儿童热性惊厥临床特征及其变化趋势

目的 探讨热性惊厥(FS)患儿的临床特征及其变化趋势.方法 回顾性总结2004~2013年10年间收治的1 922例FS患儿的临床资料,分析其临床特征及前后5年的变化趋势.结果 1 922例FS患儿中,男女比例为2.27 : 1,平均发病年龄3.0±1.8岁,发病年龄高峰为1~3岁.单纯型1 556例(80.96%),复杂型366例(19.04%).2008~2013年FS患儿1 202例,比2004~2008年(720例)增长了66.9%,且复杂型FS患儿比例升高(21.13% vs 15.56%)(P<0.05).与2004~2008年相比,2009~2013年单纯型FS患儿在惊厥发病年龄、发作时体温、抽搐持续时间及合并症发生率等方面差异均无统计学意义(均P>0.05);而复杂型FS患儿的发病年龄更小,发作时体温更低,抽搐时间更长,且出现心肌损伤、低钠血症等合并症的发生率更高(均P<0.05).结论 10年间FS患儿病例数呈上升趋势,复杂型FS发生率升高,且各临床特征均向不利方向变化,应重视复杂型FS的及时诊断和治疗.     

Slow sleep oscillation, rhythmic K-complexes, and their paroxysmal developments

This paper presents the relations between the slow (< 1 Hz) oscillation (characterizing the activity of corticothalamic networks during quiescent sleep in cats and humans), sleep K-complexes, and some paroxysmal developments of sleep patterns. At the cellular level, the slow oscillation is built up by rhythmic membrane depolarizations and hyperpolarizations of cortical neurons. The EEG expression of this activity is marked by periodic K-complexes which reflect neuronal excitation. The slow oscillation triggers, groups and synchronizes other sleep rhythms, such as thalamically generated spindles as well as thalamically and cortically generated delta oscillations. We discuss the distinctness of the slow (< 1 Hz) and delta (1–4 Hz) oscillations. We also show that the slow cortical oscillation underlies the onset of spike-wave seizures during sleep by transforming the periodic K-complexes into recurrent paroxysmal spike-wave complexes.     

Protective effect of adaptation to hypoxia and its prolongation by pharmacological agents in rats prone to audiogenic epilepsy

Cytophotometric determination of RNA in various brain structures of hypoxia-adapted and unadapted epilepsy-prone rats at different times after an epileptic seizure shows much smaller decreases in RNA levels and their much more rapid return to normal in the adapted than in the unadapted rats. The adaptation to hypoxia produces a marked anticonvulsive effect, and this effect is enhanced and prolonged considerably by pharmacotherapy. Translated fromByulleten' Eksperimental'noi Biologii i Meditsiny, Vol. 117, N ^o 2, pp. 120–123, February, 1994     

Brain malformations associated to Aldh7a1 gene mutations: Report of a novel homozygous mutation and literature review

Background

The ALDH7A1 gene is known to be responsible for autosomal recessive pyridoxine-dependent epilepsy (OMIM 266100). The phenotypic spectrum of ALDH7A1 mutations is very heterogeneous ranging from refractory epilepsy and neurodevelopmental delay, to multisystem neonatal disorder.

Anticonvulsant properties of the cerebrospinal fluid due to antiepileptic system activation

Laboratory of General Pathology of the Nervous System, Institute of General Pathology and Pathological Physiology, Academy of Medical Sciences of the USSR, Moscow. Department of Pathological Physiology, N. I. Pirogov Odessa Medical Institute. Translated from Byulleten' Éksprimental'noi Biologii i Meditsiny, Vol. 106, No. 8, pp. 146–149, August, 1988.     

热性惊厥患儿血清硫化氢水平测定及其与血清神经元特异性烯醇化酶的相关性

目的 探讨血清硫化氢(H2S)在热性惊厥(FS)发生发展过程中的病理生理学作用及其与血清神经元特异性烯醇化酶(NSE)的相关性.方法 选择2012年3月15日至11月10日就诊于南方医科大学珠江医院儿科中心的65例FS合并急性上呼吸道感染患儿为FS组,51例急性上呼吸道感染伴发热的患儿为上感组,同期于门诊体检的43例健康儿童为健康对照组.采用酶标仪测定各组儿童血清H2S与NSE水平.结果 FS组患儿血清H2S水平显著低于上感组和健康对照组,差异均有统计学意义(P均＜0.01);血清NSE水平均显著高于上感组和健康对照组,差异均有统计学意义(P均＜0.01).上感组患儿血清H2S、NSE水平与健康对照组比较差异均无统计学意义(P均＞0.05).FS组患儿血清H2S水平与血清NSE水平呈负相关(r=-0.279,P=0.024);不同惊厥发作次数(＜2次和≥2次)患儿血清NSE水平比较差异有统计学意义(t=-2.955,P=0.004);惊厥发作次数与血清H2S水平呈负相关(r=-0.269,P=0.03),与血清NSE水平呈正相关(r =0.322,P=0.009);惊厥持续时间(≥5 min)与血清H2S水平呈负相关(r=-0.532,P=0.019).结论 H2S可作为评估早期惊厥性脑损伤的客观指标,也可能是FS发生的重要因素.     

癫(癎)患儿预后与相关因素的远期随访研究

目的 分析影响癫(癎)患儿预后的相关因素,为癫(癎)的诊治和预后评估提供依据.方法 回顾性分析2003年1-12月在北京大学第一医院儿科门诊就诊的290例癫(癎)患儿的临床资料,随访患儿发作、用药、智力及精神运动发育等情况,以至少1年无发作为疗效控制指标.结果 ①经正规抗癫(癎)药物治疗,57.9%患儿发作控制满意,多数可以正常学习或生活;(②各型癫(癎)均有控制发作的可能,不同发作类型癫(癎)的控制率不同;③起病年龄越早,特别是1岁内起病者发作控制较差;④原发性癫(癎)控制率明显高于症状性或隐源性癫(癎),症状性癫(癎)预后最差;⑤大部份病例经单药治疗可以控制发作,2种药物治疗未控制者,再添加药物进行治疗,控制率无明显提高.结论 儿童癫痫的预后大多良好,有下列情况者预后差:①起病年龄小,尤其是1岁者;②同时有多种发作形式;③症状性癫(癎).     

听源性惊厥易感大鼠室旁核神经元c—fos表达的研究

目的为下丘脑室旁核参与惊厥应答提供形态学资料。方法：以听源性忭厥易大鼠为动物模型，用免疫组织化学ＡＢＣ法显示下丘脑室旁核神经元即早基因ｃ－ｆｏｓ的表达。