Nonspecific mitochondrial disease with epilepsy in children: diagnostic approaches and epileptic phenotypes

OBJECTIVES: This study sought to characterize epileptic phenotypes in children with nonspecific mitochondrial disease (MD) and to evaluate MD diagnostic approaches. METHODS: A retrospective analysis of the medical, electroencephalogram, and laboratory records of 142 patients with epilepsy was performed. The patients were evaluated for MD, and 124 patients were included in the final cohort. The MD criteria used included an oral glucose lactate stimulation test (OGLST) and urine organic acid/plasma amino acid (UOA/PAA) assays as metabolic indicators of modified Walker criteria, as suggested by Bernier et al. (Neurology 59:1406-1411, 2002). RESULTS: Twenty-two patients were classified as having definite MD (9), probable MD (5), possible MD (6), or pyruvate dehydrogenase (PDH) deficiency (3), including one patient which showed a respiratory chain (RC) defect and PDH deficiency. Seven out of eight patients in whom significant RC defects were observed showed complex I defects. In 14 patients, epileptic seizures start at infantile ages. Of 17 patients who substantially presented generalized seizures, 4 patients started with partial seizures. Five patients consistently presented only partial seizures. The OGLST and UOA/PAA assays were useful for a more precise diagnosis of MD, although low positive predictive value of the OGLST was regrettable. No patient was classified as definite MD by Walker's original criteria, but the use of our revised MD criteria resulted in the classification of nine additional patients as definite MD. CONCLUSIONS: MD manifested considerable diverse epileptic phenotypes and should be considered in the differential diagnosis of epilepsy in children with unexplained encephalomyopathy and progressive and fluctuating clinical courses.     

平肝止痛法联合西比灵胶囊治疗偏头痛35例

目的:观察平肝熄风,理气止痛类中药联合西比灵胶囊治疗偏头痛的疗效。方法:采用自拟平肝止痛汤(天麻、钩藤、川牛膝、生龙骨、生牡蛎等)联合西比灵胶囊治疗偏头痛35例,并设对照组仅采用西比灵胶囊对比。结果:治疗组总有效率82.86%,对照组54.28%,治疗组显著优于对照组(P<0.05)。提示:偏头痛采用平肝止痛类中药配西药具有良好疗效。     

电针治疗周围性面瘫疗效观察

目的:寻找能提高治疗周围性面瘫疗效的适宜的电针波型.方法:将147例周围性面瘫患者随机分为治疗组和对照组,治疗组进行穴位分组,电针波型先用连续波后用疏密波治疗;对照组穴位不分组,并单纯采用连续波治疗.结果:两组的总有效率和痊愈所用时间经统计学处理,差异有非常显著性意义(P＜0.01).结论:在电针治疗周围性面瘫中,将疏密波和连续波配合使用,效果显著优于单纯使用连续波者.     

复杂性手指离断再植方法的选择

随着人们生活水平的不断提高,患者对断指要求再植的迫切程度以及再植后的功能、外形要求越来越高。但临床上,几乎一半为复杂性断指,由于对部分复杂性断指的认识不足及处理方法不当,仍有不少断指放弃再植或再植后失败,因此复杂性断指再植一直是手外科领域重视的课题。自2003年11     

宫腔镜在辅助生殖技术中的应用

目的：探讨宫腔镜检查术在辅助生殖技术中应用的重要性。方法：对150例拟行辅助生殖技术的不育妇女行常规宫腔镜检查。结果：宫腔镜150例中47例（31％）有镜下异常，其中：子宫内膜息肉或息肉样增生18例（12％），子宫内膜单纯性增生17例（11％），子宫畸形7例（5％），宫腔粘连狭窄3例（2％），子宫内膜结核1例（0．7％），子宫黏膜下肌瘤或腺肌瘤1例（0．7％）。以宫腔镜诊断为标准，阴道超声诊断敏感性、特异性、阳性预测值、阴性预测值、准确率分别为66％（31／47）、98％（101／103）、94％（31／33）、86％（101／117）、88％（132／150），子宫造影分别为26％（12／47）、100％（95／95）、100％（12／12）、73％（95／130）、75％（107／142）。结论：宫腔镜检查术具有良好的可接受性和准确性，应作为辅助生殖技术前的常规检查。     

大脑中动脉支架内血栓形成1例报告

支架内血栓形成是支架置入术后的一种常见并发症。术后血管内皮损伤、胶原组织暴露和作为异物的支架均为引发血栓形成的可能机制。不能及时识别处理则成为再狭窄的重要原因。我们报告1例发生在大脑中动脉（middle cerebral artery，MCA）支架内的血栓形成，探讨其识别处理过程和可能的机制。     

Evaluation of left ventricular mechanical dyssynchrony as determined by phase analysis of ECG-gated SPECT myocardial perfusion imaging in patients with left ventricular dysfunction and conduction disturbances

Background  Cardiac resynchronization therapy (CRT) is approved for the treatment of patients with advanced systolic heart failure and evidence of dyssynchrony on electrocardiograms. However, a significant percentage of patients do not demonstrate improvement with CRT. Echocardiographic techniques have been used for more accurate determination of dyssynchrony. Single photon emission computed tomography (SPECT) myocardial perfusion imaging has not previously been used to evaluate cardiac dyssynchrony. The objective of this study is to evaluate mechanical dyssynchrony as described by phase analysis of gated SPECT images in patients with left ventricular dysfunction, conduction delays, and ventricular paced rhythms. Methods and Results  A novel count-based method is used to extract regional systolic wall thickening amplitude and phase from gated SPECT images. Five indices describing the phase dispersion of the onset of mechanical contraction are determined: peak phase, phase SD, bandwidth, skewness, and kurtosis. These indices were determined in consecutive patients with left ventricular dysfunction (n=120), left bundle branch block (n=33), right bundle branch block (n=19), and ventricular paced rhythms (n=23) and were compared with normal control subjects (n=157). Phase SD, bandwidth, skewness, and kurtosis were significantly different between patients with left ventricular dysfunction, left bundle branch block, right bundle branch block, and ventricular paced rhythms and normal control subjects (all P<.001) Peak phase was significantly different between patients with right ventricular paced rhythms and normal control subjects (P=.001). Conclusions  A novel SPECT technique for describing left ventricular mechanical dyssyn-chrony has been developed and may prove useful in the evaluation of patients for CRT. This study was funded in part by a research grant from the Medtronic-Duke Strategic Alliance, of which Dr Borges-Neto is the primary investigator.     

医用水刀的临床研究进展

人们发明了各种不同的手术工具,以便减少手术的危险。连续水流切割提供了一个相对出血量少的解剖过程,并且为手术者提供了一个清晰的视角。高压水流在解剖动脉外膜和邻近于脉管的软组织器官时性能优势,选择性的结扎能够完全的保护脉管和尿路。减少手术时间。因此,深入的研究这项技术是很有必要的。     

神经病学临床见习教学的思考

本文从多方面介绍了神经病学临床见习的教学方法,认为临床见习的重点是理论联系实际及学生临床思维能力的培养,促进医学生向医生的转变。     

《傅青主女科》调经特色探讨

从条目、辨证、治法、制方、用药几个方面探讨了<傅青主女科>调经的特色.指出傅青主治疗月经病善抓主症,辨证精要,治法注重扶正,善补肝血疏肝郁,制方严谨,主次分明,用药纯和,强调炮制以增药效等特点,并加以举例说明,对临床有一定指导意义.