伴有皮层下梗死和白质脑病的常染色体显性遗传性脑动脉病的一个家系研究报告

目的 探讨伴有皮层下梗死和白质脑病的常染色体显性遗传性脑动脉病(CADASIL)患者的临床特点、病理改变及基因改变.方法 对1例CADASIL先证者及其家族共16例的遗传史、临床表现、影像学特点、病理改变及基因改变等方面进行系统研究.结果 临床表现为缺血性脑卒中发作、偏头痛、精神异常、痴呆,均无高血压及动脉硬化证...     

The effects of bromvincamine and vincamine on regional cerebral blood flow and mental functions in patients with multi-infarct dementia

The effects of vincamine and bromvincamine (BV 26-723) on mental functions and regional cerebral blood flow (rCBF) measured by the ¹³³Xe inhalation method, were investigated in ten patients with mild to severe symptoms of multi-infarct dementia. The double blind cross over design included three treatment periods, each of 2 weeks' duration. The patients were drug free at the time of the first investigation in each treatment period. Then placebo, vincamine or bromvincamine was given orally (4×20 mg/day). Psychiatric evaluation, psychometric assessment and measurement of rCBF were done at start of medication and after 1 and 2 weeks during medication. There were 2 drug-free weeks between the first and second, and second and third treatment periods. Results from treatment with vincamine showed a significant increase in the global CBF level and reduction of initial right-left asymmetry of hemispheric means. No effects were seen in regional flow patterns. Performance score on a verbal memory test increased significantly. No significant effect on the global rCBF level was indicated for bromvincamine. However, the number of ischemic regions decreased significantly during treatment. Performance on two memory tests improved significantly. No significant changes in overall psychiatric ratings were observed for any of the treatment periods.     

多发性脑梗死性痴呆死亡病例分析

目的 :了解多发性脑梗死性痴呆 (MID)死亡的危险因素。　方法 :设计统一的调查表 ,对 6 2例 MID死亡病例进行调查分析。　结果 :平均生存时间为 (4 .6± 3.4)年 ,死亡原因依次为肺部感染、全身衰竭、脑血管意外和心血管疾病。 L ogistic回归分析表明 ,发病年龄、烟酒嗜好、有无配偶等为影响 MID死亡的主要危险因素。　结论 :影响 MID病人死亡的因素是多方面的 ,死亡原因以躯体并发症为主 ,在临床上应给予重视。     

伴有皮层下梗死和白质脑病的常染色体显性遗传性脑动脉病(附一家系临床及治疗观察)

目的:探讨伴有皮层下梗死和白质脑病的常染色体显性遗传性脑动脉病(CADASIL)患者的临床特征、影像学特点及基因诊断.方法:调查先证者及其家族的发病情况及遗传方式,对其临床表现、影像学特征、分子遗传学及治疗等方面进行研究.结果:患者均有记忆力减退、乏力、脑卒中样发作等特点,头颅CT、MRI检查可见多发性梗死灶、脑白质变性,有家族聚集性,没有高血压、动脉硬化,符合CADASIL的临床诊断.基因测序显示 Notch3 基因第3、4 号外显子突变.全家系5代已有4代14人呈临床或亚临床发病,符合常染色体显性遗传.患者经活血化瘀、改善微循环、保护脑细胞治疗,症状略有缓解.结论:该家系的临床、影像及分子遗传学特征符合CADASIL诊断标准.本病病理特点目前尚无法改变,但积极地改善脑供血、保护脑细胞治疗似可改善肢体肌力,改善生活质量.     

多发性脑梗塞痴呆大鼠多脑区精氨酸加压素水平的测定

通过对Wistar大鼠颈内动脉注入血凝块栓子．制作多发性脑梗塞模型，模拟人类多发性梗塞痴呆的病理损害，研究不同脑区AVP含量的变化。结果表明模型组与对照组相比，在水迷宫中游全程的时间显著延长，正确次数明显减少（P＜0．01）。采用放射免疫测定法测定额叶、顶叶、海马、纹状体四个脑区内AVP含量，模型组比对照组含量显著降低（54％～73％，P＜0．01）。实验结果揭示：这种多发生梗塞模型是成功的，大鼠的学习、记忆障碍可能与重要脑区使塞后AVP含量下降有关。     

综合康复治疗多梗死性痴呆的疗效观察

目的 观察应用不同治疗方案对多梗死性痴呆患者认知功能和日常生活功能的疗效.方法 将60例患者随机分为联合治疗组、醒脑静组和常规治疗组.常规治疗组予甲钴胺胶囊口服,环磷腺苷注射液静滴;醒脑静组在常规治疗基础上加醒脑静注射液;联合治疗组在醒脑静组治疗基础上加针刺治疗.观察三种治疗方法对患者MMSE评定和ADL评定的效果.结果 三组患者治疗后的MMSE评分和ADL评分均比治疗前提高,联合治疗组MMSE评分比常规组MMSE评分显著提高,差异有统计学意义（P〈0.05）.结论 针刺配合醒脑静注射液治疗多梗死性痴呆可显著提高患者的认知功能.     

Alzheimer氏病与多灶脑梗塞性痴呆的临床对照分析

对５１例ＡＤ及５０例ＭＩＤ进行了临床对照分析。结果显示ＭＩＤ９４．０％（４７／５０）有精神症状，而ＡＤ８０．４％（４１／５１）有精神症状，两组病人在发病年龄、性别、家族史、病程及住院时间相同的情况下ＭＩＤ的痴呆程度轻于ＡＤ。经治疗ＭＩＤ痴呆的好转率大于ＡＤ（Ｎ＜０．０１），精神症状则有不同程度减轻（Ｐ＞０．０５）。     

阿尔采末氏病与多发脑梗塞性痴呆病人认知功能的比较研究

目的：探讨阿尔采末氏病（ＡＤ）和多发脑梗塞性痴呆（ＭＩＤ）病人认知功能的异同。方法：用韦氏成人智力量表和韦氏记忆量表对２１例ＡＤ和１５例ＭＩＤ病人的认知功能进行测查。结果：韦氏智力量表的分析结果表明，ＡＤ组在知识、领悟、算术、相似性、数字广度、词汇和图片排列的量表分以及言语ＩＱ和总ＩＱ显著低于ＭＩＤ组，其余指标差异不显著。ＡＤ组累加、视觉再生、定向、再认、背数的得分以及总量表分和记忆商均明显低于ＭＩＤ组。结论：ＡＤ组和ＭＩＤ组均表现出明显的智能和记忆障碍，ＡＤ组的受损更为严重，以言语理解和记忆注意受损为突出特点，ＭＩＤ组则相对受损较轻，并以操作能力受损为突出特点。     

The nucleus basalis of Meynert in multi-infarct (vascular) dementia

Summary The number and nucleolar volume of nerve cells within the nucleus basalis of Meynert were estimated in 10 patients with Alzheimer's disease, 12 with multi-infarct dementia, 9 with a mixed Alzheimer/multi-infarct dementia and in 10 age-matched controls. As reported previously in Alzheimer's disease, both the number and nucleolar volume of surviving cells was reduced, whereas in multi-infarct dementia no significant change in either measure was noted. In patients with Alzheimer's disease/multi-infarct dementia the loss of nerve cells and reduction in nucleolar volume varied greatly in severity from patient to patient according to the relative balance of Alzheimer and vascular type pathological changes present within each patient.Supported in part by a grant from the North Western Regional Health Authority to one of us (BM)     

Cerebrospinal fluid investigations in multi-infarct dementia and senile dementia

Thirty-eight demented patients were clinically investigated and classified into a multi-infarct dementia (MID) and senile dementia (SD) group. Total protein, albumin, immunoglobulin G, agar gel electrophoretic protein and iso-electric focusing pattern were determined in CSF and serum as well as the lactate dehydrogenase, LD-iso-enzyme pattern and uric acid. No significant differences were found regarding total protein, albumin and immunoglobulin G. The agar gel electrophoresis showed a tau-globulin increase in 18% of the SD-group against 6% of the MID group. The patterns were, however, plasma-like in 56% of the MID group against 23% of the SD group. The iso-electric focusing pattern showed an abnormal band in some SD-patients which was not found in the MID group. The uric acid and LD-enzyme concentration was equal in both CSF and serum. An index was calculated to give an approximate correction for the influence of different permeability of the blood-brain and CSF barrier. A significantly higher LD-index was found in the SD-group, suggesting a continuous cell degeneration.