婴儿痉挛的临床和视频脑电图特征分析

目的总结婴儿痉挛(West综合征)的临床和视频脑电图(Video-EEG)特征。方法对28例婴儿痉挛患儿的临床资料及视频脑电图特征进行同步分析。结果发病年龄2~14月。28例患儿均监测到痉挛发作,发作形式有屈肌型、伸肌型和混合型多种形式,多发生于入睡后不久或睡眠觉醒时,一日内可散发或密集发作。27例患儿可寻找到病因。发作间期脑电图19例表现为背景活动呈典型高峰节律紊乱,9例为背景活动呈变异型高峰节律紊乱。发作期脑电图17例表现为高波幅慢波夹杂棘波爆发-低电压抑制,10例表现为全脑各导呈广泛性低电压抑制,1例表现为假性正常脑电图改变。结论婴儿痉挛的特点:①绝大多数婴儿期内发病;②发作类型特殊;③脑电图有特征性改变,视频脑电图监测对其诊断及分型有重要意义;④绝大多数患儿伴有智力低下或下降,随着年龄的变化可转变成其他形式的发作;⑤绝大多数患儿治疗困难,预后差;仅少数患儿智力发育正常,无病因可寻;药物治疗疗效满意,为特发性癫。     

36例脑瘫合并癫痫的临床发作类型与脑电图分析

目的了解脑瘫合并癫痫的临床发作类型与脑电图的关系。方法对36例癫痫发作间歇期的脑瘫患儿采用视频脑电图仪，按国际10-20系统放置头皮电极检测脑电图。结果脑瘫合并癫痫患儿发作间歇期脑电图表现为限局性高波幅的棘慢波发放占55．6％（20／36）；典型高度失律4例；高度失律改善型3例；双侧导联棘慢波发放的占25％（9／36）。结论脑瘫患儿癫痫发作类型及脑电图改变多符合部分性发作的特点。     

婴儿痉挛20例临床与脑电图特征分析

目的：认清婴儿痉挛的临床发作特征及脑电图特征对诊断婴儿痉挛有重要的意义。方法：对2001-01～2006-01我院儿科20例婴儿痉挛患儿采用脑电图仪检测患儿脑电图,并结合临床发作形式及发作间期脑电图特征进行诊断。结果：20例患儿发作形式多表现为屈肌型,少数为伸展型,并于发作间期给予脑电图检测：发作间期脑电图表现为高峰节律紊乱,睡眠波形消失。结论：婴儿痉挛发作多为成串丛集发作,表现为迅速点头,躯干屈曲,双手拥抱状,或向后成角弓反张样,间歇期脑电图为高峰节律紊乱,为临床诊断提供依据。     

41例婴儿痉挛症临床和脑电图分析

报道41例婴儿痉挛症患儿的临床特点和脑电图改变,所有病例脑电图均为高度失律改变、并有各种痫性放电,提示小儿睡眠脑电图描记能提高婴儿痉挛症脑电图阳性率。     

婴儿良性癫痫的临床特征以及对智力发育的影响:3年随访

目的观察婴儿良性癫痫的发作特征、治疗反应及本病对智力发育的影响。方法1998-03/2002-12,对2岁内起病,排除高热惊厥、婴儿痉挛症及发育异常的婴儿惊厥进行临床观察、脑电图监测、用药效果,在3岁后进行了智力测定。结果经临床观察12例患儿确诊为婴儿良性癫痫。其中表现为全身性惊厥者5例,发作时间多为入睡前或凌晨;口—咽自动症3例;阵发性青紫4例,均发生在白天。全部病例均为短期内频繁发作,无癫痫持续状态。其中8例脑电图正常;2例发作期出现一侧和双侧枕叶局限而持续的棘慢波;2例出现一侧性颞区小尖棘波,发作间期背景活动正常。11例接收抗癫痫单药治疗,平均用药时间1年,1例未用任何药物治疗,全部患儿起病1年内发作均消失。并在3～6岁内接受了智力测定。与正常儿童对照差异无显著性意义(t=9.032,P>0.05)。结论婴儿良性癫痫临床特征明显,患儿的智力发育不受影响。     

首选多药联合治疗新诊断的婴儿痉挛患儿动态脑电图及血清神经元特异性烯醇化酶水平的变化

目的 观察首选多药联合治疗新诊断婴儿痉挛患儿动态脑电图(EEG)及血清神经元特异性烯醇化酶(NSE)的变化情况,探讨其疗效及预后的影响因素.方法 对2007年9月至2010年11月我院新诊断的108例婴儿痉挛患儿采用首选多药联合治疗,观察动态脑电图及血清NSE水平改变情况.结果 (1)90例完全控制患儿完全控制1周内复查动态脑电图结果显示:正常动态EEG 56例(62.2％),高峰失律消失、散发尖慢波32例(35.6％),仍存在高峰失律2例(2.2％),总有效率97.8％.(2)收集入院时、痉挛发作完全控制后48 h内及痉挛发作控制后1个月均行血清NSE检测的35例患儿的资料,结果表明痉挛发作完全控制后48 h内血清NSE含量(17.7±5.5)μg/L较人院时(21.2±6.8)μg/L明显降低(t =6.54,P＜0.05);痉挛控制后1个月血清NSE含量(9.0±3.1) μg/L较入院时进一步降低(t =9.29,P＜0.01).结论 多药联合治疗婴儿痉挛患儿脑电图改善较明显,发作控制后血清NSE含量降低.     

促肾上腺皮质激素联合咪达唑仑治疗首发婴儿痉挛症的护理

[目的]总结促肾上腺皮质激素(ACTH)联合咪达唑仑治疗首发婴儿痉挛症的护理。[方法]对10例首发婴儿痉挛症患儿采用ACTH联合咪达唑仑治疗,之后过渡到口服醋酸泼尼松与硝基安定,用药前做好解释宣教工作,用药时密切观察,加强护理,及时干预。[结果]治疗后完全控制痉挛发作7例,减轻发作3例;治疗后再次行1次长达2h的视频脑电图检查,高峰失律波消失4例;10例患儿出院后定期门诊复查,抽搐控制良好。[结论]加强首发婴儿痉挛症患儿行ACTH联合咪达唑仑治疗的护理有利于预后。     

婴儿良性癫痫的临床特征以及对智力发育的影响：3年随访

目的观察婴儿良性癫痫的发作特征、治疗反应及本病对智力发育的影响。方法1998—03／2002—12，对2岁内起病，排除高热惊厥、婴儿痉挛症及发育异常的婴儿惊厥进行临床观察、脑电图监测、用药效果，在3岁后进行了智力测定。结果经临床观察12例患儿确诊为婴儿良性癫痫。其中表现为全身性惊厥者5例，发作时间多为入睡前或凌晨；口一咽自动症3例；阵发性青紫4例，均发生在白天。全部病例均为短期内频繁发作，无癫痫持续状态。其中8例脑电图正常；2例发作期出现一侧和双侧枕叶局限而持续的棘慢波；2例出现一侧性颞区小尖棘波，发作间期背景活动正常。11例接收抗癫痫单药治疗，平均用药时间1年，1例未用任何药物治疗，全部患儿起病1年内发作均消失。并在3～6岁内接受了智力测定。与正常儿童对照差异无显著性意义(t=9．032，P&;gt;0．05)。结论婴儿良性癫痫临床特征明显，患儿的智力发育不受影响。     

脑性瘫痪合并癫痫的临床及脑电图分析

目的 探讨脑瘫合并癫痫患儿的临床特征以及相关脑电图特点.方法 收集84例脑瘫合并癫痫患儿的临床资料及发作间歇期脑电图结果,与124例脑瘫不伴癫痫患儿的相关资料对比分析.结果 脑瘫合并癫痫的84例患儿中46例(54.76%)为部分性发作,其次为婴儿痉挛26例(30.95%)、强直-阵挛发作6例(7.14%),痉挛型所占比例最高,为82.14%,其中以痉挛型四肢瘫最多;脑电图异常以局灶或多灶痫样放电多见,脑电图异常率显著高于不伴癫痫的患儿(P＜0.001).结论 脑瘫合并癫痫患儿的癫痫类型主要为部分性发作;痉挛型脑瘫是脑瘫中伴发癫痫最多的类型;脑电图异常以局灶痫样放电和多灶痫样放电多见.     

偏侧惊厥-偏瘫-癫痫综合征的诊断学特征并文献复习

目的探讨偏侧惊厥-偏瘫-癫痫综合征的诊断学特征。 方法回顾性分析2018年4月16日河北省人民医院儿科收治的1例临床表现为偏侧惊厥-偏瘫-癫痫综合征患儿的临床资料,并复习相关文献。 结果患儿以右侧肢体抽搐起病,后表现为惊厥持续状态,左侧肢体偏瘫,好转后反复癫痫发作。头颅CT及磁共振成像(MRI)均提示钙化灶。发作期视频脑电图检查提示右侧枕区低中幅连续性棘波、棘慢波节律。患儿好转后复查视频脑电图,间歇期：双侧半球各导联可见大量弥漫性中高幅1.5～3.0 Hz慢波活动,左侧半球明显多于右侧;发作期：监测期未见癫痫。 结论在偏侧惊厥-偏瘫-癫痫综合征患儿中,脑电图及特征性神经影像学异常对诊断有一定的帮助。     

Ictal and interictal EEG findings in children with migraine

Abstract We describe the electroencephalographic findings in nine pediatric migraineurs during the ictal phase of pain. We observed abnormal patterns on electroencephalography (EEG) in seven patients with migraine with aura and in two patients with basilar migraine. These patients were also evaluated with EEG during headache-free interval, at least 7 days after the ictal event; these interictal findings were unremarkable, confirming the observations reported in the literature. Finally, we review migraine and its EEG characteristics as reported in the literature, and discuss the value of EEG performed during interictal and ictal phases of migraine.     

N-甲基-D-天门冬氨酸(NMDA)诱导幼鼠痫样发作的特征与评价

目的探讨N-甲基-D-天门冬氨酸(NMDA)诱导幼鼠痫样发作的特征,并评价它与人类婴儿痉挛症的异同。方法实验一:24只乳鼠随机分为对照组和实验组,注射不同剂量NMDA,观察行为变化及最佳NMDA剂量。实验二:30只18天乳鼠随机分为对照组和实验组,于不同时间点注射NMDA(45mg/kg),观察发作高峰期并记录头皮脑电图。实验三:6只乳鼠分别于生后18~30天腹腔注射NMDA(45mg/kg),观察慢性点燃模型建立情况。实验四:4只成鼠及4只乳鼠分别腹腔注射NMDA,观察NMDA引起的癫痫发作是否具有年龄依赖性。结果不同剂量的NMDA均可诱导乳鼠出现特异的屈曲样痉挛发作,发作期脑电图出现节律紊乱,表现为高幅慢波,夹有棘波。18天的幼鼠需要的最佳NMDA剂量为45mg/kg,该量处理后20~40分钟为发作高峰。乳鼠连续腹腔注射NMDA至生后27天开始无特异的屈曲样痉挛发作。成鼠腹腔注射NMDA后不出现特异的屈曲样痉挛发作。结论Wistar乳鼠经腹腔注射NMDA,其行为学表现与人类婴儿痉挛症的临床发作极为相似,提示可以作为一种建立婴儿痉挛症动物模型的方法。     

Secondary myocardial hypertrophy in newborn infants and infants without congenital heart defect

Secondary hypertrophic cardiomyopathy (HCM) was diagnosed in 55 newborns and infants without heart disease. The 17 newborn infants of diabetic mothers, 23 infants given ACTH treatment for infantile spasms, 9 ventilated premature infants or babies with bronchopulmonary dysplasia and 6 infants with different underlying diseases comprising this group underwent 2-dimensional echocardiography. In almost all cases echocardiographic evidence of HCM resolved after removal of the exogenous or endogenous causative factor. Myocardial effects of several substances are known and their pathophysiological mechanisms are discussed. Since HCM may precede systemic disease or may often be the first pointer to a triggering agent, further diagnostic procedures are always indicated. Hence, the diagnosis of primary HCM should be made by exclusion.     

Autonomic epilepsy--the influence of epileptic discharges on heart rate and rhythm.

PURPOSE: To study cardiac alterations (changes in heart rate and cardiac arrhythmias) at the transition from the pre-ictal to the ictal state during focal epileptic seizures. METHODS: We assessed ECG changes during 92 seizures recorded with scalp EEG in 30 patients and 35 seizures in 11 patients evaluated with subdural strip and/or grid electrodes. Consecutive RR intervals were analyzed with a newly developed mathematical model for a total of 90 seconds (60 seconds pre-ictal, 30 seconds ictal). RESULTS: We found an ictal tachycardia (heart rate increase > 10 bpm) in 82.5% of seizures, and an ictal bradycardia (heart rate decrease > 10 bpm) in 3.3% of seizures. Bradycardia was only observed in seizures of frontal lobe origin. Heart rate changes occurred several seconds prior to EEG seizure onset on scalp-EEG in 76.1% of seizures, but also prior to EEG seizure onset on invasive EEG in 45.7% of seizures. Early tachycardia occurred significantly more often in temporal than in frontal lobe origin seizures. We found no significant effect of the side of seizure onset on both the quality and quantity of ictal heart rate changes. The occurrence of an aura or of awakening prior to the seizure had no influence on peri-ictal heart rate changes. Low risk cardiac arrhythmias were more frequently observed in frontal lobe origin seizures. CONCLUSIONS: Epileptic discharges directly influence portions of the central autonomic network, within a brain area too small or too deep to be detected on EEG, most likely deep mesial structures such as the amygdala or portions of the hippocampus. The potential clinical applications of our results include (1) automatic seizure detection, (2) differentiation between seizures of temporal and frontal lobe origin, (3) detection of peri-ictal cardiac arrhythmias, and (4) clarification of SUDS (sudden unexplained death syndrome) in epilepsy.     

局部脑血流SPECT显像与其他影像对癫痫定位诊断比较的研究

目的:研究癫痫发作期与发作间期同侧对照99mTc-ECD局部脑血流(rCBF)单光子发射计算机体层摄影术(SPECT)显像对癫痫灶的定位诊断价值。方法:对54例癫痫患者行发作间期显像,其中10例行发作期显像,并与皮层脑电图(EEG)、计算机断层扫描或磁共振显像(CT/M RI)、深部皮层脑电图(EcoG)结果进行对比分析。结果:癫痫发作期与发作间期rCBF SPECT显像对癫痫灶具有较高的检出率和定位率,优于EEG、CT/M RI,低于EcoG,但rCBF在发作期与发作间期的比较差异无显著性(P>0.05)。结论:发作间期rCBF SPECT显像可以成为癫痫灶方便而有效的定位方法,若加上发作期rCBF显像则定位更佳。     

Cardiac vagal activation by adrenocorticotropic hormone treatment in infants with West syndrome

West syndrome (WS) is a generalized epileptic syndrome of infancy and early childhood with various etiologies, and consists of a triad of infantile spasm, arrest or regress of psychomotor development and specific electroencephalogram (EEG) pattern of hypsarrhythmia. WS had been believed to be refractory, but recent evidence supports effectiveness of adrenocorticotropic hormone (ACTH) treatment. The ACTH treatment, however, has a problem that it is often accompanied by adverse autonomic symptoms. We therefore examined heart rate variability (HRV) for assessing cardiac autonomic functions in WS and prospectively observed the changes during ACTH treatment. We studied 15 patients with WS and 9 age-matched controls during sleep (EEG stage 2). Compared with controls, the patients with WS were greater in the low-frequency component (LF) of HRV, an index reflecting sympatho-vagal interaction (p = 0.02), but were comparable for high-frequency component (HF) and LF-to-HF ratio (LF/HF), indices reflecting cardiac vagal activity and sympathetic predominance, respectively. During ACTH treatment, heart rate decreased (p < 0.01), LF and HF increased (p < 0.01), and LF/HF did not differ significantly. These results indicate that WS might be accompanied by autonomic changes and that ACTH treatment enhances parasympathetic function and causes bradycardia.     

Infantile Gaucher's disease.

This case report calls attention to the fact that infantile Gaucher's disease can occur in black infants. The infant's age, neurologic manifestations, and pathologic findings support the diagnosis.