经元,并用免疫荧光检测神经元特异性表面标志β-tubulin. 结果 原代mAFSC培养7～10 d可以贴壁生长至80%以上,细胞数达3.5×105～4.0×105个.经3次以上传代以后,mAFSC呈现较均一的梭形细胞,并呈栅栏样排列.mAFSC在体外活性及增殖能力较强,经90 d以上的体外培养,P17代的细胞才出现细胞形态变化及增殖能力明显下降.流式细胞仪检测结果显示,表面抗原Sca-1、CD29阳性;CD11b、CD34及CD45为阴性.mAFSC经神经元诱导液诱导3 d之后,可于体外形成神经样细胞,同时部分细胞表达β-tubulin. 结论 mAFSC在体外具有很好的活性及增殖能力,且拥有定向分化的潜能,为今后的小鼠动物实验细胞治疗研究提供了一种新的干细胞.     

年龄和康复锻炼对Duchenne型肌营养不良患者肺功能的影响

康复锻炼干预对Duchenne型肌营养不良(muscular clystro-phy,DMD)患者的肺功能变化是否有正面的积极作用一直未受到足够的重视和关注,有文献报告,12岁是DMD患者肺活量变化的分界线,12岁以前肺活量随年龄增长而增加,13岁以后迅速下降[1]。本文拟通过65例DMD患者肺通气功能测定结     

面肩肱型肌营养不良症71例的临床和遗传特点

面肩肱型肌营养不良症（ｆａｃｉｏｓｃａｐｕ－ｌｏｈｕｍｅｒａｌｍｕｓｃｕｌａｒｄｙｓｔｒ－ｏｐｈｙ，ＦＳＨＤ），由Ｌａｎｄｏｕｚｙ和Ｄｅｊｅｒｉｎｅ于１８８４年首先报道，临床以选择性侵犯面肌、肩带肌及上臂肌群为特征，国内报道较少，我院１９８０～１９９７年共收治７１例ＦＳＨＤ患者，现就其临床资料结合有关文献报告如下。１　临床资料１．１　一般资料　患者７１例，男５４例，女１７例，男∶女＝３∶１。发病年龄１～５３岁，平均１６．７岁；其中１～１０岁８例，１１～２０岁５２例，２１～３０岁８例，３１～４０岁…     

遗传性运动感觉性周围神经病Ⅰ型的临床及电生理特点

目的研究遗传性运动感觉性周围神经病I型(CMT1型)患者的临床特征、肌电生理特点及二者的相关性。方法观察临床确诊的36例CMT1型患者的临床特征,检测双侧上肢远端及下肢常规肌电图及周围神经传导速度及波幅改变情况,并探讨CMT1的肌无力、萎缩程度与胫神经、正中神经及尺神经CMAPs及MCV的关系。结果36例临床确诊的CMT1型患者,男性多于女性,均在青少年期起病,主要临床症状为进行性双下肢或四肢对称性远端肌无力和/或肌萎缩,本组患者症状局限于双下肢者20例(55.56%)。肌电图检查示所有患者均表现为神经源性损害,胫神经、腓神经、正中神经和尺神经均有不同程度的传导速度下降甚至不能引出,波幅降低。随起病年龄增大、病程延长,正中神经、尺神经及胫神经的MCV有下降的趋势并可能测不出(r=0.39,P0.05),CMAPs也出现类似的变化趋势(r=0.28,P0.05)。且肌肉萎缩、无力程度与CMAPs正相关而与MCV无相关关系。结论CMT1多为青少年起病,以慢性进行性双下肢或四肢远端肌无力和肌萎缩为主要特征,肌电图和周围神经传导速度是诊断CMT1的可靠方法,并能预测疾病的进展程度。     

肌萎缩侧索硬化患者轻度认知功能损害

Objective To explore the cognitive status of amyotrophic lateral sclerosis (ALS) patients, and to explore the involved cognitive domains, subtypes and risk factors of mild cognitive impairment in ALS ( ALS-MCI).Methods Twenty-nine cases of ALS and 58 healthy volunteers were included.The severity of the bulbar and spinal functions of the patients was evaluated by the Improved Norris Scale.According to the Diagnostic and Statistical Manual of Mental Disorders 4th Edition-Revised( DSM-Ⅳ-R) criteria of dementia, ALS cases were classified as demented and non-demented.For non-demented ALS cases, the common cognitive batteries evaluating mental state, verbal memory, executive, attentional and visuospatial abilities were performed.Hamilton Anxiety Scale ( HAMA) and Hamilton Depression Scale (HAMD) were evaluated too.They were further classified into ALS-cognitively normal (ALS-CogNL) and ALS-MCI groups according to Petersen criteria of MCI.Risk factors possibly correlated with ALS-MCI were analyzed by comparing the differences in age, age of onset, duration of the disease, sites of onset, symptoms of bulbar and limb function between ALS-CogNL and ALS-MCI groups.Results Among 29 ALS cases, 14 (48.3% ) cases with cognitively normal( ALS-CogNL), 15 cases (51.7% ) with ALS-MCI,and none with dementia were identified.Among 15 ALS-MCI cases, 12 cases with executive dysfunction, 8 cases with memory deficits,9 cases with attention impairment and none with visuospatial impairment were found.ALSMCI cases could be further classified into three subtypes; 1 case with amnestic MCI (aMCI) ,6 cases with single domain non-memory MCI ( sdMCI), and 8 cases with multiple domains slightly impaired MCI (mdMCI).Between ALS-MCI and ALS-CogNL groups, there were significant differences (t = -2.435,- 2.576, both P ＜ 0.05) in education ((8.7 ± 2.8) years vs (11.3 ± 3.0) years) and Improved Norrisscale (bulbar score: (28.4 ± 7.7) scores vs ( 34.0 ± 3.4) scores) , however, no significant differences in sex, age, age of onset, duration,site of onset,HAMA or HAMD scores,and Improved Norris scale( spinal score) were found.Conclusions Cognitive deficits commonly exist in ALS patients.For the involved domains, executive dysfunction is the most common, deficits of attention and memory are also common, and deficit in visuospatial function is not found.The most common subtype of ALS-MCI is mdMCI.Severe bulbar symptoms and lower education may be the risk factors of ALS-MCI.     

神经电生理检查判断眼肌型重症肌无力的敏感度分析

目的：分析眼肌型重症肌无力（OMG）患者的神经电生理特点,为临床诊断提供有价值的依据.方法：对42例临床诊断为OMG患者进行单纤维肌电图、重复神经电刺激和肌电图检测.结果：伸指总肌的单纤维肌电图34例异常,重复电刺激异常23例;肌电图示14例肌源性损害.结论：OMG患者单纤维肌电图是一种敏感度较高的检测方法,其次为重复神经电刺激,其肌肉检测阳性率高低依次为眼轮匝肌、肱二头肌及小指展肌.     

针刺联合耳穴贴压治疗抑郁症睡眠障碍疗效观察

目的:探讨针刺联合耳穴贴压治疗抑郁症睡眠障碍的临床疗效.方法:76例抑郁症睡眠障碍患者随机分为观察组(39例)和对照组(37例).观察组治疗采用针刺百会、上星、神庭、风池、神门等穴位,同时联合耳穴贴压;对照组口服盐酸氟西汀分散片(百优解),两组疗程均为4周.比较两组汉密尔顿抑郁量表(HAMD)以及匹兹堡睡眠质量指数(P...