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1.
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。  相似文献   

2.
目的:探讨结节性硬化症(TS)所致婴儿痉挛症患者发作期脑电活动局灶改变及其分布优势侧的特征现象,以及与术后疗效的关系.方法:回顾分析了31例TS术前脑电图定位在发作期呈现的局灶、一侧多灶,或双侧均有病灶而以一侧占优势的脑电图现象,并结合术后随访结果对比分析其差异.结果:局灶性放电(单灶)6例,一侧多灶14例,双侧改变以...  相似文献   

3.
86例癫痫痫灶定位与脑电图懒波   总被引:1,自引:0,他引:1  
目的探讨86例脑电图一侧懒波、另一侧痫样放电癫痫患者的病史特点及痫灶定位。方法回顾性总结分析86例一侧懒波、另一侧痫样放电癫痫患者的病史、脑电图特点,并结合影像学改变、临床发作及发作期偶极子定位系统密度压缩谱阵(DSA)分析进行讨论。结果蛛网膜囊肿、外伤后脑软化灶和脑萎缩所致懒波常见,分别为24.4%(21/86)、23.3%(20/86)和18.6%(16/86)。42例行术中皮质及深部脑电图探测,证实了术前定位。结论脑电图一侧懒波,另一侧痫样放电时,其懒波侧多为原发痫灶侧。存在此类脑电图特有表现的癫痫患者中,以继发性癫痫多见(94.2%,81/86),但少数病程较长的顽固性原发性癫痫也可出现类似表现(5.8%)。准确找出原发致痫灶并予以切除,可获得良好的治疗效果。  相似文献   

4.
目的分析伴有癫痫发作的结节性硬化症(TSC)患者的脑电图特点。方法总结101例有痫性发作的TSC患者的临床资料,对其发作间期及发作期脑电图进行分析。结果癫痫的发病年龄从10d~28岁不等,3岁以内发病者68人(67%)。101例患者均行24h视频脑电图监测,3例脑电图正常,98例脑电图异常,其中3例存在高幅失律,23例为慢波背景活动,72例背景活动正常或接近正常,但其中11例患者脑电图可见较多慢活动。在有痉挛发作的13例患者中,3例脑电图背景为高幅失律,7例为慢波背景活动。发作间期显示痫样波在双侧均明显者35例,一侧痫样波明显者63例,主要集中在前头部。48例患者监测到发作,监测到的发作最常见为部分性发作(27例),26例可以明确起源,主要集中在额颞叶。结论大多数TSC患者脑电图异常,可见痫样波发放,54%TSC患者通过视频脑电图可以明确痫样波起源,主要在额颞叶,左右两侧无明显差异。  相似文献   

5.
目的通过51例婴儿痉挛症的临床与录像脑电图分析,探讨同步录像脑电图(VEEG)监测对癫发作类型的诊断及鉴别诊断的临床价值。方法采用Nicolet录像脑电图仪,按照国际10/20系统安放电极,采用网帽固定,按时间常数0.3s,走纸速度3cm/s,常规单双导联描记。所记录的图像与脑电信号存储于计算机硬盘内,可在检查后随意调用回放分析。结果51例患儿脑电图均为重度异常,其中典型高度失律EEG及非典型高度失律EEG共40例(占总数的78.4%),其他形式的样放电11例(占总数的21.6%)。51例中同时伴有爆发抑制现象3例(占总数的5.9%)。结论VEEG监测可显著提高样放电的检出率,并有助于区别其他类型的癫发作。  相似文献   

6.
目的探讨长程视频脑电监测和偶极子分析在痫灶切除术中的定位价值。方法对35例开颅痫灶切除术患者术前行常规脑电和长程视频脑电监测,脑电痫性放电进行偶极子分析,并与手术中硬膜下电极记录的脑电相对照。结果35例患者视频脑电图监测异常35例,有痫性放电者35例;常规脑电图异常28例,有痫性放电者18例;行常规脑电图监测无典型癫痫发作出现。两者之间脑电异常率、痫性放电阳性率、典型临床发作率都有统计学意义。以术中硬膜下电极记录为标准,头皮脑电偶极子定位异常放电灶的准确率为97%,切除病灶病理检查异常率86%。偶极子定位误差一般<15mm。结论长程视频脑电监测和头皮脑电偶极子定位癫痫起源灶可为开颅痫灶切除手术提供有价值的信息。  相似文献   

7.
目的 探讨儿童结节性硬化症合并癫痫的临床特征。方法 对36例结节性硬化症合并癫痫患儿的临床资料进行分析。结果 所有患儿均以癫痫发作为首发症状,发病年龄〈1岁23例(63.89%)、1-3岁8例(22.22%)、〉3岁5例(13.89%);发作类型以部分性发作[12例(33.33%)]、全面性发作[5例(13.89%)]、痉挛发作[7例(19.44%)]和混合性发作[12例(33.33%)]为主,其中伴智力低下或发育落后者19例(67.86%)。MRI可见室管膜下结节(30例),部分病例[26例(86.67%)]伴皮质和皮质下结节、室管膜下巨细胞型星形细胞瘤2例(6.67%)或巨脑回畸形1例(3.33%)。脑电图显示发作间期样放电[34例(94.44%)],表现为多灶性放电[12例(35.29%)]、广泛性放电[8例(23.53%)]、局限性放电[7例(20.59%)]和高度失律[7例(20.59%)];其中8例监测到临床发作,包括痉挛发作(4例)、局灶性发作(3例)、肌阵挛发作(1例)。17例患儿(47.22%)服用1种抗癫痫药物、12例(33.33%)服用2种药物、7例(19.44%)服用3种药物,其中手术治疗1例;随访0.50~10年,无发作7例(19.44%),有效16例(44.44%),无效13例(36.11%)。结论 癫痫发作是儿童结节性硬化症最常见的神经系统表现,多在婴儿期发病,主要发作类型为部分性发作和痉挛发作,多数患儿抗癫痫药物治疗有效。  相似文献   

8.
研究背景婴儿痉挛症是一种以痉挛发作、脑电图高度失律和精神运动发育迟滞为特征的难治性癫癇,抗癫癇药物治疗效果欠佳。促肾上腺皮质激素作为一线治疗药物存在感染和高血压等不良反应,本研究旨在通过开放性临床试验评价硫酸镁联合促肾上腺皮质激素治疗婴儿痉挛症的疗效和耐受性。方法共计55例婴儿痉挛症患儿接受统一标准的硫酸镁联合促肾上腺皮质激素治疗,比较治疗前后痉挛发作频率和脑电图改变,治疗过程中通过监测患儿生命体征、实验室指标和药物不良事件评价药物耐受性。结果治疗2周后痉挛发作控制有效率为78.18%(43/55)、脑电图改善率为55.77%(29/52),其中无发作25例(45.45%)、脑电图恢复正常6例(11.54%)。不同发病年龄[≤4个月和〉4个月(χ2=0.595,P=0.441)]、病程[≤2个月和〉2个月(Fisher确切概率法:P=0.735)]和病因学[特发性或隐源性和症状性婴儿痉挛症(Fisher确切概率法:P=1.000)]对痉挛发作控制有效率,以及不同发病年龄(χ2=1.325,P=0.250)、病程(χ2=2.668,P=0.102)和病因学(χ2=2.215,P=0.145)对脑电图改善率之差异均无统计学意义;治疗期间无一例患儿发生高血压等不良事件。29例患儿经长期随访(治疗后1、3和12个月)无痉挛发作患儿比例基本维持不变(χ2=0.945,P=0.815)。结论硫酸镁联合促肾上腺皮质激素方案治疗婴儿痉挛症疗效肯定且耐受性良好。  相似文献   

9.
目的探讨儿童结节性硬化症合并癫的临床特征。方法对36例结节性硬化症合并癫患儿的临床资料进行分析。结果所有患儿均以癫发作为首发症状,发病年龄1岁23例(63.89%)、1~3岁8例(22.22%)、3岁5例(13.89%);发作类型以部分性发作[12例(33.33%)]、全面性发作[5例(13.89%)]、痉挛发作[7例(19.44%)]和混合性发作[12例(33.33%)]为主,其中伴智力低下或发育落后者19例(67.86%)。MRI可见室管膜下结节(30例),部分病例[26例(86.67%)]伴皮质和皮质下结节、室管膜下巨细胞型星形细胞瘤2例(6.67%)或巨脑回畸形1例(3.33%)。脑电图显示发作间期样放电[34例(94.44%)],表现为多灶性放电[12例(35.29%)]、广泛性放电[8例(23.53%)]、局限性放电[7例(20.59%)]和高度失律[7例(20.59%)];其中8例监测到临床发作,包括痉挛发作(4例)、局灶性发作(3例)、肌阵挛发作(1例)。17例患儿(47.22%)服用1种抗癫药物、12例(33.33%)服用2种药物、7例(19.44%)服用3种药物,其中手术治疗1例;随访0.50~10年,无发作7例(19.44%),有效16例(44.44%),无效13例(36.11%)。结论癫发作是儿童结节性硬化症最常见的神经系统表现,多在婴儿期发病,主要发作类型为部分性发作和痉挛发作,多数患儿抗癫药物治疗有效。  相似文献   

10.
目的探讨硬膜下电极脑电图(ECo G)监测对磁共振阴性癫痫患者致痫灶的定位作用。方法对经临床、影像学和头皮EEG检查不能确定致痫灶部位的6例难治性额叶癫痫患者,植入硬膜下条状电极进行视频EEG监测,观察颅内电极发作期及发作间期EEG变化,结合头皮EEG、临床发作结果对癫痫灶进行综合定位;术后随访,评估致痫灶定位的准确性。结果 6例患者颅内电极埋藏时间为2~5 d,每例监测到2次临床发作并记录发作间期和发作期的异常放电活动。5例患者发作期颅内电极EEG均能准确定位,5例显示一侧局灶性放电起源,1例患者显示双侧放电起源。术后按Engel疗效分级:EngelⅠ级4例(57.1%),EngelⅡ级1例(14.3%),随访不满1年的按谭氏术后效果分级,达到了满意。结论颅内电极EEG监测可为癫痫手术治疗提供可靠的病灶定位依据。  相似文献   

11.
目的 探讨结节性硬化所致婴儿痉挛症的手术适应证、方法和预后.方法 回顾性分析采用外科手术治疗17例结节性硬化症伴婴儿痉挛症的经验.结果 术后疗效按Engel分级,Ⅰ级11例,Ⅱ级4例,Ⅲ级2例.术前评估为单致痫灶的2例患者均为Ⅰ级.局限在一侧大脑半球的8例多灶性癫痫患者中,6例Ⅰ级,2例Ⅱ级.双侧大脑半球均有致痫灶但以一侧为主的7例患者中,3例Ⅰ级,2例Ⅱ级,2例Ⅲ级.平均智商(IQ)从术前的52.6分提高到61.8分.结论 结节性硬化所致婴儿痉挛症具有良好的外科预后,对部分药物难治性患者在评估确定责任结节后可以考虑进行外科干预.
Abstract:
Objective To investigate the surgical indications, methods and outcomes of infantile spasms with tuberous sclerosis(TS). Method Surgical treatment of 17 infantile spasms patients with TS was reviewed. Single epileptogenic tuber or lobe was resected when a focal epileptic discharge was indicated according to preoperative evaluation and EcoG, and multiple lobes or tubers resection were chosen to deal with hemisphere limited multiple epileptic foci. Anterior corpus callosotomy was added when contralateral hemisphere showed epileptic discharges. Results Acording to follow - up of 3 years in average after surgery, 11 patients had an Engel Class Ⅰ outcome, 4 patients had rare seizure ( Engel Class Ⅱ ), and 2 patients had a reduction in seizure frequency (Engel Class Ⅲ ). The mean IQ of patients was improved from 52.6 to 61. 8. Conclusions Epilepsy surgery in tuberous sclerosis with infantile spasms showes a favourable outcome,and surgical intervention should be considered in some intractable infantile spasms after corresponding tuber is derermined.  相似文献   

12.
Introduction: Duplications of 14q12 encompassing FOXG1 gene have been recently associated with developmental delay, severe speech impairment, epilepsy, aspecific neuroimaging findings and minor dysmorphisms. Aim and methods: In order to refine the epileptic phenotype associated with 14q12 duplications, we have performed a review of the electroclinical picture of the patients reported to date in the literature, adding a new personal case. A comprehensive set of clinical and instrumental data (with a particular focus on the electroclinical aspects including seizure type, age of onset, EEG at onset and after antiepileptic therapy, drug efficacy) has been taken into account. Results: 9/14 patients carrying 14q12 duplications developed seizures, all in the first months of life. Most of them developed infantile spasms (8/9 epileptic patients) and presented hypsarrhythmia or modified hypsarrhythmia on EEG. After therapy 5/9 patients became seizure free and 3/9 present a good seizure control. At last available follow up, 2/3 of the epileptic patients displayed an almost normal EEG, or a quite organized background activity, with diffuse or focal (mostly temporal) slowing. Conclusions: The review of the available data allowed to recognize a common epileptic core, characterized by early onset, age dependent epileptic encephalopathy with infantile spasms and typical, atypical or modified hypsarrhythmia. Antiepileptic therapy soon led to a good or complete control of seizures with a nearly normal background activity in most patients.  相似文献   

13.
Reevaluation of neonatal EEGs and polygraphic tracings of 40 infants with infantile spasms and/or hypsarrhythmia resulted in the constitution of a compound score for the identification of infants at risk for infantile spasms by neonatal EEG. The score comprises 8 distinct items: 2 concern behavioral characteristics, 6 abnormality of EEG background activity and paroxysmal events. A tracing registered at conceptional age 36 to 44 weeks (eventually up to 50 weeks) presenting at least 4 of these 8 items is scored positive for the risk of evolving hypsarrhythmia. In a prospective study the polygraphic tracings of 941 newborn infants were evaluated for risk: 18 infants suffering from perinatal distress and 7 newborns with malformations of the brain were scored positive and all 25 developed infantile spasms and/or hypsarrhythmia. One infant with later infantile spasms was missed by the scoring system. None of the remaining infants scored negative manifested infantile spasms. Thus, correct positive prognostication was 100% and false negative 0.1%. By conventional EEG 5 out of 8 patients with infantile spasms were correctly predicted. The high validity of the risk-score based on polygraphic tracing between conceptional age 36 to 44 weeks may allow pre-onset treatment preventing secondary mental deterioration due to hypsarrhythmia and infantile spasms.  相似文献   

14.
Summary: Progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO syndrome) is an apparently autosomal recessive disorder manifested by infantile spasms, severe hypotonia, and early arrest of psychomotor development. Subcutaneous edema in the limbs, typical facial features, and blindness with optic atrophy are also present. Neuropathologic and radiographic studies show progressive brain atrophy, which is accentuated infratentorially. We recorded 85 EEGs from 10 patients between the ages of 3 weeks and 12.7 years; follow-up ranged from 7 months to 12.1 years. The infantile spasms were preceded by other neurological symp- toms in all patients. Seven of nine patients showed focal or generalized epileptiform activity or abnormal EEG background. All patients developed hypsarrhythmia, first recorded between 3 and 11 months of age, that was resistant to therapy with ACTH and antiepileptic drugs. After the hypsarrhythmia disappeared, five patients showed slow spike-wave activity generally seen in the Lennox-Gastaut syndrome, and three patients showed background EEG abnormality with generalized or diffuse paroxysmal activity. There were no specific EEG features that could help in the diagnosis of PEHO. The PEHO syndrome should be borne in mind in the diagnostic work-up of patients with infantile spasms, so that potentially harmful treatment can be avoided, and the parents can be counseled about the inheritability of the disorder.  相似文献   

15.
目的探讨手术治疗结节性硬化症所致难治性癫痫的适应证、方法和预后。方法回顾性分析了我院从2002年1月至2006年6月间,采用外科手术治疗10例结节性硬化症伴难治性癫痫的经验。术前评估和术中脑电检查显示为局灶性改变者行致痫灶切除、脑叶切除或多软膜下横纤维切断(MST)。检查提示一侧半球为主多灶性或弥漫性改变者,术中行多脑叶切除为主的联合手术方式。结果本组随访1~4年,平均2.5年。10例患者中有6例获得I级(Engel分级),3例获得Ⅱ级,1例为III级。平均智商(IQ)从术前的59.6分提高到74.2分。本组3例患者出现暂时性的并发症,无手术死亡。结论外科手术是治疗结节性硬化症所致难治性癫痫的良好手段。表现为单致痫灶的患者预后较佳,虽有多结节,但临床资料、脑电图、影像学资料吻合的多致痫灶亦具有较好的手术效果。  相似文献   

16.
Infantile spasms   总被引:12,自引:0,他引:12  
  相似文献   

17.
The purpose of this study was to investigate early electroclinical manifestations and evaluate treatment responses by video-EEG in infants with newly diagnosed spasms. Spasms were recorded in 44 infants (27 males, 17 females) before adequate treatment. Mean ages at onset of spasms and at first video-EEG were 5.3 months (range 0.9 to 9 months) and 5.9 months (range 2.4 to 11.5 months) respectively. Thirteen infants had cryptogenic and 31 had symptomatic aetiology. First treatment was vigabatrin in 36 infants. All infants were followed until 12 months of age or death. Treatment response in the first months of therapy was assessed by repeated video-EEG studies in 23 infants. On the first video-EEG, 34 infants had typical symmetric motor spasms, three infants showed asymmetric or asynchronous behaviour, and seven infants had only subtle spasms. Interictal EEG showed hypsarrhythmia in 27 infants and multifocal spikes with normal or nearly normal background in 17 infants. Subtle spasms, asymmetric or asynchronous spasms, and asymmetric ictal or interictal EEG abnormalities were associated with symptomatic aetiology and poor cognitive and seizure outcome at 12 months. Serial video-EEG recordings showed a transition from motor to subtle spasms during the first 2 weeks of vigabatrin therapy in four infants and only subtle spasms in two therapy-resistant infants at 12 months. Cessation of spasms usually preceded disappearance of hypsarrhythmia or multifocal spikes, but persistence of multifocal spikes over several weeks was always associated with existing spasms. Transition of hypsarrhythmia into multifocal spikes was observed during vigabatrin therapy even in infants with intractable spasms. Initial diagnosis of infantile spasms requires video-EEG studies especially in infants with symptomatic aetiology who may show only subtle spasms. Video-EEG is the only reliable method for assessing treatment response as spasms and interictal EEG abnormalities are modified by treatment and may become subtle.  相似文献   

18.
Early infantile epileptic encephalopathy, or Ohtahara syndrome, is characterized by tonic spasms and a suppression-burst pattern on the electroencephalography (EEG). The EEG demonstrates a suppression-burst pattern during waking and sleeping states that often evolves into hypsarrhythmia and followed later by a diffuse slow spike-wave pattern. In other patients, the EEG evolves into focal spike discharges or multiple independent spike foci. We report a 5-year-old girl with Ohtahara syndrome that persistently demonstrated tonic spasms and suppression-burst on multiple EEGs. Over her lifetime, neither hypsarrhythmia nor diffuse slow spike-wave pattern were seen. This suggests that in Ohtahara syndrome, a suppression-burst pattern can persist over a long period of time.  相似文献   

19.
Unusual variants of infantile spasms   总被引:1,自引:0,他引:1  
During evaluation of video-electroencephalograms (EEGs) performed in our laboratory, we identified 11 patients who had unusual repetitive movements that appeared to be variants of infantile spasms. Movements included yawning, facial grimacing, eye movements, and transient focal motor activity. These symptoms coincided with generalized attenuation, slow-wave transients, or other EEG ictal changes characteristic of infantile spasms. The background EEGs showed true or modified hypsarrhythmia. This series of patients shows that infantile spasms may be extremely subtle and clinically atypical. Patients who have these variants may or may not also have typical infantile spasms. In some patients, the seizures appear to be time-related or medication-induced modifications of more typical infantile spasms.  相似文献   

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