伴基底节钙化的婴幼儿外伤后腔隙性脑梗塞

目的 探讨伴有基底节钙化的婴幼儿外伤后腔隙性脑梗塞的发病机理和治疗效果。方法 总结20例婴幼儿较轻微头颅外伤后发生脑梗塞的症状、影像学表现、治疗效果及预后。结果 婴幼儿外伤后脑梗塞均发生于一侧基底节区。临床表现为不同程度的一侧急性偏瘫、无意识改变及颅内高压症状。CT扫描发现双侧基底节区存在细小点状钙化,腔隙性脑梗塞灶毗邻钙化点,经治疗预后好,随访3个月全部康复。结论 婴幼儿外伤后腔隙性脑梗塞的发生与基底节钙化关系密切,但发病机理及基底节钙化的生理过程尚有待探究,本病诊断需与脑弓形虫病及巨细胞包涵体等相鉴别。     

小儿急性偏瘫综合征40例临床分析

为探讨小儿急性偏瘫综合征(AHS)的病因，并对该病的实验室检查、诊断和治疗作一评价，对40例AHS患儿的临床资料、实验室检查及治疗结果进行分析。结果：40例患儿中，非特异性脑血管缺血性病变及颅脑外伤为主要病因；磁共振成像(MRI)及磁共振血管成像(MRA)检查在AHS诊断方面提供重要信息，有指征者可行数字减影血管造影(DSA)检查；采用综合治疗方法，预后良好。提示MRI及MRA是AHS重要的诊断技术之一，脑血管造影是脑血管病变确诊的主要方法；改善微循环、扩张血管药物及高压氧的使用等综合治疗是AHS治疗所需。     

小儿交替性偏瘫1例

患儿，男，18个月。主因反复发作交替性偏瘫14个月就医。患儿于4个月时起出现交替性左右侧肢体偏瘫，每次发病之前多有癫痫发作，表现为阵挛性惊厥，意识不清。多于清醒或活动后发病，深睡后消失。发病时偏瘫侧肢体软弱、肌张力低，同时多汗。平时生长发育正常，14个月会行走，但不稳，可开始叫爸、妈等单字，听力正常。每次偏瘫发作1天至数天自然缓解，数日至10余日后对侧出现类似发作。患儿父母无头痛病史，家族中无类似病史。多次查头颅CT未见异常。曾给予抗癫痫药治疗无效。     

13例儿童交替性偏瘫的临床特征

目的 总结儿童交替性偏瘫(AHC)的临床特征。方法 对13例AHC患儿的临床与实验室资料进行分析。13例均做头颅MRI、脑电图(EEG)检查,血氨基酸、尿有机酸分析,血乳酸、丙酮酸测定。部分患儿做头颅磁共振血管成像(MRA)、数字减影动脉造影(DSA)及录像脑电图(VEEG)监测。结果 13例中男12例,女1例。发病年龄2d～55个月,平均13．1个月。起病症状表现为眼的运动异常(包括斜视、双眼向上或向一侧凝视、眼震)2例,眼的运动异常伴肌张力不全姿势9例,偏瘫2例。本组均有反复的偏瘫发作,每次发作持续时间数分钟～10d。发作频率从每天8次到每两月1次。10例在偏瘫发作时反复出现眼的运动异常或肌张力不全姿势,伴舞蹈手足徐动2例,共济失调1例。7例曾有四肢瘫的病史,在四肢瘫发作时伴构音障碍或失语4例,伴呼吸急促和吞咽困难各2例。无力和上述发作性症状在睡眠时减轻。有智力障碍者9例,惊厥3例。除3例有EEG或VEEG异常外,其余实验室检查均正常。12例用氟桂利嗪治疗的患儿中,有8例偏瘫发作严重程度、持续时间和发作频率有降低。结论 AHC是一种以频繁发作的交替性偏瘫伴锥体外系症状和智力低下为临床特征的疾病,氟桂利嗪治疗对部分病例有效。     

首次热性惊厥患儿的复发率及相关因素研究

目的研究首次热性惊厥(FC)患儿106例的复发情况及可能引起复发的11种相关因素。探讨有效的防治措施，从而减少FC的复发。方法随机选择首次FC发作就诊的患儿106例，对其进行1．5～5．5年随访，随访率为95．1％，对可能影响复发的11种因素进行多元回归分析。结果首次FC的复发率为6l％，与患儿首次发病年龄，首次发病热度呈负相关；与热性惊厥家族史及患儿营养不良、反复呼吸道感染呈正相关；与患儿性别、贫血、佝偻病、围生期异常、喂养方式无相关。结论由于FC可造成惊厥性脑损伤，减少或控制FC的复发，可预防或治疗引起FC复发的上述5种因素着手，减轻或防止患儿智能损害。     

小儿急性偏瘫综合征16例诊断与治疗探讨

目的探讨小儿急性偏瘫综合征(AHS)的诊断与治疗。方法对16例AHS临床资料，结合实验室检查进行分析，特别是与磁共振血管造影(MRA)检查对比；均采取针对病因及抗凝和扩血管治疗。结果16例AHS病例中12例(75％)发现脑血管异常改变(大脑底部血管网l例、基底节梗死灶4例、大脑中动脉分支狭窄或闭塞7例)；采用综合治疗方法，预后良好。结论MRA是AHS重要的诊断技术；早期抗凝和扩血管药物的使用是AHS治疗所需。     

小儿急性偏瘫综合征的病因探讨及治疗体会

目的 探讨小儿急性偏瘫综合征（AHS）的病因及治疗方法。方法 对40例小儿急性偏瘫综合征患儿的临床表现、实验室检查及影像学检查结果进行综合分析。结果 40例中有13例为颅内感染伴有偏瘫，9例为脑梗死致偏瘫（6例有脑外伤病史），3例为先天性脑血管畸形突发脑出血，3例为晚发性维生素K缺乏致脑出血，3例为颅内占位性病变。2例烟雾病，2例为癫痫持续状态后脑损伤，1例为线粒体脑肌病．乳酸酸中毒．卒中样发作，1例脑面血管瘤病，1例神经纤维瘤病，1例风湿性心脏病引起脑栓塞致偏瘫，1例为白血病。采用综合治疗方法，预后良好。结论 感染是小儿急性偏瘫综合征最常见的病因，脑血管病变引起偏瘫也不容忽视。结合临床进行脑脊液化验是颅内感染伴有偏瘫的重要诊断方法，头部CT或MRI。磁共振血管造影是脑血管病变确诊的主要方法。     

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。     

儿童动脉缺血性卒中的病因

目的总结儿童动脉缺血性卒中(AIS)的临床特点、病因及危险因素。方法回顾性分析2003年1月-2011年3月在重庆医科大学附属儿童医院住院治疗的AIS患儿资料,采用SPSS 17.0软件进行分析,描述患儿年龄、性别分布等基本情况、相关病因及危险因素,并分析头部轻微外伤与基底核钙化是否存在相关性。结果本组患儿中,男31例,女27例;年龄2个月～13岁,平均3岁10个月。常见神经系统表现依次为肢体偏瘫52例(92.8%),中枢性面瘫34例(58.6%),运动性失语16例(27.6%),头痛、呕吐11例(19.0%),意识障碍8例(13.8%),惊厥7例(12.1%)。40例进行血管影像学检查,其中33例异常,左侧大脑中动脉受累最常见(19例,47.5%)。常见病因为轻微头部外伤(26例,44.8%)、烟雾病(8例,13.8%)和颅内感染(5例,8.6%),18例(31.0%)患儿为多病因共存,6例(10.3%)患儿未找到任何病因或危险因素,22例(37.9%)患儿有呼吸道感染。结论儿童AIS发病高峰人群为幼儿及学龄前期儿童,最常见的神经功能障碍为肢体偏瘫,神经影像学显示左侧大脑中动脉狭窄或中断最常见,常见病因为轻微头部外伤、烟雾病及颅内感染,较多患儿病前或病程中有上呼吸道感染。积极预防感染、减少头部外伤,对突发偏瘫患儿早期行头颅影像和血管检查将有利于AIS的预防和早期诊断。     

癫和热性惊厥患儿血清及脑脊液神经元特异性烯醇化酶测定的临床意义

目的探讨癫(EP)和热性惊厥(FC)患儿血清和脑脊液神经元特异性烯醇化酶(NSE)的含量及其与脑损伤的关系。方法采用电化学发光法测定EP和FC患儿惊厥发作后24h内血清和脑脊液NSE的水平。结果EP组和FC组惊厥发作后24h内血清和脑脊液NSE水平均明显高于对照组(P<0.05) ;惊厥严重组 (24h内发作≥3次和/或单次发作时间≥15min)EP患儿血清和脑脊液NSE水平也明显高于较轻组 (24h内发作<3次及单次发作时间<15min) ,P<0.05 ;FC严重组患儿血清和脑脊液NSE水平也明显高于较轻组(P<0.05)。结论EP和FC发作可引起血清和脑脊液NSE水平的升高 ,NSE水平与惊厥发作的严重程度有关 ;提示EP和FC发作可导致神经元损伤 ,频繁发作或长时间持续发作神经元损伤更严重。     

Neuroimaging findings (ultrasonography,CT, MRI) in 3 infants with congenital rubella syndrome

Neuroimaging observations of three infants with congenital rubella syndrome are reported. We have observed congenital rubella syndrome lesions in the subependymal area, the basal ganglia and the deep white matter. Cranial ultrasonography defines subependymal cysts, calcification and possible vascular changes in the basal ganglia while MRI is the most sensitive to minor atrophic changes and white matter lesions. Although CT defines calcification, it is less sensitive than MRI to white matter changes and does not demonstrate subependymal cysts.     

Cerebral infarction after mild head trauma in children

We conducted this retrospective, case record review to determine the risk factors and clinical features associated with cerebral infarction after mild head trauma in children. The median age of the cohort was 2.18 years (range, 6 mo–8 y). Most (26/29) of the patients developed the neurological symptoms and signs within 72 hours after trauma, 51.7% within 30 minutes. The first symptoms included hemiparesis (20), facial paresis (7), and convulsion (7). 86.21% of the lesions lay in basal ganglia region. Pre-existing basal ganglia calcification was identified in 13 as a risk factor.     

Controversies in the evaluation and management of minor blunt head trauma in children

PURPOSE OF REVIEW: We present data from recently conducted research regarding controversial aspects of the evaluation and management of children with minor blunt head trauma. RECENT FINDINGS: Clinicians frequently but at times indiscriminately perform computed tomography scans for children with minor blunt head trauma resulting in potentially harmful radiation exposure. Recent guidelines recognize the limited but increasing data available to make strong recommendations regarding appropriate neuroimaging decisions. Investigators have derived and validated clinical prediction models to accurately identify patients with substantial traumatic brain injury, though no clear definitive rule exists. Children younger than 2 years appear to have a higher risk of intracranial injury following minor head trauma. These patients can be difficult to assess, with the evidence suggesting the need for a more conservative approach to diagnostic imaging. We present current and accepted definitions of concussion along with risk factors and treatment for postconcussion syndrome. Current return-to-play guidelines suggest that athletes who have sustained concussion should not resume play until symptoms have resolved because of the possibility, though rare, of second impact syndrome. SUMMARY: Research in the management of children with minor head trauma is actively evolving. We present a review of recent developments that can influence current clinical practice.     

基底核钙化并脑梗死的临床特点

目的探讨基底核钙化并脑梗死小儿的临床特点和发病机制。方法分析53例脑梗死患儿的临床及影像学资料,其中有基底核钙化31例,无基底核钙化22例。结果基底核钙化并脑梗死多发生于婴幼儿、外伤后,CT均显示腔隙性梗死,MRA结果表明受累血管损害程度不重,患肢肌力恢复快,预后好。结论基底核钙化并脑梗死小儿具有突出临床特点;其发生与基底核钙化关系密切;而基底核钙化出现可能与其既往脑组织缺氧缺血有关。     

Predictors of intracranial injuries in children after blunt head trauma

This study was conducted to determine if clinical features can predict the risk of intracranial injury (ICI) in pediatric closed head trauma. We enrolled 3,806 children under 16 years consecutively referred for acute closed head trauma to the paediatric emergency room of five Italian children’s hospitals. Relevant outcomes were death and diagnosis of ICI. Clinical symptoms and signs were evaluated as possible outcome predictors. Children were also classified into five groups according to their clinical presentation. The association of ICI with signs and symptoms and the appropriateness of the five-group classification in predicting the likelihood of ICI were evaluated by logistic regression analyses. ICI was diagnosed in 22 children; 2 of them died. The risk of fatal and nonfatal ICI was 0.5 and 5.2 per 1,000 children with closed head trauma respectively. Significant associations were found between ICI and loss of consciousness, prolonged headache, persistent drowsiness, abnormal mental status, focal neurological signs, signs of skull fracture in non-frontal areas and signs of basal skull fracture. The five-group classification of children allowed an excellent prediction in terms of likelihood of ICI (ROC area 0.972). Conclusions: Selection of children with closed head trauma based on different combinations of signs and symptoms allows for early identification of subjects at different risk for ICI. In patients with minor head injuries, the absence of loss of consciousness, drowsiness, amnesia, prolonged headache, clinical evidence of basal or non-frontal skull fracture identified 100% of children without lesions. Validation of our results with a larger sample of patients with ICI would be highly desirable.     

磁共振血管造影对小儿急性偏瘫综合征的诊断价值探讨

目的探讨磁共振血管造影（ＭＲＡ）对小儿急性偏瘫综合征（ＡＨＳ）的临床应用价值。方法对３４例ＡＨＳ采用３ＤＴＯＦＭＲＡ技术方法,并联合应用磁共振成像协助诊断。结果２４例示脑血管异常改变（狭窄９例,严重狭窄和闭塞１５例）,其中颈内动脉受累１１例,大脑中动脉受累２４例,大脑前动脉受累６例,大脑后动脉受累５例。ＭＲＡ示有血管代偿现象２０例。结论ＭＲＡ较好地反映了ＡＨＳ颅内血管病变部位和范围。ＭＲＡ因其无创性、快捷、不需造影剂等特点,有可能成为儿科脑血管疾病诊断中重要的、常用的技术方法。     

Impact of cerebral palsy in patients discharged from neonatal intensive care units

AIM: The aim of this study was to assess the impact and the peculiarities of cerebral palsy (CP) in children discharged from our neonatal intensive care unit (NICU) from January 1998 to April 2004. METHODS: A total of 2 303 children were discharged from our NICU during this period and 1 912 were followed up for 1 year through neurological examination (traditional, Brazelton, general movements) and cranial ultrasound (US); high-risk newborns were evaluated with brain magnetic resonance imaging (MRI) too. RESULTS: In 65 children (3.4% of the follow-up group) were diagnosed CP, and classified as follows: 21 (32%) diplegia, 19 (29%) quadriplegia, 20 (31%) hemiplegia, 4 (6%) double hemiplegia, 1 (2%) dyskinetic form. In diplegia and quadriplegia prevailed low birth weight infants (less than or equal to 2,500 g) and preterm infants, while in hemiplegia prevailed normal birthweight infants (greater than 2,500 g) and infants at term. The main MRI findings were: in diplegia 82% periventricular white matter lesions; in quadriplegia 94% periventricular and/or subcortical white matter lesions; in hemiplegia 95% bilateral periventricular or subcortical white matter lesions, predominating on contralateral cerebral hemisphere; in double hemiplegia 100% periventricular and/or subcortical white matter lesions, 100% enlargement of subarachnoid spaces; in dyskinetic form 100% basal ganglia lesions. CONCLUSIONS: The impact of CP in children discharged from our NICU, in agreement with the literature, is higher than in the total population of newborns, thus it is very important to evaluate carefully high-risk newborns during hospitalization and follow-up, through neurological examination and radiologic imaging (US, MRI), for an accurate and early treatment.     

Are skull radiographs useful in the evaluation of asymptomatic infants following minor head injury?

Head injuries constitute a common problem in the pediatric population. Recent studies indicate that infants are at increased risk for skull fractures following head trauma. The purpose of our study is to examine the utility of skull radiographs in asymptomatic infants presenting after a minor head injury. We retrospectively reviewed the records of all head-injured infants who presented to our emergency department between March 1990 and July 1991. All symptomatic patients and all infants who did not undergo radiologic evaluation were excluded from the analysis. During the study period, 35 asymptomatic infants were evaluated in our emergency department following head trauma. The mean age of the study patients was 5.5 +/- 3.8 months; 54% were male; and falls accounted for the injury in 88% of cases. The skull radiograms were normal in 30 patients, equivocal in two, and positive for a parietal skull fracture in three. The three infants who sustained skull fractures were male, were younger than three months, and had fallen from heights not exceeding three feet. Computed head tomograms revealed no intracranial pathology in these patients. We conclude that all infants who present following minor skull trauma should undergo radiologic evaluation.     

儿童基底节病变17例临床分析

目的总结儿童基底节病变的临床特征。方法对17例经头颅CT和/或MRI证实的存在基底节病变的患儿临床资料进行分析。结果17例中起病时分别为肢体活动障碍7例,手震颤2例,惊厥5例,智力运动落后3例。所有患儿影像学检查均存在基底节区病变。17例患儿诊断分别为特发性甲状旁腺功能低下1例,线粒体脑肌病4例(1例具体诊断为Leigh病),Hyalinebody肌病1例,Aicardi-Goutieres综合征1例,偏侧惊厥-偏瘫综合征1例,脑肿瘤1例,风湿性舞蹈病2例,特发性基底节钙化4例,2例未做出具体诊断。结论基底节病变是影像学上的一个征象,涉及的疾病种类很多,对其病因进行探讨,将有助于提高对本组疾病的诊断。