脑性瘫痪伴临床下癫癎样放电临床特征分析

目的 探讨脑性瘫痪患儿伴发临床下癫癎样放电的临床特征及相关危险因素.方法 对2004年1月-2007年12月期间在青岛市残疾儿童医疗康复中心治疗的279例脑瘫患儿进行详细的病史采集、脑电图及影像学检查、认知功能评定,分析不同类型脑瘫患儿临床下癫癎样放电的伴发率及腩电图表现,采用Logistic逐步回归分析显示认知水平、影像学改变、新生儿期惊厥史、出生胎龄及合并症等因素,对脑瘫伴发临床下癫癎样放电的影响.结果 18.64%(52/279)的脑瘫患儿伴发了临床下癫癎样放电,痉挛型偏瘫患儿的发生率最高(36.36%),四肢瘫患儿最低(6.25%).所有脑瘫类型均以局灶性或多灶性放电占多数.不同DQ/IQ水平局灶性与全面性放电的比例差异无统计学意义.皮层损伤及存在合并症是脑瘫伴发临床下癎样放电的危险因素,而认知水平、新生儿期惊厥史及出生胎龄与之无明显关系.结论 脑性瘫痪常伴发临床下癫癎样放电,尤其见于痉挛型偏瘫,以局灶性或多灶性放电多见.皮层受损及存在合并症对脑瘫伴发临床下癎样放电有预测价值.     

脑性瘫痪伴临床下癫痫样放电临床特征分析

目的探讨脑性瘫痪患儿伴发临床下癫痫样放电的临床特征及相关危险因素。方法对2004年1月-2007年12月期间在青岛市残疾儿童医疗康复中心治疗的279例脑瘫患儿进行详细的病史采集、脑电图及影像学检查、认知功能评定,分析不同类型脑瘫患儿临床下癫疴样放电的伴发率及脑电图表现,采用Logistic逐步回归分析显示认知水平、影像学改变、新生儿期惊厥史、出生胎龄及合并症等因素,对脑瘫伴发临床下癫痫样放电的影响。结果18．64％（52／279）的脑瘫患儿伴发了临床下癫疴样放电,痉挛型偏瘫患儿的发生率最高（36．36％）,四肢瘫患儿最低（6．25％）。所有脑瘫类型均以局灶性或多灶性放电占多数。不同DQ／IQ水平局灶性与全面性放电的比例差异无统计学意义。皮层损伤及存在合并症是脑瘫伴发临床下疴样放电的危险因素,而认知水平、新生儿期惊厥史及出生胎龄与之无明显关系。结论脑性瘫痪常伴发临床下癫痫样放电,尤其见于痉挛型偏瘫,以局灶性或多灶性放电多见。皮层受损及存在合并症对脑瘫伴发临床下疴样放电有预测价值。     

痉挛型偏瘫脑电图癫癎样放电危险因素分析

目的探讨痉挛型偏瘫的临床表现及脑电图发作间期癫癎样放电(IED)特征,并分析IED相关危险因素。方法收集83例痉挛型偏瘫患儿的临床资料、视频脑电图、影像学检查及认知功能评定结果等。采用多因素logistic回归分析IED的影响因素。结果 13%痉挛型偏瘫患儿伴有癫癎。34%患儿伴IED,IED组癫癎发生率(32%)较非IED组者(4%)显著升高(PPPP结论痉挛型偏瘫常伴IED,存在IED者癫癎发生率更高。皮层受累及认知水平低下对痉挛型偏瘫伴IED有预测价值。     

脑瘫共患病及其与脑瘫类型和粗大运动功能分级的关系

目的 探讨脑性瘫痪(简称脑瘫)患儿常见共患病及其与脑瘫类型和粗大运动功能分级水平的关系.方法 对2007年1月至2009年6月在我院脑瘫康复中心住院治疗的脑瘫患儿进行智力测查、眼科检查、语言测查、听觉诱发电位、脑电图及粗大运动功能分级评估等,从临床分型和粗大运动功能分级两个角度分析脑瘫共患疾病.结果 354例脑瘫患儿中,共患智力低下166例(46.89%)、听觉障碍15例(4.24%)、视觉障碍138例(38.98%)、语言-言语障碍216例(61.02%)、癫癎82例(23.16%).相关分析显示,痉挛型双瘫与视觉障碍相关性最强,痉挛型偏瘫与癫癎相关性最强,痉挛型四肢瘫与癫癎和智力低下最相关,不随意运动型及混合型均与语言障碍最相关.除视觉障碍在不同GMFCS分级间的分布差异无统计学意义(χ~2=1.90,P>0.05)外,其他共患病的发生情况与脑瘫类型、GMFCS分级水平明显相关(P均<0.05),四肢瘫、不随意运动型和混合型脑瘫患儿及GMFCS分级水平Ⅳ～Ⅴ级者多重障碍率明显高于痉挛型双瘫、偏瘫患儿及GMFCS分级水平Ⅰ～Ⅲ级者(P<0.05).结论 脑瘫患儿共患病的发生率和病种与脑瘫类型以及GMFCS分级水平有关,建议临床医生充分认识脑瘫患儿的临床分型、运动水平以及共患病,多学科协作全面评价和康复,以改善不良预后.     

儿童睡眠中癫癎性电持续状态的相关因素分析

目的：儿童睡眠中癫癎性电持续状态（ESES）的病因不明，该研究旨在分析ESES的相关因素，为有效预防和治疗此病提供依据。方法：以唐山市妇幼保健院2000年1月至2006年7月确诊为ESES的30例患儿为研究对象，选择与ESES组相匹配的非ESES癫癎组30例为对照组，设计问卷调查表获取资料。对癫癎发作类型、癫癎样放电起源部位、家族史及围生期情况、影像学、智商、癫癎综合征等多项因素采用χ2检验及t检验进行分析。结果：ESES组与对照组在癫癎家族史、智力低下及癫癎综合征、语言障碍之间比较差异有显著性（P<0.05）。癫癎发作类型、癫癎样放电起源部位、父母育龄、患儿出生史、热性惊厥家族史、影像学异常两组间差异均无显著性意义（P＞0.05）。既往患热性惊厥、脑瘫、颅内感染两组间比较，差异亦无显著性意义（P＞0.05）。结论：ESES与癫癎家族史、癫癎综合征、智力水平及语言障碍相关。［中国当代儿科杂志，2009，11（2）：110-112］     

癫(癎)患儿预后的危险因素

儿童期癫癎患儿的预后与成年人不同,该文从一般因素、临床特征、辅助检查及药物治疗等方面复习国外近年文献,寻找儿童癫癎预后的危险因素。认为发病年龄大、病因明确、有癫癎持续状态、初期发作频率高、治疗前发作次数多、症状性癫癎综合征、有癫癎家族史、热性惊厥史、有神经系统体征、神经精神缺陷、脑电图异常、影像学异常、未正规应用抗癫癎药和对首次用药反应差的患儿预后不佳。     

伴有非癫癎发作的322例脑性瘫痪临床分析

目的：了解伴有非癫癎发作的脑性瘫痪（脑瘫）的临床特点,以提高与癫癎发作的鉴别能力。方法：1 198例9个月至6岁的脑瘫患儿纳入研究,对伴随发作性症状的患儿进行24 h视频脑电图监测,明确伴随的发作是否为癫癎发作,并观察伴随非癫癎发作患儿的发作症状、年龄、脑瘫型别及脑电图特征。结果：共578例（48.24%）患儿伴随发作性症状,其中伴随癫癎发作者231例（19.28%）,非癫癎发作322例（26.88%）。322例伴随非癫癎发作的脑瘫患儿中,发作性症状包括非癫癎性强直发作、发作性摇头、耸肩或头后仰、发作性哭闹、惊恐发作、睡眠肌阵挛、刻板性运动等;158例（49.1%）显示脑电图有非特异性异常;111例（34.5%）在基层医院误诊为癫癎;1岁以内患儿非癫癎发作频率高于1岁~和3~6岁组患儿;痉挛型脑瘫患儿非癫癎发作频率最高（168例,52.2%）,其次为不随意运动型（69例,21.4%）和混合型(65例,20.2%)。结论：脑瘫患儿伴随的发作性症状部分为非癫癎发作,注意与癫癎发作相鉴别。非癫癎发作发生频率与患儿年龄及脑瘫型别有一定关系。［中国当代儿科杂志,2010,12(12)：933-935］     

伴高热惊厥史的儿童癫痌病例分析

分析伴高热惊厥史的癫痌患儿的临床特点,探讨高热惊厥脑损伤及其与颞叶癫痌的关系。 方法对1996～1999年本院儿科神经病房480例住院癫痌患儿进行回顾性分析,包括首发年龄、家族史、持续时 间、癫痌发作类型、神经影像学及脑电图改变等。结果115例(23.9％)患儿有前期高热惊厥史。伴高热惊厥史 的患儿癫痌发作早且易于出现癫痌持续状态。与无高热惊厥史的患儿相比,伴高热惊厥史的患儿强直-阵挛发作 较多,复杂部分性发作较少。408例患儿曾行影像学检查,4例提示有海马硬化者均无高热惊厥史。在伴高热惊厥史 的癫痌患儿中脑电图局灶起源的异常放电显著低于无高热惊厥史的癫痌患儿。有6.08％(7/115)伴高热惊厥史的癫 痌惠儿和6.84％(25/365)无高热惊厥史的癫痌患儿脑电图表现为单纯颞叶异常放电,二组相比无明显差异。结论 在癫痌患儿中,高热惊厥可能伴有脑损伤,且可能与后期的癫痌发生有关,伴高热惊厥史者不一定发展为颞叶癫痌。     

皖南山区家族性全面性强直-阵挛性发作患儿智商及影响因素

目的 分析家族性全面性强直-阵挛性发作(GTCS)患儿和他们同胞的智商(IQ),探讨影响癫(癎)患儿IQ的相关因素.方法 选择皖南山区20个癫(癎)家系中的9～16岁的GTCS患儿28例作为试验组,随机抽选28例与患儿年龄差距最小同胞儿童为对照1组,30例同村的年龄、性别匹配相近的健康儿童为对照2组,比较3组儿童的IQ水平;并记录癫(癎)发病的相关资料和影响儿童IQ的因素.结果 1.试验组32%儿童IQ在平均水平,21%高于平均水平;与对照1组及对照2组比较均有显著差异(Pa <0.01);2.试验组儿童的总智商较对照1组、对照2组均显著降低(Pa <0.001),除了填图与图片排列外,试验组所有其他分测验得分和2个对照组之间均有显著性差异;3.2个对照组之间的IQ水平及各分测验得分无显著性差异;4.癫(癎)患儿IQ与父亲受教育程度、癫(癎)发病年龄、癫(癎)发作病程、癫(癎)发作频率、癫(癎)持续状态显著相关(r=0.453,0.506,-0.533,-0.708,-0.732 Pa <0.05);5.多元线性逐步回归分析,父亲受教育程度与癫(癎)发作病程是影响癫(癎)患儿IQ的主要因素.结论 家族性GTCS患儿的IQ及各分测验得分明显低于健康儿童;其同胞的IQ水平与健康儿童无统计学差异;癫(癎)发作病程、父亲受教育程度是影响患儿IQ最重要的癫(癎)因素,其他依次为:癫(癎)持续状念、起病年龄、癫(癎)发作次数、癫(癎)发作频率.     

偏侧惊厥-偏瘫-癫(间)综合征的临床特点及诊断

目的探讨偏侧惊厥-偏瘫-癫癎(HHE)综合征的临床特点及诊断。方法对5例HHE综合征患儿的临床资料进行回顾性分析,总结临床特征及其发病的危险因素,进行必要的辅助检查,包括头颅CT和(或)MRI、脑电图及诊断性智力测定或精神运动发育评价。结果4例4岁内起病,3例伴热性惊厥,最长惊厥时间均在2 h以上,并惊厥侧肢体偏瘫。5例均在偏瘫后2年内出现癫癎反复发作。4例为局限性运动发作,1例为精神运动性发作。5例均存在智力障碍或精神发育迟滞。头颅MRI检查4例发现左侧海马硬化,1例CT示右半球萎缩。脑电图均异常,4例见异常放电,1例示明显不对称。5例均予卡马西平为主的药物治疗,癫癎发作得到控制或部分控制。结论HHE综合征是持续偏侧惊厥导致的偏瘫-癫癎综合征,海马硬化可能是反复癫癎所致海马的继发性损伤,而非癫癎的起源灶。应提高对该病的认识,早期正确处理惊厥持续状态将减少HHE综合征发生。     

脑性瘫痪儿童的MRI特征

目的 探讨脑性瘫痪(脑瘫)患儿脑MR／的表现及其与出生胎龄和脑瘫类型的关系。方法 回顾性分析104例脑瘫患儿的病史、l临床与MR／表现。结果 早产与足月儿脑瘫类型构成显著不同,早产儿以痉挛性双瘫多见(占66．O％),而足月儿偏瘫和失调型高于早产儿。104例脑瘫患儿MR／异常率为84．7％,早产和足月儿组MHI异常率差异无显著性。痉挛型双瘫、四肢瘫、偏瘫、手足徐动型和失调型脑瘫MR／异常率分别为89．4％、100％、100％、54．5％和90．O％。31／42例痉挛性双瘫表现为脑室周围白质软化症(PVL),而以早产儿双瘫更多见(90％);各类型脑瘫的MR／异常表现不同,双瘫以PVL为主,徐动型表现为基底节病变或．PVL失调型绝大部分存在先天性小脑发育不全,偏瘫型突出表现为单侧脑损伤。出生胎龄与MRI特点有关,早产儿组以PVL为特征,见于除失调型外的其他脑瘫类型;足月儿脑瘫MR／异常表现变化多且病变广泛。结论MR／有助于评价各型脑瘫的病理特点及其与出生胎龄的关系．对脑瘫病因的推测有帮助。     

Changing Panorama of Cerebral Palsy in Children of Low Birth Weight

Of 2,840 out-patients who visited the Tokyo Metropolitan Kita Rehabilitation Center in 1965, 1968, 1970, 1973, 1975 and 1978, 278 children with cerebral palsy (CP) of low birth weight were studied to analyse changes in type of CP in relation to gestational week, birth weight, perinatal factors and subsequent complications. 1. The incidence of handicap associated with low birth weight in new out-patients had not changed. 2. The number of doubly handicapped CP patients was gradually increasing while number of uncomplicated CP patients was gradually decreasing. 3. The number of children with tension athetosis was remarkably decreasing; the number of children with spastic quadriplegia was gradually increasing. Increase in number of the spastic type was found especially in the group whose birth weight was over 1,500 gm. and gestational week over 37 weeks. The numbers of spastic diplegia and hemiplegia patients had not significantly changed. 4. In the athetoid group, the complication of severe jaundice was apparently decreasing since 1970 while the number of asphyxia cases was gradually increasing. In the spastic group, there were no remarkable changes in the perinatal complications. 5. Of spastic quadriplegia patients 78.3% were of IQ of less than 67 while 33.3% of spastic hemiplegia and 23.5% of spastic diplegia patients had IQs of less than 67.31.4% of the athetoid type had IQs of less than 67. 6. In the asphyxia group, 34 of 82 children with low birth weight CP (41.5%) had epilepsy. Our results suggest that the causes of brain damage in low birth weight infants are not merely preventable perinatal factors, but are due to a more complex interaction of prenatal and perinatal factors.     

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目的探讨合并癫疒间的脑性瘫痪患儿的临床特点及癫疒间治疗效果。方法总结1999年10月至2004年5月在西安交通大学第二医院住院的合并癫疒间的脑性瘫痪患儿的癫疒间发生率、临床类型、头颅计算机体层成像(CT)和(或)磁共振成像(MRI)、脑电图等方面的特点以及抗癫疒间治疗效果。结果139例脑性瘫痪患儿中有32例合并癫疒间,占23.0%(32/139);常见的癫疒间类型为强直阵挛发作12例(37.5%)和部分性发作6例(18.8%);25例(78.1%)癫疒间首发于1岁前;合并癫疒间脑性瘫痪儿童的头颅影像异常及脑电图异常分别占30例(93.8%)和27例(84.4%);常见的头颅影像异常为脑发育不良11例(36.7%)、脑积水6例(20.0%),余为脑萎缩、缺氧缺血性脑病样改变等13例(43.3%);脑电图异常中局灶性和弥漫性所占比例分别为48.2%(13/27)和51.8%(14/27);癫疒间多发生于痉挛性脑性瘫痪中(68.8%);81.3%(26/32)的癫疒间需要2种及2种以上的抗癫疒间药物治疗。结论脑性瘫痪患儿中癫疒间的发生率较高,其头颅影像学和脑电图分别以脑发育不良和弥漫性背景活动异常伴疒样波发放为主;脑性瘫痪儿童的癫疒大多为难治性,需要联合用药治疗。     

Epilepsy in a representative series of Rett syndrome

In a representative series of 53 females with Rett syndrome (RS), aged 5-55 y, a history of epilepsy was present in 50 (94%), 45 of whom had 5-y active epilepsy. Compared with severe mental retardation in general, the median age of seizure onset was significantly later (4 vs 0.8 y) and partial complex seizures were more frequent (54% vs 23%). Neonatal seizures had occurred in only one and infantile spasms in none compared with 26% and 12%. After teenage, the severity of epilepsy tended to decrease, i.e. lower seizure frequency and relatively more partial seizures. The rate of being seizure-free for 1 y was 8% after 10 y and 40% after 27 y of epilepsy duration. Frequent seizures were associated with smaller head circumference. Conclusion: This epilepsy profile could fit in with present-day knowledge of RS as a form of dendrito-synaptogenic developmental failure with mainly late postnatal consequences, as well as being a relatively stationary condition in adulthood.     

Seizures and EEG patterns in Pallister–Killian syndrome: 13 New Italian patients

Background and objectivesPallister–Killian syndrome (PKS) is a rare genetic disorder caused by a tissue-limited mosaic supernumerary isochromosome 12p. Typical facial dysmorphisms, pigmentary abnormalities, and some major malformations are frequently present. Neurological manifestations include mental retardation, hypotonia, and seizures. Epilepsy incidence ranged from 39 to 59% in a previously reported series. No specific clinical and EEG phenotype has ever been reported to describe seizure features, electroclinical patterns, and response to therapy in PKS.MethodsThis was a multicentre study conducted on 13 Italian children with PKS, as diagnosed by clinical phenotype and confirmed in cultured fibroblasts. All patients underwent several polygraphic video-EEG recordings and brain magnetic resonance imaging.Results and conclusionsAll the patients presented with epilepsy and seizures that started at a mean age of 19 months. In six cases, epilepsy started with epileptic spasms (ES) combined with focal seizures in another case. In four cases, seizures were focal, and this was followed by ES in two patients. In only two cases, epilepsy started with myoclonic seizures, and spasms were never observed. The study provides further evidence that epilepsy is a part of the phenotype of PKS, although a specific clinical and EEG pattern could not be identified. Our cases show how ES with late- or first-year onset is the most common type of seizure. Despite a variable prognosis in terms of response to therapy, a significant proportion of patients achieved good seizure control.     

Utility of brainstem auditory evoked potentials in children with spastic cerebral palsy

In a retrospective study of 75 children with spastic cerebral palsy (CP), brainstem auditory evoked potentials (BAEP) were recorded and subsequently correlated with birthweight, gestational age, aetiology and type of CP, neuroradiological findings, additional impairments and disabilities (including the inability to walk independently). Seventeen patients (22.7%) had abnormal BAEP recordings. Thirteen of these 17 patients (76.5%) had spastic tetraplegia, 16 patients (94.1%) were full-term infants, 12 patients (70.6%) had myoskeletal problems, 9 (52.9%) had epilepsy, 16 (94.1%) had visual impairment, 13 patients (76.5%) were unable to walk independently, while all 17 patients (100%) had speech impairment and mental retardation. The aetiology of CP was prenatal in 2 of these 17 patients (11.8%) and perinatal in 15 patients (88.2%). Thirteen patients (76.5%) had cortical atrophy determined by either computed tomography or magnetic resonance imaging, two patients (11.8%) had an infarct picture and two patients (11.8%) had maldevelopment of the central nervous system. There was a definite statistically significant association between abnormal BAEP recordings and full-term delivery, perinatal aetiology of CP, spastic tetraplegia, speech, visual and myoskeletal impairments, epilepsy, mental retardation, inability to walk independently and cortical atrophy on neuroimaging (p < 0.001). We conclude that abnormal BAEP recordings in children with spastic CP are indicative of poor prognosis and associated with a "multihandicap state". BAEP testing should be incorporated into the diagnostic plan of all children with spastic CP newly referred to neurodevelopmental centres.     

Febrile seizures

Febrile seizures (FS) are the most common seizure disorder in childhood, affecting 2–5% of children between the ages of 3 and 60 months. Differentiation of FS from acute symptomatic seizures secondary to central nervous system infection is essential. Those with a focal onset, prolonged duration or which occur more than once within the same febrile illness are considered complex and have an increase in risk of subsequent epilepsy development. The vast majority of febrile convulsions are simple, lasting only a few minutes and without need of drug intervention. They have an excellent outcome with no increased risk of decline in IQ, subsequent epilepsy or increased mortality. Febrile seizure can recur, and as it often is a frightening and anxiety-provoking event for parents and caregivers, an understanding of the natural history and prognosis should enable the physician to reassure the parents providing an appropriate counselling and reassurance.
Conclusion: Febrile seizure can recur, and as it often is a frightening and anxiety-provoking event for parent and caregivers. An understanding of the natural history and prognosis should enable the physician to reassure the parents providing an appropriate counselling and reassurance.