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.     

脑瘫患儿的健康教育

小儿性瘫痪是由脑损伤所致的运动发育落后及姿势异常的一种临床综合征。常伴有智力低下、癫痫、语言障碍、行为异常和视听觉障碍等并发症。是儿童残疾的主要原因，给患儿带来了极大的痛苦，给家庭和社会带来了很重的负担。为了早期干预及早期治疗，减少并发症发生及提高脑瘫患儿的生活质量，应及时进行健康教育。     

Duchenne肌营养不良患儿的智力及与孤独症谱系障碍的关系研究

目的 探讨Duchenne肌营养不良(DMD)患儿的智力水平和智力结构特点,以及与孤独症谱系障碍(ASD)关系及其所占比例.方法 收集2006年1月-2010年5月诊断的DMD患儿118例(其中78例已经dystrophin基因确诊),应用修订的韦氏儿童智力量表-中国版(C-WISC)和美国精神疾病诊断统计手册(DSM-Ⅳ)结合儿童孤独症评定量表(CARS)对其进行智力及智力结构分析和孤独症谱系障碍评估.结果 118例DMD患儿中有39例进行了智力测定,其中有12例总智商(FIQ)低于70,占30.8%(12/39),轻度智力低下7例(50≤IQ<70),中、重度智力低下5例(IQ<50);DMD患儿总智商、言语智商(VIQ)、操作智商(PIQ)及其11项分测验得分中,除图形拼凑与对照组差异无统计学意义(P=0.237),其余均低于对照组(P<0.05);且言语智商低于操作智商(P<0.05).在118例DMD患儿中有3例(2.5%)同时合并孤独症谱系障碍,CARS评分均≥30分.结论 DMD合并孤独症谱系障碍可能并不是偶然事件,DMD与孤独症谱系障碍可能存在一定联系.     

脑室周围白质软化早产儿126例

目的 探讨早产儿脑室周围白质软化(PVL)的治疗措施.方法 PVL早产儿126例按家长意愿分为干预1组(57例)、干预2组(35例)和常规育儿组(34例).干预1组予脑细胞保护剂,接受常规育儿指导及早期干预治疗.干预2组接受常规育儿指导外,进行针灸及按摩传统中医治疗.常规育儿组只接受常规育儿指导.3组患儿均在纠正胎龄后3、9、12、24、30个月行智力检查.纠正胎龄9个月后行神经运动检查.结果 干预1组患儿智力发育及运动发育指数明显高于常规育儿组(Pa<0.05);与干预2组比较,智力发育指数明显提高(Pa<0.05),纠正胎龄9个月后运动发育指数无显著性差异(Pa0.05).3组间脑性瘫痪发病率比较均无显著差异(Pa0.05).结论 早期综合治疗早产儿PVL有一定疗效,可促进智力发育及运动发育,减少认知、行为缺陷,减轻运动障碍,但不能降低PVL脑性瘫痪发生率.     

成都地区脑性瘫痪患儿422例伴发小头畸形临床分析

目的分析伴小头畸形脑性瘫痪(脑瘫)患儿的临床特征,为合理康复管理提供依据。方法总结2013年2月至4月对成都市辖区残联登记为脑性瘫痪的0～18岁儿童进行了横断面调查结果,共计422例。根据头围对患儿进行分组,分析脑瘫患儿头围与粗大运动功能损伤程度(GMFCS)、智力、合并症等相关性。结果两组不同程度头围组与GMFCS分级的分布情况差异有统计学意义(P=0.001)、两组不同程度头围组与认知分级分布情况差异有统计学意义(P0.001),Spearman相关分析示伴小头畸形脑瘫患儿的伴发障碍数量、GMFCS和认知损伤程度与头围呈负相关(P0.001)。结论伴小头畸形的脑瘫患儿头围减小程度与GMFCS、伴发障碍的数量和认知损伤程度呈负相关。     

白介素18与早产儿脑室周围白质软化的相关性研究

目的 探讨白介素18(IL-18)在早产儿脑室周围白质软化(PVL)发病机制中的作用,为早产儿PVL的早期诊断及防治提供可能的理论依据.方法 经头颅B超诊断为PVL早产儿21例,对照组早产儿20例,应用酶联免疫吸附试验测定出生6 h内血清IL-18值.结果 根据头颅B超结果,将PVL组患儿分为Ⅰ级8例,Ⅱ级6例,Ⅲ级5例及Ⅳ级2例.Ⅰ级PVL患儿血清IL-18水平明显高于对照组(P<0.05);Ⅱ级PVL患儿明显高于Ⅰ级(P<0.05);Ⅲ级明显高于Ⅱ级(P<0.05).PVL组脑瘫发生率明显高于对照组(P<0.05).结论 早产儿血清IL-18水平升高与早产儿PVL的发生密切相关,在出生时可作为一个早期预测指标,并为防治早产儿PVL发生提供可能的理论依据.     

儿童脑性瘫痪CT形态学改变与临床关系的分析

研究目的 探讨儿童脑性瘫痪CT形态学改变与临床的关系。 研究方法 儿童脑瘫患者100例,男66例,女34例。所有患者共分为5个年龄组,分别行颅脑CT检查。 结果 儿童脑瘫多发于6个月～3岁间。100例患儿中CT扫描异常者73例,异常率为73％。脑室周萎缩(PVL)为最常见的CT形态学改变,共23例。中间部异常18例,皮质或皮质下萎缩20例,其它改变 13例。CT扫描正常者(27例)症状大多较轻,较严重的PVL,脑皮质及皮质下缩萎、脑囊性变,临床上多有严重的运动障碍。 结论 CT形态学检查对儿童脑瘫患者的诊断及预后判定具有重要意义。     

脑室周围白质软化早产儿血清半胱氨酸蛋白酶-1和白细胞介素-18水平的变化

目的 研究脑室周围白质软化(PVL)早产儿血清半胱氨酸蛋白酶-1(Caspase-1)和IL-18水平的变化及二者的相关性,探讨PVL的发病机制及Caspase-1、IL-18的临床意义.方法 采用酶联免疫吸附法(ELISA)检测日龄48～72 h的13例PVL早产儿(PVL组)、17例脑室内出血(IVH)早产儿(IVH组)及20例健康对照早产儿(对照组)出生第3天血清Caspase-1和IL-18的水平.采用SPSS 13.0软件进行统计学分析.结果 PVL组早产儿血清Caspase-1和IL-18均明显高于IVH组和对照组早产儿,两组比较差异均有统计学意义(Pa＜0.05);IVH组和对照组早产儿血清Caspase-1和IL-18比较差异无统计学意义(P＞0.05);PVL组早产儿血清Caspase-1和IL-18呈正相关(r=0.965,P=0.000).结论 Caspase-1和IL-18参与了早产儿PVL的发病机制,联合测定Caspase-1和IL-18可作为早期诊断早产儿PVL的方法之一.     

神经系统相关性蛋白质与新生儿脑损伤

脑损伤是新生儿时期危害最大的常见病，围生期常见的脑损伤形式有缺氧缺血性脑病（HIE）、颅内出血（ICH）及脑室周围白质软化（PVL）等，易导致患儿神经系统发育障碍如脑瘫、癫痫及精神发育迟缓等。目前脑损伤的诊断主要依靠患儿的临床表现和影像学检查，其诊断价值和对患儿预后的判断尚存不足，寻找更加灵敏且特异度高的预告脑损伤的生化指标一直是新生儿临床所期盼的。     

脑白质损伤早产儿血清髓鞘碱性蛋白和S1OOB蛋白的变化及与预后的关系

目的 探讨脑白质损伤( periventricular leukomalacia,PVL)患儿血清髓鞘碱性蛋白(myelin basic protein,MBP)及S100B蛋门(S100B protein,S100B)的动态变化及其与患儿预后的关系.方法 对2007年11月至2008年7月我院住院的78例PVL早产儿(PVL组)和43例正常早产儿(正常对照组),分别在其生后第1、3、7、14天测定血清中MBP及S100B含量.30例正常早产儿及69例PVL患儿出院后每3个月随方1次,直至纠正胎龄至1岁,用Gesell发育量表测定其智力以及运动发育情况.结果 (1) PVL组患儿血清MBP于生后第1天升高[(7.61±1.78) μg/L]、第3天达峰值[( 14.53±3.12) μg/L],后随病情好转,逐渐降低;与正常对照组比较,PVL组患儿在生后第1、3、7、14天血清MBP水平均明显高于正常对照组(P＜0.05).(2) PVL组患儿血清S100B水平在生后第1、3、7天明显升高[(3.82±0.68),(4.41±0.91),(5.78±1.54) μg/L],第7天达峰值,与正常对照组比较,差异有统计学意义(P＜0.05);至生后第14天时,S100B明显降低,两组比较已无明显差异(P＞0.05).(3) PVL组患儿生后第7天血清S100B、MBP持续升高者,随访至1岁时其发育商比生后第7天血清S100B及MBP明显下降者落后;也明显落后于正常早产儿(P＜0.05).结论 PVL患儿生后血清MBP及S100B水平与病情严重程度相关.如患儿血清MBP及S100B持续升高超过7d,则发育商明显落后,预后不良.     

Correlation between neuroimaging and neurological outcome in periventricular leukomalacia: Diagnostic criteria

BACKGROUND: Periventricular leukomalacia (PVL) is the most important factor in cerebral palsy in preterm infants. METHODS: In the present study, we investigated 747 preterm infants of less than 36 weeks gestation who were repeatedly examined by cranial ultrasonography and computed tomography (CT) scanning at around 40 weeks of corrected post-menstrual age. The clinical course of these infants was followed for more than 3 years and they were examined by magnetic resonance imaging (MRI) between 12 and 18 months of age. RESULTS: Single examinations in early infancy were not sufficient to diagnose PVL, but the combination of ultrasonography, CT and MRI examinations allowed the clinical diagnosis of PVL. In preterm infants, clinical PVL could be predicted from cystic PVL and periventricular echogenicity (PVE) 3 or PVE 2 prolonged over 3 weeks on ultrasonography and confirmed by MRI after 11 months of corrected age. CONCLUSIONS: We tried to determine diagnostic criteria for PVL by neuroimaging. Such criteria from neuroimaging for PVL may be useful for determining the exact occurrence rate of and clinical risk factors for PVL.     

磁共振弥散加权成像在早产儿脑室周围白质软化早期的预测价值

目的 探讨磁共振弥散加权成像(DWI)早期评价和预测早产儿脑室周围白质软化(PVL)的作用及意义.方法 回顾分析2005年8月至2007年4月,在我院新生儿科住院,且经头部MRI确诊的12例PVL早产儿生后7 d内、2 w和4 w的DWI及常规MRI资料.结果 初次检查(平均生后4.5 d)全部病例DWI均显示双侧脑室周围脑白质对称性、弥漫性高信号,常规MRI基本正常;出生后2周DWI示脑白质内不规则高、低混杂信号,而常规MRI则显示相应部位小片状或点状T1WI高信号,T2WI稍低信号;出生后4W DWI示侧脑室后角、枕部三角区大小不等的囊性低信号,常规MRI显示相应病灶的T1WI低信号,T2WI高信号(即囊性PVL改变);出生后4个月常规MRI示囊腔逐渐变小、消失,脑白质减少、脑室扩大.结论 DWI显示的双侧脑室周围白质对称性弥漫性高信号是PVL的最早期表现;所提供的影像学异常变化与晚期常规MRl所证实PVL发生的高度相关性,表明了DWI可能是早期评价脑白质损伤及预测早产儿PVL发生的重要检测手段.     

[18F]-Fluorodeoxyglucose-positron emission tomography findings in preterm infants with severe periventricular leukomalacia and hypsarrhythmia

Two preterm infants with extensive periventricular leukomalacia (PVL) were examined by [¹⁸F]-fluorodeoxyglucose-positron emission tomography (FDG-PET) at the corrected ages of 18 and 34 days. They showed similar clinical courses including oculoclonic seizure, hypsarrhythmia and severe mental retardation, in addition to spastic quadriplegia. FDG-PET study of these two infants with severe PVL disclosed poorly developed metabolic activity in the primary sensorimotor cortex, while the MRI images displayed only periventricular white matter lesions. Conclusion Positron emission tomography may dis‐close cortical involvement in infants with severe periventricular leukomalacia. Received: 23 March 1996 / Accepted: 10 August 1996     

脑室周围白质软化早产儿出生早期MRI表现及其演变

目的 探讨最终发展为脑室周围白质软化（periventricular leucomalacia,PVL）的早产儿出生早期头部MRI表现及其演变.方法 选取2010年1月至2013年12月中国医科大学附属盛京医院新生儿科住院经MRI确诊为PVL的患儿12例,所有病例均在生后2～7d（平均5.5d）及17～23 d（平均20.3 d）完成2次常规MRI及弥散加权成像（diffusion-weighted imaging,DWI）扫描.结果 最终被确诊的12例PVL患儿,初次扫描结果：全部病例DWI显示脑室周围白质高信号,其中6例弥漫、对称性高信号,3例线状高信号,3例簇状高信号,而常规MRI仅有5例显示T1加权像稍高信号,T2加权像稍低信号,余7例无改变;再次扫描结果：全部患儿脑室周围白质出现大小不等、数量不一的病灶,常规MRI显示T1加权像低信号,T2加权像高信号,相应病变部位DWI呈低信号.结论 早产儿出生早期,DWI对发现和预测PVL优于常规MRI;弥漫性脑白质损伤易发展为PVL,较严重的局灶性脑白质损伤,如多簇状、线状损伤也有发展成PVL的可能.     

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。     

Cerebral MRI of very low birth weight children at 6 years of age compared with the findings at 1 year

Background. We have previously reported the results of cerebral MRI examinations in an unselected year cohort of very low birth weight (VLBW) infants at one year of corrected age. Twenty-one (78 %) of 27 infants had abnormal myelination, mainly in the central occipital white matter (COWM) and in the centrum semiovale (CS), seen on T2-weighted images. Twelve infants had irregular and dilated lateral ventricles. We speculated whether these findings indicated perinatal periventricular leukomalacia (PVL). Only two infants had completely normal MRI at age 1 year. Objective. To determine whether the abnormal myelination seen at 1 year of age, was still present, either as delayed myelination or as gliosis caused by perinatal PVL. Materials and methods. In the present study, we report the results of follow-up cerebral MRI in 20 of these infants at 6 years of age. Results. Most of the children with MRI deviations at 1 year still had abnormalities at 6 years. Abnormal myelination in the central occipital white matter combined with abnormalities in the CS or with ventricular dilatation at age 1 year, presented as gliosis in 12 of 13 children at 6 years of age. Abnormalities solely in the COWM at age 1 year had normalised in two of five children and persisted as delayed myelination in three at age 6 years. Gliotic changes in periventricular white matter were found in 12 of 20 children (60 %). Areas most affected were the CS (11 children) and the COWM (9 children). Delayed myelination in COWM was found in six children (30 %), combined with gliosis in CS in three children. Twelve infants had ventricular dilatation both at 1 and 6 years of age. Conclusions. The MRI correlates of PVL, i. e. gliosis and ventricular dilatation, are common findings on cerebral MRI at 6 years of age in VLBW infants. Received: 20 February 1997 Accepted: 1 December 1997     

新生儿脑室周围白质软化的危险因素及预后

目的调查5年来新生儿脑室周围白质软化（PVL）发生率、危险因素及预后。方法对2000年12月～2005年11月本院记录资料较完整的806例新生儿中遗留脑性瘫痪61例患儿,至少2次头颅B超或MRI检查,确诊且排除其他疾病的PVL患儿26例为研究组。同期住院的35例无PVL的脑性瘫痪患儿为对照组。行PVL高危多因素回归分析、预后调查。结果PVL26例中早产儿占8.45%,足月儿占1.35%。重度窒息（x3）、孕周（x1）、颅内出血（x14）、低血压（x8）是PVL发生的主要高危因素,其OR值分别为2.843、3.575、3.268、1.947。妊娠高血压综合征、新生儿呼吸窘迫综合征、宫内感染对PVL发生的影响差异也有显著性（Pa〈0.05）。结论PVL发生率高,致病因素复杂,预后差,主要后遗症为痉挛性瘫痪及智力低下。     

Periventricular leukomalacia: overview and recent findings.

Periventricular leukomalacia (PVL), the main substrate for cerebral palsy, is characterized by diffuse injury of deep cerebral white matter, accompanied in its most severe form by focal necrosis. The classic neuropathology of PVL has given rise to several hypotheses about the pathogenesis, largely relating to hypoxia-ischemia and reperfusion in the sick premature infant. These include free radical injury, cytokine toxicity (especially given the epidemiologic association of PVL with maternofetal infection), and excitotoxicity. Among the recent findings directly in human postmortem tissue is that immunocytochemical markers of lipid peroxidation (hydroxy-nonenal and malondialdehyde) and protein nitration (nitrotyrosine) are significantly increased in PVL. Premyelinating oligodendrocytes, which predominate in periventricular regions during the window of vulnerability to PVL (24 to 34 postconceptional weeks), are the targets of this free radical injury, and suffer cell death. Susceptibility can be attributed, at least in part, to a relative deficiency of superoxide dismutases in the preterm white matter, including premyelinating oligodendrocytes. Several cytokines, including interferon-gamma (known to be directly toxic to immature oligodendroglia in vitro), as well as tumor necrosis factor-alpha and interleukins 2 and 6, have been demonstrated in PVL. Microglia, which express toll-like receptors to bacterial products such as lipopolysaccharide, are increased in PVL white matter and may contribute to the injury. Preliminary work suggests a role for glutamate receptors and glutamate transporters in PVL, as has been seen in experimental animals. These findings pave the way for eventual therapeutic or preventive strategies for PVL.     

新生儿缺氧缺血性脑损伤的三种影像学诊断特性比较

目的：比较头颅超声（US）、CT及磁共振成像（MRI）三种影像学方法对新生儿缺氧缺血性脑损伤（HIBD）的诊断特性,方法：对20例新生儿HIBD和15例正常新生儿进行US、CT及MRI同步对照检查。结果：头颅US、CT、MRI以诊断新生儿HIBD的正确诊断率及阳性率分别为89％和80％、94％和90％、91％和85％,三者的阴性率均为100％。US对早期脑室周围白质软化的分辨率明显高于CT和MRI。MRI能清晰地诊断以往US和CT未能诊断的旁矢状区损伤,借助MRI诊断,旁矢状区损伤在CT中的表现也得以辨认,三种影像诊断方法对脑水肿、基底神经节丘脑损伤、脑动脉梗死及晚期脑室周围白质软化的分辨率基本相似,结论：US、CT和MRI对诊断不同的新生儿颅内病变各具特点,临床应合理选择,互补诊断,以进一步提高新生儿颅内病变的正确诊断率。     

Gray matter volume decrements in preterm children with periventricular leukomalacia

Periventricular leukomalacia (PVL) is the prototypic lesion in the encephalopathy of prematurity. Although PVL is identified by targeting cerebral white matter (WM), neuropathological and MRI studies document gray matter (GM) loss in cortical and subcortical structures. This study aimed to investigate the distribution of GM changes in children with a history of premature birth and PVL. Voxel-based morphometry was used to examine regional GM abnormalities in 22 children with a history of preterm birth and PVL. Preterms with PVL were compared with 22 terms and 14 preterms without PVL of similar GA and birth weight. GM and WM global volumetric volumes were found to decrease in comparison with both control groups. Regional GM volume abnormalities were also found: compared with their term peers, preterm children with PVL showed several regions of GM reduction. Moreover, PVL differed from preterms without PVL in the medial temporal lobe bilaterally, thalamus bilaterally, and caudate nuclei bilaterally. In addition, in our preterm sample with PVL, birth weight showed a statistical significant correlation with decreased GM regions. In conclusion, the voxel-based morphometry methodology revealed that PVL per se does involve GM reductions.