10例多发性硬化伴癫痫发作与MRI相关性分析

目的:探讨多发性硬化(MS)伴癫癎发作患者的临床特点与MRI所示病灶的相关性。方法:回顾性分析121例确诊为MS住院患者中10例(8.29%)伴癫癎发作的临床特点及MRI表现。结果:癫癎在MS其他症状或体征之前出现2例;癫癎发作为MS复发时唯一症状的1例;癫癎发作时已伴MS其他症状或体征者7例。10例患者头颅MRI均示双侧半球的深部白质、侧脑室旁数个斑块病灶,其中5例伴皮质-皮质下斑块病灶,2例伴局灶性皮质萎缩。5例癫发作与皮质-皮质下斑块病灶有相关性。结论:癫癎可以是MS的首发症状或复发时唯一临床表现,MS患者癫癎发作与皮质-皮质下斑块病灶相关。     

发作性运动诱发性运动障碍临床分析

目的探讨发作性运动诱发性运动障碍的临床特点。方法对23例PKD患者的临床资料进行分析,归纳其特点。结果 23例患者起病年龄6~18岁,病程1~27a,男性占大多数,男女比例为3.6∶1。突发启动的自主运动诱发,以单侧肢体舞蹈样手足徐动多见,部分表现为双侧,持续10s左右,无意识障碍,脑电图、头颅MRI、CT正常。小剂量卡马西平控制发作。结论 PKD是一种发作性运动诱发的、短暂的局部或全身不随意运动,卡马西平治疗有效。     

多发性硬化的发作性症状分析

目的 探讨多发性硬化中发作性症状的临床特点、发病机制和治疗方法.方法 对15例具有发作性症状的MS患者根据不同临床特点分类,分析影像学、电生理特点,总结其异同点.结果 多发性硬化中发作性症状的发生率、临床表现不一,其中以痛性强直发作、发作性感觉异常、Lhermitte征和癫最为常见.结论 MS发作症状共同点为突发突止,刻板重复,时间短暂,可能的发病机制是由于中枢神经系统脱髓鞘损害,使神经纤维间的冲动发生横向扩散所致,卡马西平等抗癫药治疗有效.     

卡马西平联用盐酸哌甲酯控释片治疗小儿癫痫合并注意缺陷多动障碍的疗效观察

目的探讨卡马西平联合盐酸哌甲酯控释片治疗小儿良性癫痫(benign epilepsy with centro-temporal spikes.BECTS)伴中央颞区棘波合并注意缺陷多动障碍(attention deficit hyperactivity disorder.ADHD)的临床疗效。方法选择2011年1月至2016年1月于本院精神内科治疗且确诊BECTS伴ADHD的患儿80例作为研究对象,首先采用卡马西平单药治疗,若6个月内复查脑电图稳定,联用盐酸哌甲酯控释片进行继续治疗。观察盐酸哌甲酯控释片治疗前后患儿注意力和行为改善情况、癫痫发作情况、不良反应和治疗效果。结果卡马西平治疗3个月后有效的56例患儿联合盐酸哌甲酯治疗6个月治疗后,ADHD治疗总有效率为92.73%(51/55),且与联合治疗前相比,Conner儿童行为的问卷评分显著降低。1例(1.82%)患儿在治疗2个月后癫痫发作频率和EEG异常放电频率增加,停药后即未发作,停药4周后再服药,未再出现癫痫发作增加现象。结论卡马西平联用盐酸哌甲酯控释片能够有效的控制BECTS伴ADHD患儿的临床症状,明显改善患儿的注意力及其社会功能,且具有一定的临床安全性。     

发作性运动诱发性运动障碍(附7例临床报道)

目的探讨发作性运动诱发性运动障碍(PKD)的临床特点,以加强对本病的认识。方法分析7例PKD患者的临床资料。结果7例患者平均发病年龄14岁,男性多见,临床主要表现为由突然起始动作诱发的发作性姿势性肌张力障碍,单侧受累多见,发作时间不超过2min,发作时意识清楚,口服卡马西平片均得到有效控制。结论本病的诊断依靠对其临床特点的认识,卡马西平治疗效果好。     

脊髓型多发性硬化的诊断(附22例报告)

目的　探讨脊髓型多发性硬化 (MS)的临床和影像学特点及其诊断意义。方法　对 2 2例脊髓型 MS的临床特点和磁共振 (MRI)结果进行回顾性分析。结果　脊髓型 MS,占同期 MS住院患者的 1 1 .8%。女性比例较高 ,多见于中年发病 ,常以急性或亚急性起病 ,表现为复发 -缓解或慢性进展病程 ,最常累及中高位颈髓和中段胸髓 ,大多复发仍局限于原发部位。MRI特点为髓内散在的长 T1、长 T2斑块状病灶 ,其长度一般小于 2个椎体的长度 ,位于脊髓的后外侧 ,面积小于脊髓横截面的 1 /2。应用糖皮质激素试验性治疗对诊断 ,尤其是对首次发病时脊髓出现可疑脱髓鞘病灶者诊断有一定帮助。结论　诊断脊髓型 MS主要依据其临床表现 ,MRI为最敏感的检查方法 ,激素试验性治疗有时是必要的。     

ChiarⅠ型畸形的MRI表现及其手术治疗

目的探讨ChiariⅠ型畸形的MRI分型及治疗方法.方法 23例Chiari Ⅰ型畸形患者,21例有颈神经根症状,16例表现为中央管周围损害症状,另有8例分别存在小脑及颅神经损害症状.MR检查显示,单纯小脑扁桃体下疝(B型)7例(30.43%),小脑扁桃体下疝合并脊髓空洞症(A型)16例(69.57%).根据MRI所显示病变部位,采用不同的手术方式,其中11例行后颅窝减压术,7例行后颅窝减压并小脑扁桃体切除术,5例行后颅窝减压并脊髓空洞切开引流术.结果手术后18例患者(78.26%)症状改善,5例(21.74%)与手术前比较无明显变化.对16例伴脊髓空洞症患者中的13例进行随访,其中3例空洞消失,7例空洞显著缩小,3例空洞较手术前缩小50%.结论手术治疗可使延髓、颈髓充分减压,有效缓解临床症状,并对脊髓空洞症具有良好的治疗效果.     

卡马西平治疗新诊断部分性发作癫痫患者的预后

癫痫是神经科常见疾病,其中约2/3为部分性发作患者.多数癫痫患者预后良好,但仍有30％～40％的患者经适当抗癫痫药物(AEDs)治疗后仍有发作,成为耐药性癫痫[1-3].既往有关癫痫患者预后的研究,观察对象大多是所有发作类型癫痫患者,很少有针对部分性发作癫痫患者的研究;有研究发现部分性较全面性发作癫痫患者预后差,易发展为耐药性癫痫[4-6].卡马西平是公认治疗部分性发作癫痫的首选药物,新诊断部分性发作癫痫患者初次给予卡马西平治疗效果如何?对卡马西平初次治疗效果差的患者再次给予其他AEDs治疗,其预后如何?     

奥卡西平与卡马西平单药治疗儿童部分性癫(癎)的对照研究

目的 比较奥卡西平与卡马西平单药治疗儿童部分性癫(癎)的疗效.方法 71例新诊断的儿童部分性癫(癎)患者按单双号顺序分为奥卡西平组(35例)和卡马西平组(36例),并给予相应的药物治疗;6个月后进行疗效评价,观察不良反应.结果 奥卡西平组和卡马西平组分别有25例及29例完成6个月治疗;脑电图改善率分别为44.0%及44.8%;显效率分别为92.0%及86.2%;不良反应发生率分别为22.2%及41.2%,两组间比较差异无统计学意义(均P>0.05);卡马西平组2例出现严重不良反应.结论 奥卡西平与卡马西平单药治疗新诊断的儿童部分性癫(癎)均有很好的疗效,不良反应及耐受性相似,但奥卡西平组未见严重的不良反应.     

卡马西平联合丙戊酸钠治疗额叶癫痫的疗效研究

目的 研究丙戊酸钠和卡马西平联合治疗对额叶癫痫的临床疗效和安全性.方法 将额叶癫痫患者随机分为联合用药组(86例)和对照组(83例),联合用药组给予丙戊酸钠和卡马西平联合治疗,剂量分别为20mg(kg·d)-1、10 mg(kg·d)-1;对照组给予卡马西平治疗,随访半年.结果 联合用药组的疗效为:显效率54.7%、有效率30.2%、无效率15.1%;对照组的疗效为:显效率42.2%、有效率14.5%、无效率43.3%.两组的疗效有统计学差异.结论 丙戊酸钠和卡马西平联合治疗额叶癫痫能够明显地提高疗效,并且不良反应无明显增加,值得临床作为首选治疗方案推广应用.     

Denervation study of synapses of organ of corti of old world monkeys

Fine structural characteristics of synapses in the spiral organ of Corti were examined, with reference to differences between inner and outer haircell systems, and to location of neurons of origin of efferent axons. Surgical interruption of crossed olivocochlear bundle, of vestibular nerve, of facial nerve, and excision of superior cervical ganglia were used to determine the pathways of efferent axons. Interruption of the vestibular nerve near the brainstem results in degeneration of all efferent terminals on outer hair cells. Mid-line lesions at, and caudal to, the facial colliculus result in degeneration of about half of these efferent terminals. Efferent synaptic bulbs to the inner hair-cell system are small, of the order of one micron, and form type 2 junctions with afferent dendrites. They tend to have more large dense-core vesicles (about 80 nm) than the large efferent terminals of the outer hair-cell system, and appear to be the terminals of axons in the habenula perforata, which exhibit varicosities laden with large dense core vesicles. The varicosities are unaffected by excision of the superior cervical ganglia. So far as our material can reveal, it appears that the varicosities in the habenula perforata do not survive vestibular root interruption, nor do the efferent processes in the internal spiral bundle or at the base of inner hair cells. Most interestingly, the afferent processes of the inner hair-cell system, as identified for example by their relation to pre-synaptic bodies in the inner hair cells, are subject to a trans-synaptic reaction after severance of the vestibular root. They undergo a dramatic cytological transformation, characterized by increase of volume, engorgement with microtubules, microfilaments, microvesicles of various sizes, and clusters of lysosomes. Thus, both the efferent and afferent terminals of the inner hair-cell system show marked cytological differences from the corresponding terminals of the outer hair cell system.     

Mechanism of action of tubocurarine on nicotinic cholinoreceptors of neurons of the sympathetic ganglia of rats

Tubocurarine (Tc) effect on membrane currents elicited by acetylcholine (ACh) was studied in isolated superior cervical ganglion neurons of rat using patch-clamp method in the whole-cell recording mode. The "use-dependent" block of ACh current by Tc was revealed in the experiments with ACh applications, indicating that Tc blocked the channels opened by ACh. Mean lifetime of Tc-open channel complex, tau, was found to be 9.8 +/- 0.5 s (n = 7) at -50 mV and 20-24 degrees C. tau exponentially increased with membrane hyperpolarization (e-fold change in tau corresponded to the membrane potential shift by 61 mV). Inhibition of the ACh-induced current by Tc (3-30 microM/1) was completely abolished by membrane depolarization to the level of 80-100 mV. Inhibition of ACh-induced current was augmented at increased ACh doses. It is concluded that the open channel block produced by Tc is likely to be the only mechanism for Tc action on nicotinic acetylcholine receptors in superior cervical ganglion neurons of rat.     

The effect of age of onset of PD on risk of dementia

Background Dementia occurs in the majority of patients with Parkinson’s disease (PD). Late onset of PD has been reported to be associated with a higher risk for dementia. However, age at onset (AAO) and age at baseline assessment are often correlated. The aim of this study was to explore whether AAO of PD symptoms is a risk factor for dementia independent of the general effect of age. Methods Two community-based studies of PD in New York (n = 281) and Rogaland county, Norway (n = 227) and two population-based groups of healthy elderly from New York (n = 180) and Odense, Denmark (n = 2414) were followed prospectively for 3–4 years and assessed for dementia according to DSM-IIIR. All PD and control cases underwent neurological examination and were followed with neurological and neuropsychological assessments. We used Cox proportional hazards regression based on three different time scales to explore the effect of AAO of PD on risk of dementia, adjusting for age at baseline and other demographic and clinical variables. Findings In both PD groups and in the pooled analyses, there was a significant effect of age at baseline assessment on the time to develop dementia, but there was no effect of AAO independent of age itself. Consistent with these results, there was no increased relative effect of age on the time to develop dementia in PD cases compared with controls. Interpretation This study shows that it is the general effect of age, rather than AAO that is associated with incident dementia in subjects with PD. Received in revised form: 22 December 2005     

Limits of deinstitutionalization--perspectives of relatives of psychiatric patients

After a hopeful beginning, the social process of the reintegration of those with severe mental illness has come to a standstill. I am led to wonder whether "the community" really wants to live together with people suffering from severe mental illness, and if so, how closely? As long as the medical treatment of mental illness provided by the general practitioners is fundamentally deficient, as they are not able to prescribe the necessary interventions--such as out-patient psychiatric nursing, and service providers in the out-patient sector are content with offering increasingly intensive forms of care for the less seriously ill at the cost of the Social Welfare System--the reintegration of those with serious mental illness remains an illusion--which is mainly to the benefit of providers of residential care in homes and hostels.