Mortality in epilepsy

Both community-based studies and reports from more selected epilepsy populations consistently reveal persons with epilepsy to have a mortality rate two to three times that of the general population. This increased rate is most pronounced in patients with remote symptomatic epilepsy, although many studies also report a significant excess mortality also among those with idiopathic epilepsy. The highest standardized mortality ratios (SMR) are seen in young age groups, mainly due to the low expected mortality in children, and during the first 5-10 years after diagnosis. Many of these observations suggest that the higher mortality is partly related to the underlying disorder causing epilepsy rather than a direct consequence of the seizures. For example, mortality in cerebrovascular diseases is increased, with SMRs ranging from 1.8 to 5.3 in the various studies. Deaths due to neoplasms, and in particular brain tumors, is also increased among patients with epilepsy. Accidents, status epilepticus, and sudden unexpected death (SUD) are more directly seizure-related causes of death. The incidence of such deaths vary considerably depending on the population being studied. While rare among patients with new-onset epilepsy, seizure-related deaths may account for up to 40% of all deaths in patients with chronic epilepsy. SUD is probably the most frequent seizure-related cause of death among young adults with epilepsy, with an SMR more than 20 compared with the general population. Seizure-induced autonomic cardiorespiratory effects have been suggested, but the mechanisms behind SUD are far from fully understood. It appears, however, that the risk of SUD is closely related to seizure frequency, being 40 times higher in patients who continue to have seizures than in those who are seizure-free.     

心理干预对突发性聋疗效影响的相关性研究

目的探讨心理因素与突发性聋疗效的关系。方法将150例突发性聋患者随机分为实验组和对照组，两组常规的治疗和护理方法相同，实验组则在此基础上首先评估心理状态并给予系统的心理干预，建立“共同参与”型的护患关系。结果实验组治愈率86．58％，对照组治愈率54．40％（P〈0．01），两组治疗前后心理状态经统计学处理，差异有统计学意义。结论重视在疾病发生发展中心理、社会、应激源等因素的相互作用，进行心理干预有助于突发性聋患者听力的恢复。     

中药针灸治疗突发性耳聋的疗效观察

目的观察中药、针灸对突发性耳聋的治疗效果。方法将符合突发性耳聋的108例(112耳)住院患者随机分成实验组和对照组,实验组以中药加针灸加西药治疗,对照组以西药治疗,对听力、耳鸣、眩晕的改善情况及起效时间进行观察比较。结果实验组听力愈显率为71·9%,耳鸣愈显率为86·5%,听力起效时间为2·50±2·09天,耳鸣起效时间为1·96±1·72天,对照组分别为58·2%、66·7%、4·20±2·10、3·52±2·20,2组各数据比较,均差异有显著意义;而2组对眩晕改善程度及起效时间比较,分别为100%、97·4%;1·80±1·50天、1·96±1·27天,差异无显著意义。结论中药、针灸治疗突发性耳聋有乐观的前景。     

灯盏细辛注射液与常规西药治疗突发性耳聋随机对照研究

目的对比灯盏细辛注射液与常规西药治疗突发性耳聋的疗效,提供较为规范的随机对照研究方法.方法灯盏细辛注射液治疗作为治疗组,静脉输入低分子右旋糖酐、能量合剂等常规西药治疗作为对照组;每组各50例,对照观察其临床疗效.结果治疗组痊愈21例,显效19例,有效7例,无效2例,总有效率94%,疗效优于常规西药组(P＜0.05).结论灯盏细辛注射液治疗突发性耳聋疗效及对听力的改善优于常规西药对照组.     

血小板、蛋白激酶C、脂蛋白(a)检测在老年突发性聋诊断中的价值

目的研究血小板(PLT)、蛋白激酶C(PKC)及脂蛋白a[LP(a)]在老年性突发性聋中的变化及作用.方法老年性突发性耳聋和健康对照组各30例,分别检测脂蛋白(a)[LP(a)],总胆固醇(TC)、甘油三脂(TG)、高密度脂蛋白(HDL-C)、低密度脂蛋白(LDL-C)、载脂蛋白AL(apoAI)、载脂蛋白B(apoB)、血小板(PLT)、膜和浆蛋白激酶C(M-PKC和P-PKC),对检测结果做统计学分析.结果突发性耳聋组LP(a)、apoB、TG均明显升高,HDL-C则明显减低,与对照组比较,差异均有显著性(LP(a)t=2.33,P＜0.05.apoB:t=2.83,P＜0.晒.TG:t=2.81,P＜0.05.HDL-C:t=3.58,P＜0.01),PLT计数明显减低,与对照组比较,差异具有显著性t=2.25,P＜0.05.M-PKC活性增高、P-PKC活性减低,与对照组比较,前者差异具有非常显著性(t=3.26,P＜0.01),后者差异具有显著性(t=2.22,P＜0.05).结论老年性突发性耳聋时LP(a)、apoB及M-PKC增高,而PLT减低,在血栓形成过程中起重要的作用,对突发性耳聋的发病起促进作用.     

清肝通窍汤对突发性聋豚鼠血液流变学影响的研究*

目的观察清肝通窍汤对突发性聋模型豚鼠血液流变学的影响。方法30只豚鼠，以噪声刺激加肾上腺素皮下注射法制备突发性聋模型，然后随机分为模型组、治疗组、阳性对照组，每组10只，并设立空白对照组（10只）。治疗组予以清肝通窍汤治疗，阳性对照组用血府逐瘀口服液治疗，模型组和空白对照组分别以蒸馏水灌胃。观察比较各组豚鼠的血液流变学指标及纤维蛋白原含量变化。结果造模后，模型豚鼠不同切变率下的全血粘度、红细胞压积、血浆粘度及纤维蛋白原含量均明显升高。服用清肝通窍汤组动物各项指标值均降低而接近正常。结论清肝通窍汤能较好改善突发性聋模型豚鼠因血液流变性改变而造成的微循环障碍，为临床治疗突发性聋提供了实验依据。     

Hypertrophy of lymphoid organs is a possible phenotypic characteristic of R420W mutation of the cardiac ryanodine receptor gene: A study using a knock-in mouse model

Cardiac ryanodine receptor gene (RyR2) mutations sometimes result in sudden cardiac death due to fatal arrhythmias. N-terminal R420W mutation of RyR2 is known to show similar phenotypes to arrhythmogenic right ventricular cardiomyopathy and to cause juvenile sudden death. We previously reported two sudden death cases with the same R420W mutation. Interestingly, the cases showed hypertrophy of lymphoid organs such as the thymus and mesenteric lymph nodes. The present study examined whether R420W mutation of RYR2 causes hypertrophy of lymphoid organs by generating a mouse model carrying the mutation. Homozygous (RyR2^R420W/R420W) mice showed significant increases in thymus and spleen weights but not in kidney, heart, and brain weights compared with wild-type mice. The mice also showed remarkable hypertrophy of mesenteric lymph nodes. Immunohistochemical study revealed that RyR2 protein was prominently expressed in epithelial cells of the thymic medulla in the thymus. These findings show that mice with R420W mutation of RyR2 exhibit hypertrophy of lymphoid organs. Sudden unexplained death cases with the mutation may display such findings at autopsy.     

The multi-channel cochlear implant and the relief of severe-to-profound deafness

Abstract

This personal reflection outlines the discoveries at the University of Melbourne leading to the multi-channel cochlear implant, and its development industrially by Cochlear Limited. My earlier experimental electrophysiological research demonstrated temporal coding occurred for only low frequencies, i.e. below 200–500 pulses/second. I was able to confirm these findings perceptually in behaviourally conditioned animals. In addition, these studies showed that temporal discrimination occurred across spatial coding channels. These experimental results correlated with the later conscious experience for electrical stimulation in my implant patients. In addition, the mid-to-high frequencies were coded in part by place of stimulation using bipolar and monopolar stimulation to restrict current spread. Furthermore, place of stimulation had the qualities of sharpness and dullness, and was also experienced as vowels. Owing to the limitation in coding speech with a physiological model due to the overlap of electrical current leading to unpredictable variations in loudness, a speech coding strategy that extracted the most important speech features for transmission through an electro-neural ‘bottle-neck’ to the brain was explored. Our inaugural strategy, discovered in 1978, extracted the second formant for place of stimulation, voicing for rate of stimulation, and sound pressure for current level. This was the first coding strategy to provide open-set speech understanding, as shown by standard audiological tests, and it became the first clinically successful interface between the world and human consciousness. This strategy was improved with place coding for the third formant or high-frequency spectrum, and then the spectral maxima. In 1989, I operated on our first patient to receive a bilateral implant, and in 1990, the first with a bimodal processor. The psychophysics and speech perception for these showed that the stimuli from each side could be fused into a single image, and localized according to differences in intensity and time of arrival of the stimuli. There were significant improvements for speech perception in noise. In 1985, I implanted our first children with the multi-channel prosthesis and found that speech understanding and spoken language were greatly improved the younger the child at surgery, and especially when younger than 12 months. Speech understanding was strongly related to the development of place coding. In 1990, the US Food and Drug Administration approved the implant for deaf children, the first by any world health regulatory body making it the first major advance in helping deaf children to communicate.