甲状旁腺功能亢进性肌病二例报道

1临床资料病例1，男，45岁，因进行性四肢无力2年于2006年10月31日入院。2004年11月开始出现腰背部酸痛和上下楼梯及下蹲起立时双下肢无力，并有足跟及足背部针刺感。2个月后出现双上肢无力，同时双下肢无力加重，上下楼梯需手扶，2006年8月上下楼梯困难，丧失劳动力，近半年尚感咀嚼力弱。近1年来小便量增多，夜间明显。既往健康，无类似疾病家族史。体格检查：神志清楚，语言清晰，双侧咀嚼肌肌力轻度减退，余颅神经检查（－），双上肢肩胛带、近端肌肉及双下肢近端肌肉轻度萎缩，四肢肌张力正常，双上肢近端肌力4级、远端4+级,左下肢近端肌力4-级、右下肢近端3级、双下肢远端肌力4+级，行走时呈鸭步。四肢有短手套袜套样痛触觉减退，深感觉正常。双上肢腱反射、双侧膝腱反射（），双侧踝反射未引出。病理反射阴性。肌电图检查示：双侧股直肌、三角肌见短棘波多相电位，四肢运动感觉神经传导速度减慢。入院后查血尿粪常规、肝肾功能正常，CK、LDH均正常，碱性磷酸酶(AKP) 1 113 U/L(参考值 32~92 U/L)，血叶酸、B12水平正常，免疫指标全套、肿瘤标志物均正常，尿本周蛋白阴性，甲状腺功能正常，甲状腺球蛋白抗体（TGAb）42.2％（参考值<30％），抗甲状腺微粒体抗体（TMAb）34.5％（参考值<15％）。血清钙 2.73 mmol/L（参考值2.15~2.6 mmol/L）、磷 0.79 mmol/L（参考值0.82~1.6 mmol/L），PTH 1 854 pg/ml(参考值15~16 pg/ml）。脑脊液检查正常。心电图正常。双手正位片示：手掌指骨骨皮质变薄、毛糙，部分皮质下见小囊状透光区，骨小梁显示清晰，各小关节正常，提示符合甲旁亢性骨改变。左足跟骨密度检测示骨密度下降。肌肉活检示：肌纤维萎缩，肌横纹及纵纹均存在，肌核边集，数目减少，肌间隙增宽(图1)。B超示：双侧甲状旁腺实性结节，考虑腺瘤可能；右侧甲状腺结节伴钙化，倾向髓样癌；双侧肾上腺实性结节，考虑腺瘤可能。CT及ECT检查考虑为多发性内分泌腺瘤。诊断为：（1）甲状旁腺功能亢进性神经肌肉病；（2）多发性内分泌瘤病Ⅱ型。2006年11月30日患者在本院外科行右甲状腺癌清扫加双侧甲状旁腺瘤切除术，术后病理：（1）(左下、右下)甲状旁腺腺瘤；（2）(右上甲状腺)髓样癌。术后1周内出现低钙性搐愵，经补钙治疗后好转，肌无力症状明显好转，能单独上下楼梯。[第一段]     

以与血压升高无关的发作性头晕为表现的不典型原发性醛固酮增多症5例分析

目的 总结以发作性头晕为表现的与血压升高无相关性的不典型原发性醛固酮增多症的神经系统表现、诊治及预后。 方法 对2016-2019年我科确诊的5例以发作性头晕为表现的不典型原发性醛固酮增多症患者的临床资料进行回顾性分析。 结果 5例患者中男性1例,女性4例,平均年龄65.4±16.1岁,均以反复头晕起病,病程3.34±2.78月,发作频次1次/日至1次/月不等,每次持续数秒至30min,可伴头痛、视物旋转、恶心、耳鸣、乏力、多汗、心悸等,面色可发红或自觉头面部发热感,体位变化或活动时明显,发作间歇期无不适。2例既往诊断高血压,3例入院后评估24小时动态血压发现血压波动。5例入院血钾均在正常范围内,2例发作头晕时测血钾降低。皮质醇及甲状腺功能等激素水平正常范围内。肾上腺CT（增强）检查仅1例提示左侧肾上腺增生,余4例未见明显异常。临床定期随访头晕明显好转。 结论 以反复发作性头晕为主要表现与血压升高无相关性的不典型原发性醛固酮增多症少见,需提高认识。要仔细了解高血压病史,并进行醛固酮测定,以避免误诊和漏诊。低钾血症由于其敏感性和特异性较低,已不能作为筛查PA的良好指标。     

新生大鼠大脑皮质神经干细胞的分离培养与胆碱能神经元的鉴定

目的:体外分离和培养新生大鼠大脑皮质神经干细胞并进行鉴定,为用于脑梗死动物模型梗死区移植细胞作准备.方法:分离新生大鼠皮质神经干细胞,进行体外培养,使用免疫细胞荧光染色技术对细胞的特性进行鉴定,并对细胞的胆碱能特征进行鉴定.结果:获得了Nestin阳性的神经干细胞,其分化后可获得MAP-2、GFAP和 CNPase阳性的神经元、星形胶质细胞和寡突胶质细胞;通过胆碱能鉴定发现大脑皮质干细胞可分化为胆碱能神经元. 结论:体外分离和培养的大脑皮质神经干细胞具有增殖分化的能力,并可以分化为胆碱能神经元,有望应用于皮质损伤后认知障碍及运动障碍的细胞移植治疗.     

EFFECT OF RADIX SALVIAE MILTIORRHIZAE ON EXTRACELLULAR ADENOSINE AND EVALUATION OF ITS PROTECTIVE EFFICACY IN ISCHEMIC REPERFUSION RAT——Microdialysis, HPLC and Histopathologic Studies

The effects of Radix Salviae Miltiorrhizae (RSM) on extracellular adenosine (Ade) and its metabolites, Le. inosine, hypoxanthine and xanthine, were studied with microdialysis and HPLC techniques during cerebral ischemia-reperfusion induced by 4-vessel occlusion in rat brain. Histological examination of hippocampus was performed 6h after reperfusion. ECF (extracellular fluid) adenosine and its metabolites were compared, between the controls (n= 6) and RSM-treated rats (n= 6). Basal level of Ade and its metabolites release were not greatly affected by pretreatment with RSM, and no significant difference as compared with the sham-operated (n= 6). Ade and its metabolites were dramatically increased after ischemia, and decreased near basal-level and its metabolites remained high at the end of reperfusion. In the RSM-treated animals, the tendency of changes of Ade and its metabolites was just the same as in the controls, but the magnitudes of changes were significantly lower at some different tune points. In sham-operated animals, no changes were observed at different time points both during ischemia (30 min.) and reperfusion (60 min.). Histopathological findings demonstrated that RSM pretreatment results in better histologic preservation of the pyramidal cells in the postischemic reperfusion CA1 sector both qualitatively and quantitatively. These results indicated that RSM protects against cerebral ischemia reperfusion injury.     

EFFECT OF RADIX SALVIAE MILTIORRHIZAE ON NITRIC OXIDE IN CEREBRAL ISCHEMIC-REPERFUSION INJURY

It has been postulated that nitric oxide (NO) is produced in significant amount on reperfusion of ischemic brain and could cause brain edema and cell death. This study was undertaken in an attempt to determine whether Radix Salviae Miltiorrhizae (RSM) could reduce cerebral NO contents in 4 -vessel occlusion rat model after 30 min of global ischemia and 15 min of reperfusion. There was a significant increase in cerebral NO level in untreated animals as compared with sham-operated ones. By pretreatment with RSM the cerebral NO concentration was reduced to normal level. The possibilities of neuroprotective role of RSM in cerebral ischemic-reperfusion injury were discussed.     

INHIBITORY EFFECT OF 764-3 ON Ca~2 UPTAKE IN RAT BRAIN SYNAPTOSOMES

In an attempt to examine the effect of 764-3, a purified component extracted from Radix Salviae Miltiorrhizae (RSM), on calcium channel, Ca2 uptake in rat brain synaptosome stimulated by high-K and Glutamate (Glu) was investigated by using radioisotope labeled 45CaCl2. It was found that 764-3 could decrease the uptake of Ca2 induced by high-K (60 mM) and 0.1 mM glutamate in a concentration-dependent manner. The maximum inhibiting rates were 45.22± 4.88% and 37.58±4.52%, respectively. The results indicated that neuroprotective effect of RSM on cerebral ischemic injury may be related to its blocking effects on calcium channel.     

PROTECTIVE EFFECT OF RADIX SALVIAE MILTIORRHIZAE COMPOSITA ON CEREBRAL ISCHEMIA

The protective effects of Radix Salviae Miltiorrhizae Composita (RSMC) on cerebral ischemia in gerbils produced by unilateral ligation of left common carotid artery and on the development of ischemic cerebral edema were studied. It was found that the administration of RSMC resulted in a very significant decrease in neurological deficits and the time for ex-hibiting first signs of neurological deficit delayed compared with untreated animals. Al-though the values of specific gravity of fresh cerebral tissue on the ligated side of common carotid artery in untreated animals (1.0406± 0.0039 SD) and in RSMC-treated animals (1.0453 ± 0.0009) were decreased significantly compared to that (1.0468± 0.0003) of sham-operated animals, the specific gravity of cerebral tissue in RSMC-treated animals was significantly heavier than that in untreated animals (t= 5.5022, P< 0.001), i.e. the ad-ministration of RSMC decreased the severity of cerebral edema. A significant negative cor-relation was found between stroke index and specific gravity, and it suggests that one of the protective mechanisms of RSMC might be related to its ameliorating cerebral edema.     

环孢霉素的神经系统毒性

环孢霉素作为新型的免疫抑制剂,对神经系统的毒性具多样性。其不良反应可以累及中枢神经系统与周围神经系统。常见的有后部白质脑病、癫痫、头痛、震颤、锥体系与锥体外系反应、精神症状等,其中最严重的为后部白质脑病。症状具有可逆性,可在停药或减药后消失。神经影像学与神经电生理可有相应异常表现,在症状消失后可恢复,病理及实验室检查无特异性。神经毒性的发生机理可能与共投药、高血压、低镁血症、低胆固醇血症、内皮素、交感神经作用等机制有关。     

脑梗死患者血清白介素-6、白介素-8、肿瘤坏死因子-α及白介素-1β水平的研究

目的研究脑梗死患者急性期血清 IL -6、IL-8、TNF-α、IL -1β水平的变化 ,特别是 IL -6水平与脑梗死面积及神经功能缺损的关系。方法采用双抗体夹心 ELISA法检测 3 4例脑梗死急性期患者不同时间内血清 IL-6、IL -8、TNF-α、IL -1β水平 ,并随机抽取 10例健康人进行对照。结果脑梗死患者血清 IL -6水平在发病 6小时内明显升高 ,2 4～ 3 6小时达高峰 ,7天后基本降至正常 ,大面积梗死组IL-6水平明显高于小面积梗死组 ( 6小时内 ,P <0 .0 5 ;2 4～ 3 6小时 ,P <0 .0 5 ) ,且血清 IL -6水平与神经功能评分呈正相关( r=0 .87,P<0 .0 1) ,七叶皂甙钠治疗 3天后血清 IL-6水平下降明显 ( P<0 .0 1) ,脑梗死患者急性期血清 IL-8水平亦升高( P<0 .0 5 ) ,TNF-α、IL-1β水平则无明显变化。结论急性脑梗死患者血清 IL-6水平升高 ,而 IL-6升高的水平反映应激反应程度 ,与梗死面积及神经功能缺损程度有一定相关性。     

姜黄素降血脂及抗动脉粥样硬化作用的实验研究

目的:观察姜黄素对动物血脂水平及主动脉粥样硬化斑块形成的影响。方法:选用大鼠和家兔进食高脂饮食造成的高脂血症模型,连续监测给药前及给药后大鼠和家兔血清总胆固醇(TC)、甘油三酯(TG)、高密度脂蛋白胆固醇(HDL-C)、低密度脂蛋白胆固醇(LDL-C)的含量。实验结束时处死家兔,观察主动脉粥样斑块的形成情况。结果:姜黄素组大鼠血清 TC 及 LDL-C 含量的升高程度明显低于模型组(P<0.05,P<0.01)。姜黄素组家兔血清 TC、TG 的含量亦较模型组为低(P<0.05,P<0.01),主动脉粥样斑块的形成程度亦较模型组明显减轻(P<0.01)。结论:姜黄素具有降低高脂血症大鼠血清 TC 及 LDL-C 的作用,对家兔动脉粥样硬化的形成亦有抑制作用。