合成促皮质素及维生素B6联合治疗婴幼儿癫痫的疗效观察

目的：探讨合成促皮质素（Ｃｏｒｔｒｏｓｙｎ，长效确杜先）及维生素Ｂ６（吡哆醇）联合治疗婴幼儿癫痫的疗效。方法：１４例年龄为３个月至４岁的患儿（婴儿痉挛症６例，其它顽固性癫痫８例）给予维生素Ｂ６ ５０～１００ｍｇ／ｄ肌肉注射１０天后，再以逐渐延长用药间隔的方式肌肉注射Ｃｏｒｔｒｏｓｙｎ０．０１５～０．０２５ｍｇ／ｋｇ．ｄ，总疗程约２个月，观察用药后的癫痫发作情况、脑电图（ＥＥＧ）改变、血像、肝肾功能及血电解质变化。结果：治疗后１１例患儿癫痫发作停止，２例顽固性癫痫患儿发作减少，分别由周１５次及２０次减少至每周３次及８次，１例顽固性癫痫患儿因不良反应明显终止治疗。ＥＥＧ及一般临床情况有改善。治疗过程中部分患儿有低血钾、低血钙、浮肿等，但血液肝肾功能检查未见异常变化。结论：Ｃｏｒｔｒｏｓｙｎ及维生素Ｂ６联合治疗婴儿痉     

40例婴儿痉挛症临床与脑电图分析

目的 :通过 40例婴儿痉挛症的临床与脑电图分析 ,探讨出生时不同程度的窒息史在婴儿痉挛症发病原因中的作用及脑电图在婴儿痉挛症诊断中的意义。方法 :本文的采用日本光电 4418型脑电图仪 ,按国际 10 /2 0系统安放电极 ,滤波 35HZ ,时间常数0 3S ,增益为 10uv/mm走纸速度为 30mm/s ,常规单、双极导联描记。结果 :40例患儿脑电图均为重度异常。即典型高度失律EEG及非典型高度失律 2 4例 (6 4% ) ,其他形式的痫样放电 9例 (2 2 5 % ) ,有爆发抑制现象 7例 (17 5 % )。结论 :围产期引起的窒息史是婴儿痉挛症发病的主要原因之一。婴儿痉挛症除典型临床发作及智力障碍外 ,具有特异性EEG改变 (高度失律 )是重要依据。     

大剂量维生素B_6治疗婴儿痉挛症

应用ACTH或糖皮质激素治疗婴儿痉挛症已有30余年的历史,约60％的病人对此疗法有效,但约1/3的病人复发。此外,激素常有严重的、甚至是威胁生命的副作用。而丙戊酸钠(VPA)和苯二氮草(艹卓)类(BZDs)则无疗效。由于致命的肝衰竭(特别好发于2岁内的儿童),对婴儿痉挛症患儿,VPA的使用需十分谨慎。BZDs只能短暂改善痫性发作,且常需附加其他疗法,因此,作者试用大剂量维生素B_6治疗婴儿痉挛症。 本文对17例婴儿痉挛症患儿采用维生素B_6治疗,开始有100mg/kg/天,分三次口服,六天内增至300mg/kg/天,如治疗一周内发作频率减少30％和/或EEG明显改善,继续再用大剂量B_6一     

首选多药联合治疗新诊断婴儿痉挛的临床疗效及随访研究

目的探讨首选多药联合治疗新诊断婴儿痉挛的疗效、对预后的影响及影响因素。方法 (1)收集自2007年9月～2010年11月本院收治的108例新诊断的婴儿痉挛患儿的病例资料,首选多药联合治疗,并进行追踪随访,对其临床资料回顾性研究。(2)治疗方案:采用ACTH、托吡酯、维生素B6、丙种球蛋白多药联合治疗。结果 (1)108例中90例完全控制,8例有效,10例无效,完全控制率83.3%,总有效率90.7%;(2)随访全部患儿5个月～3年,无1例死亡,痉挛发作完全控制者中复发率为7.8%(7/90);随访其中21例发作完全控制者入院时及痉挛发作完全控制后1个月时Gesell量表测定结果,无统计学意义,完全控制6个月时与治疗前Gesell量表测定结果比较,有统计学意义。结论 (1)多药联合治疗新诊断婴儿痉挛发作具有较高的完全控制率及有效率,症状性婴儿痉挛完全控制所需时间长于隐源性婴儿痉挛。(2)采用多药联合治疗新诊断婴儿痉挛可提高患儿智能,有助于改善预后。     

癫痫性痉挛患儿49例

目的 分析49例癫痫性痉挛患儿的临床、脑电图(EEG)特征。方法 对2014年6月至2022年8月于哈尔滨医科大学附属第一医院进行视频脑电监测(VEEG)证实有癫痫性痉挛发作且有完整临床资料的49例患儿临床和EEG资料进行分析,并按痉挛起病年龄分为三组:早发型痉挛(EOS)组,婴儿痉挛(IOS)组,晚发型痉挛(LOS)组。结果 (1)出生异常史EOS组与IOS组相比,差异有统计学意义(P<0.05);(2)精神运动发育迟滞LOS组与IOS组相比,差异有统计学意义(P<0.05);(3)神经影像学检查异常三组分别为75%、75%、100%;(4)发作间期IOS组高度失律常见,且睡眠期增多;EOS组、IOS组癫痫样放电均为后头部优势;(5)监测时发作特点为成串或孤立性痉挛可混合不典型失神发作、肌阵挛发作、强直发作等多种发作形式。结论 婴儿期癫痫性痉挛具有特征性临床和EEG表型,临床医生应重视其不典型症状及EEG变异型。     

婴儿痉挛症国内外药物治疗的现状与研究进展

婴儿痉挛症是一种较为罕见的难治性的癫痫性脑病,其发作难以控制,易转变为其他形式的癫痫发作,患儿多遗留神经发育障碍,预后不佳。促肾上腺皮质激素、糖皮质激素和氨己烯酸为该病的一线治疗用药,但上述药物控制患儿的癫痫发作、减少复发和改善患儿神经发育结果的效果仍未达到人们的预期,对于一线治疗不敏感的患儿,如何控制发作、改善预后仍是当前研究的热点与难点。本文对婴儿痉挛症国内外药物治疗的现状与研究进展作一综述。     

长期用硝基安定治疗儿童结节性硬化癫痫

自60年代用硝基安定治疗婴儿、儿童肌阵挛性癫痫以来,很少注意到对幼儿产生的付作用。作者报导90例5岁以上的结节性硬化患儿,86例并发癫痫,73例为婴儿痉挛症和/或儿童肌阵挛性癫痫。其中接受硝基安定治疗者56例,时间为1个月至13年,26例癫痫发作减轻或停止,30例无变化,部分患儿用药时间越长,其剂量也需变大。38例患儿用硝基安定平均治疗1.1年后(0.1～8.1年)撤除药物,最大平均剂量12mg/日(2～50mg/日),持续0.9年(0.1～6.1年)。撤除药物后,立即有癫痫大发作2例,其余未见癫痫加重,显示进步。8例患儿撤除药物后变得机灵。在治疗过程中15例嗜睡,17例运动功能降低、共济失调,4例不能行走,3例不能坐立。作者发现5岁时不能行走的患儿与早期发     

一例儿童致痫性局部皮质发育不良的脑电图演变

正局部皮质发育不良(Focal cortical dysplasia,FCD)是儿童难治性癫痫的重要病因之一,在年长儿多表现为部位相关的局灶性发作,低龄儿则可表现为痉挛发作及癫痫性脑病的电-临床特征。本文报道1例FCD所致晚发型痉挛患儿的脑电图(EEG)演变及治疗反应,探讨EEG对FCD病因学诊断的作用,以及对抗癫痫药物(AEDs)治疗有效的致痫性FCD的治疗策略。     

唑尼沙胺单药治疗新诊断的婴儿痉挛

唑尼沙胺(Zonisamide,3-sulfamoylmethyl-1,2-benzisoxazole,ZNS)的确切作用机制尚不清楚,似乎是通过阻断癫痫放电的传播和抑制引起癫痫病灶活动而发挥作用。临床上,ZNS最初用于简单和复杂部分性发作以及继发性全身强直一阵挛性发作。最近,有人报导了数例婴儿痉挛(IS的)患儿使用ZNS有效。但对多数患儿而言,ZNS仍仅作为联合治疗用药。本研究目的是确定ZNS单药治疗新诊断的IS的患儿的短期疗效。 收集了1995年1月～1996年1月间,住院治疗的13例新诊断为IS的病儿。所有病儿临床上均有典型的痉挛、智力迅速恶化,EEG示高峰节律紊乱或变异高峰     

癫痫患儿大脑半球切除术的护理体会

我科自1963年起应用大脑半球切除治疗伴有偏瘫的顽固性癫痫7例,年龄7～12岁。男性5例,女性2例。行右侧大脑半球切除2例,左侧大脑半球切除5例,均获满意疗效。现结合我们的护理体会,浅谈术前后护理。一、术前护理 1.婴儿性偏瘫患儿伴癫痫,此癫痫发作,用药难以控制。常伴有智力低下,性格暴躁,行为异常,很难管理。我们应主动向家属介绍,大脑半球切除术对控制癫痫发作优缺点,以取得家属理解,主动配合治疗及以后的康复问题。 2.协助做好术前有关检查,如EEG、CT、智力测验、言语、记忆以及Wada氏试验,测定言语功能半球的位置在何侧半球,以便术后对比。     

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.