经颅多普勒超声诊断大脑中动脉狭窄的价值

目的 探讨经颅多普勒超声(transcranial Doppler ultrasound,TCD)诊断大脑中动脉(middle cerebral artery,MCA)狭窄的价值.方法 对行数字减影血管造影(digital subtract angiography,DSA)和TCD检查的缺血性脑血管病患者的临床资料进行回顾性分析,以DSA为金标准,分析TCD诊断MCA狭窄的敏感性和特异性.对正常以及不同狭窄程度MCA的TCD血流速度进行比较,计算MCA不同狭窄程度时TCD血流速度的最佳截断值.结果 103例患者DSA证实存在MCA狭窄或闭塞,其中轻度狭窄12例,中度狭窄22例,重度狭窄40例,闭塞39例.与DSA相比,TCD检测中度以上MCA狭窄或闭塞的敏感性为78.8%,特异性为96%,准确度为93%,漏诊率为21.2%,误诊率为4%.轻度狭窄组与正常组之间血流速度无显著差异,中度狭窄组与正常组之间血流速度存在显著差异(P＜0.001),中度狭窄组与重度狭窄组之间血流速度无显著差异.判定中度狭窄的最佳收缩期峰值血流速度截断值为163.5 cm/s,平均血流速度的最佳截断值为108.5 cm/s.结论 TCD在诊断MCA狭窄或闭塞方面具有一定的优势,可作为DSA检查之前的一种安全而廉价的筛选手段.

Abstract：

Objective To explore the value of the diagnosis of middle cerebral artery (MCA) stenosis with transcranial Doppler ultrasound (TCD). Methods The clinical data in patients with ischemic cerebrovascular disease examined with digital subtract angiography (DSA)and TCD were analyzed retrospectively. DSA was used as a gold standard to analyze the sensitivity and specificity of the diagnosis of MCA stenosis with TCD. The normal and TCD blood flow velocity with different degrees of stenosis were compared. The best cut-off point of the TCD blood flow velocity of MCA at different degree of stenosis was calculated. Results DSA confirmed that 103 patients had MCA stenosis or occlusion, in which 12 were mild stenosis, 22 were moderate stenosis, 40 were severe stenosis, and 39 were occlusion. Compared to DSA, the sensitivity of TCD in detection of moderate and severe MCA stenosis or occlusion was 78. 8%, the specificity was 96. 0%, and the accuracy was 93. 0%, the missed diagnosis rate was 21. 2%, and the misdiagnosis rate was 4. 0%. As to the blood flow velocity, there was no significant difference between the mild stenosis and normal groups; while there was significant difference between the moderate stenosis and normal groups (P ＜0. 001). In addition, there was no significant difference in blood flow velocity between the moderate stenosis and severe stenosis groups. Determining the cut-off value of the best peak systolic velocity of the moderate stenosis was 163. 5 cm/s, while the best cut-off value of the mean velocity was 108. 5 cm/s. Conclusions TCD has certain advantages in the diagnosis of the MCA stenosis or occlusion, and it can be used as a safe and inexpensive screening means before DSA examination.     

婴儿良性肌阵挛癫(痫)综合征的电—临床研究进展

Dravet和Bureau于1981年首次报道了7例婴儿良性肌阵挛癫(痫)(benign myoclonic epilepsy in infancy,BMEI)[1].BMEI是极为罕见的癫(痫)综合征,迄今为止,文献所报道的病例不超过300例.1989年国际抗癫(痫)联盟(International League against Epilepsy,ILAE)正式将其归类为"全面性特发性癫(痫)"中[2],此后所有关于儿童癫(痫)文献都接受了"BMEI综合征"这一名称,说明此综合征已得到公认.然而,1989年ILAE的癫(痫)综合征分类,随着影像学和分子生物学等检查手段的发展,越来越不适于临床实践.因此,2006年ILAE发表了根据癫(痫)首发年龄划分的癫(痫)综合征[3-4],将BMEI列入婴幼儿期癫(痫)综合征范畴,2010年ILAE再次进行了癫(痫)综合征相关的概念修订[5].本文结合2005-2011年8项研究报道的80例BMEI患者的资料,对BMEI综合征的研究进展作一综述.     

婴儿良性肌阵挛癫1例报告

婴儿良性肌阵挛癫（benign myoclonic epilepsy in infancy,BMEI）由 Dravet和Bureau于1981年首次报道[1],为婴儿期起病的短暂肌阵挛发作,少伴其他发作类型,是极为罕见的癫综合征。迄今为止,文献所报道的病例不超过300例。1989年国际抗癫联盟（International League against Epilepsy, ILAE）将其归类为“全面性特发性癫”[2]。2006年ILAE发表了根据癫首发年龄划分的癫综合征[3,4],将BMEI列入婴幼儿期癫综合征范畴。2010年ILAE再次进行了癫综合征相关的概念修订[5]。本文报告1例婴儿良性肌阵挛癫发作病例。     

肌阵挛-失张力癫癎（Doose综合征）1例报告

肌阵挛-失张力癫癎（epilepsy with myoclonic-atonic seizures,EMAs）,又称Doose综合征。Doose等1970年首次描述了EMAS的临床特征,1989年国际抗癫癎联盟（ILAE）将其归类为“隐源性和（或）症状性全面性癫癎”,2010年ILAE在关于癫癎发作、综合征分类及术语报告中将EMAS列为按年龄分类的电临床综合征中儿童期的一种。