中国脑性瘫痪康复指南(2022)第一章:概论北大核心CSCD

本章对脑性瘫痪(简称脑瘫)的概念、定义、定义的意义、定义中的名词解释进行了准确的诠释,同时对脑瘫的评估进行了介绍,对脑瘫的诊断和分型进行了标准化,对国内外研究热点的脑瘫早期预测、脑瘫高危儿的诊断以及脑瘫的鉴别诊断作了科学的总结和介绍。对临床医师及专业工作者对脑瘫的全面理解、精准诊断和早期预测具有重要指导意义。     

美金胺、黄芪对新生大鼠脑缺氧缺血再灌注损伤的保护作用

目的 评价美金胺和黄芪对新生大鼠缺氧缺血再灌注损伤的脑保护作用。方法 通过夹闭新生大鼠双侧颈总动脉1h、缺氧2h，建立脑缺氧缺血再灌注损伤模型。将新生大鼠随机分成正常对照组、假手术组、缺氧缺血再灌注组(HIR组)、美金胺治疗组、黄芪治疗组、黄芪和美金胺联合治疗组，每组12只，于术后6h或48h处死。取耳间线前2mm水平做冠状位脑组织切片，通过脑组织病理检查及病理评分评估美金胺和黄芪的脑保护效果。结果 缺氧缺血可引起新生大鼠明显的脑病理改变，经单独或联合应用黄芪和美金胺后，新生大鼠的脑病理改变明显减轻，各治疗组病理评分均显著低于缺血再灌注组。结论美 金胺和黄芪对治疗新生儿脑缺氧缺血损伤可能具有一定的潜在意义，尤其两药联合治疗新生儿脑缺氧缺血损伤，有望获得更好效果。     

核磁共振和核磁共振波谱结合粗大运动评定量表在儿童脑性瘫痪分度诊断的应用

目的 探讨头部核磁共振、核磁共振波谱以及粗大运动评定量表对儿童脑性瘫痪的分级诊断价值.方法 40例1.0～2.0周岁的健康儿童和42例1.1 ～2.0周岁的脑性瘫痪儿童为研究对象,对比正常儿童与脑性瘫痪患儿的MRI、MRS检测结果;根据核磁共振结果对脑性瘫痪进行分度,并用核磁共振波谱检测不同核磁共振分度的脑性瘫痪患儿脑内的NAA/Cr、CHO/Cr、LAC/Cr水平并分度;考察不同核磁共振和核磁共振波谱分度脑性瘫痪患儿的粗大运动评定量表得分情况.结果 核磁共振和核磁共振波谱均能有效区分脑性瘫痪患儿脑内的典型病变,脑内的NAA、Cr、Cho能被核磁共振波谱定量检测.不同核磁共振分度的患者脑内的NAA/Cr、CHO/Cr、LAC/Cr有所差异,重度患者与中度患者间的差异具有统计学意义(P＜0.05).脑性瘫痪的核磁共振波谱分度与核磁共振分度相符率不理想,但粗大运动评定量表评分表明各不同分度之间有差异(P＜0.05).结论 核磁共振和核磁共振波谱结合粗大运动评定量表可有效完善脑性瘫痪的分度诊断,这对不同类型儿童脑性瘫痪的临床治疗具有一定的指导意义.     

青少年隐匿性脑血管畸形的影像学诊断与分析

目的探讨临床特点、CT、MRI和显微外科在诊断青少年隐匿性脑血管畸形(AOVM)中的价值。方法对首发症状为头痛、呕吐、意识障碍15例,抽搐发作伴偏头痛7例,肢体运动及感觉障碍3例,且脑动脉数字减影血管造影(DSA)为阴性的25例青少年做了CT、MRI检查。CT平扫15例为颅内血肿,10例显示混合密度(2例有钙化),6例增强扫描可见异常血管强化。MRI分别显示环形低信号区围绕斑片状高低混合信号灶或单纯高信号。结果全部病例均经显微外科治疗,9例术中在血肿壁上或与脑组织交界处可见异常小血管。病理结果17例动静脉畸形,8例海绵状血管瘤。结论CT、MRI可对AOVM作出诊断或提示性诊断,MRI优于CT,临床特点可作为参考诊断,显微外科(包括病理检查)则是最佳确诊与治疗方法。     

Foetal supraventricular tachycardia and cerebral complications

We report on two newborn infants with foetal tachycardia and cerebral lesions. Using foetal echocardiography, the diagnosis of supraventricular tachycardia in a structurally normal heart was made at 28 and 37 weeks of gestation, respectively. One infant had a 3 week period of foetal tachycardia and hydrops before successful pharmacological cardioversion. Even several weeks after a term birth he remained hypotonic and needed gavage feeding. A computed tomography (CT) scan demonstrated cerebral lesions indicating a vascular origin. A possible thrombus was found in the heart. The other infant converted to sinus rhythm during birth by Caesarean section on the day after diagnosis. He had convulsions at the second day of life. On CT scan an infarction was found. The observations of this report suggest that cerebrovascular complications to foetal arrhythmias are more common than previously observed and should be considered when managing cases of foetal tachycardia.     

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??Abstract??Objective??To evaluate the diagnostic value of susceptibility weighted imaging ??SWI?? in cerebral vascular malformation of children. Methods??The imaging data of MRI were retrospectively analyzed in all twenty-seven cases?? which included 16 cases of venous angioma?? 8 cases of cavernous angioma and 3 cases of arterio-venous malformation. The comparison of SWI and conventional MR sequences was obtained. Results??The dilated draining veins and medulla veins were displayed well on SWI sequence. More brain cavernous angioma lesions could be detected on SWI than those on conventional MR sequences??P < 0.05??. Conclusion??SWI can detect lesions of venous angioma?? cavernous angioma and arterio-venous malformation more sensitively than other sequences?? which is a reliable diagnostic technique for cerebral vascular malformation of children. SWI has higher clinical value?? and should be the routine sequence in the MRI examination.     

儿童脑型疟128例的诊断和治疗

目的了解儿童脑型疟临床特征和降低其病死率。 方法对1996～1997年住院128例儿童脑型疟的临床特征、诊断治疗、病死原因进行分析。 结果1～5a儿童发生率66％,痊愈113例,后遗症4例,死亡11例,死亡率8．6％。临床特征有发热、咳嗽、吐泻、惊厥、意识障碍等。血片见疟原虫可确诊,首次阴性应反复多次检查。惊厥、意识障碍者应排除其他疾患。 结论及时正确诊断,尽早抗疟治疗,对抗药性及有并发症等治疗是降低儿童脑型疟病死率的关键。     

Objective assessment of limb movement in children with cerebral palsy

A preliminary report of a method for assessing the quality of movement in children with cerebral palsy, using an accelerometer, is presented. Able-bodied and disabled children performed a simple task, and there were marked differences between the two groups. Whilst the technique requires further development, it is hoped that accelerometry may eventually provide an objective tool to enable rigorous testing of various therapeutic procedures in cerebral palsy     

Childhood migraine--a dangerous diagnosis?

The diagnosis of childhood migraine cannot be confirmed in any objective way. The danger of missing brain tumours or cerebral vascular malformations in these patients was examined in two groups. 73 children who were diagnosed as childhood migraine were followed for 5.4 years. No brain tumour or vascular malformation was found, but two children diagnosed as "abdominal migraine" had a different important disease. The charts of 83 children with brain tumours and seven children with vascular malformations were examined. Only three children could be confused with migraine, and only one actually was.     

'Cerebral palsy' due to mitochondrial cytopathy

Two children aged 10 and 13 years with initially static motor problems, diagnosed as cerebral palsy, were found to have respiratory chain defects after developing a progressive course and multisystem involvement. Mitochondrial cytopathies should be considered in the differential diagnosis of cerebral palsy.     

An atypical paediatric case of malignant atrophic papulosis (Köhlmeier-Degos disease)

A new case of malignant atrophic papulosis (Köhlmeier-Degos disease) is reported. Vascular symptoms began at 17 months of age with cerebral ischaemia and progressive involvement of fingers and toes with torpid ulcers and apical necrotic amputations. At 6 years of age he developed chronic intestinal ischaemia with malabsorption and a new cerebral attack; in spite of anti-aggregant therapy the disease progressed and he died 7 months after diagnosis from a third cerebral ictus. Since the typical skin lesions of the disease were absent, the diagnosis was made on the basis of a pathological pattern of an occluded biopsed artery. The elder brother presents clinical and instrumental vascular involvement without cutaneous lesions and could be slightly affected.     

Childhood Migraine—A Dangerous Diagnosis?

ABSTRACT. The diagnosis of childhood migraine cannot be confirmed in any objective way. The danger of missing brain tumours or cerebral vascular malformations in these patients was examined in two groups. 73 children who were diagnosed as childhood migraine were followed for 5.4 years. No brain tumour or vascular malformation was found, but two children diagnosed as "abdominal migraine" had a different important disease. The charts of 83 children with brain tumours and seven children with vascular malformations were examined. Only three children could be confused with migraine, and only one actually was.     

Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma

BACKGROUND: Pleuropulmonary blastoma (PPB) is a rare tumor of pleura and lung in young children. Central nervous system (CNS) complications, particularly cerebral parenchymal metastases, occur in aggressive forms of PPB: Types II and III PPB. This article evaluates cerebral and meningeal metastases, cerebrovascular events (CVA) caused by tumor emboli, spinal cord complications, and intracranial second malignancies in PPB. PROCEDURE: International PPB Registry and literature cases were evaluated for CNS events. Cerebral metastasis patients were evaluated for gender, side of origin of PPB, PPB Type, interval from diagnosis to metastasis, status of chest disease, treatment, and outcome. Standard statistical methods were used to calculate the cumulative probability of cerebral metastasis and survival following metastasis. RESULTS: Thirty-nine cases of cerebral metastasis were identified in 5/53 Registry Type II cases, 15/44 Registry Type III cases, and 19/143 literature Type II/III cases. Metastases occurred 1-60, median 11.5 months after diagnosis. Chest disease was controlled in 50% of children at time of metastasis. The cumulative probability of cerebral metastasis by 5 years from diagnosis was 11% for Type II patients (95%CI (confidence interval): 2-20%) and 54% for Type III patients (95%CI: 31-76%). Seven children survive cerebral metastasis. Other CNS complications were post-operative CVA (five cases), spinal cord invasion or compression (six), leptomeningeal disease (three), and second intracranial malignancies (two). CONCLUSIONS: Cerebral metastasis is more frequent in PPB than in other childhood sarcomas. Clinicians should screen for this complication. Diverse other CNS complications are less common and require careful diagnosis.     

Cerebral Mantle Thickness: A Measurement Useful in Anatomic Diagnosis of Fetal Ventriculomegaly

The ultrasonographic diagnosis of cerebral ventriculomegaly carries grave implications, in that affected fetuses may suffer abnormal postnatal development or therapeutic abortion. It is important for pathologists to corroborate the clinical diagnosis, but because diagnostic methodologies and criteria differ so radically, this can be problematic. The clinical diagnosis is made primarily by serial ultrasound examinations of the cerebral ventricles, spaces that can be altered postmortem, particularly when the brain is autolysed or deformed artifactually. We therefore sought to learn if examination of tissue, rather than spaces, can identify accurately those fetuses diagnosed with cerebral ventriculomegaly by prenatal ultrasound. The thickness of the cerebral mantle was obtained in 100 control fetuses aged 14 to 26 postmenstrual weeks. Statistical analysis revealed significant correlation of cerebral mantle thickness with crown–rump length, foot length, and head circumference. Twenty fetuses diagnosed with ventriculomegaly showed mantle thicknesses that were less than the control mean. In a few cases, mantle thickness fell between the mean and −1 SD; in several others, thickness was diminished by −1 SD to −2 SD; in one-half of cases, mantle thickness was 2 SDs or more below the expected mean. Head circumference was within 2 SDs of the control mean in most cases, and increased beyond 2 SDs in only two cases. Head circumference is an unreliable indicator of ventriculomegaly in the midgestational fetus. By contrast, cerebral mantle thickness is a simple and useful way of corroborating ultrasonographic diagnoses at autopsy and may also prove useful in clinical settings. Received February 13, 1998; accepted July 20, 1998.     

Study of hemiplegic cerebral palsy with a review of the literature

Details of 66 children with hemiplegic cerebral palsy seen over a 20 year period are presented, with comparisons between right and left hemiplegia. There were slightly more right hemiplegics than left hemiplegics. There were minor differences in the frequency of associated problems, but overall there were no major differences between right and left hemiplegics. It is likely that this is due to the greater plasticity of the immature brain. The results of this study are compared with earlier studies of hemiplegic cerebral palsy.     

Interest of EEG in full-term newborns with isolated unilateral ischemic stroke]

In full-term newborns, unilateral and recurrent clonic seizures which occur during the first 48 hours of life suggest an isolated unilateral ischemic stroke. These focal seizures are isolated, occuring during a short period, or make up a status epilepticus. Electroencephalogram (EEG) is a key tool for crisis and focal cerebral process diagnosis. It also allows to assess antiepileptic drugs effectiveness. But cerebral imaging is necessary to confirm vascular origin of the cerebral impairment. Hematologic data are also needful to look for a family thromphilia. Some particular unilateral EEG abnormalities could be associated with controlateral motor sequelae or long term behavioral problems. These findings may be used for prospective studies aimed at specifying possible links between EEG abnormalities and long term outcome.     

Serial Measurements of Cerebral Blood Flow Velocity in Preterm Infants during the First 72 Hours of Life

ABSTRACT. Serial measurements of cerebral blood flow velocity (CBFV) were made in 29 preterm infants, using continuous wave Doppler ultrasound. CBFV was measured in both anterior cerebral arteries and quantitative measurements of CBFV were determined using the area under the velocity curve. In all ventilated infants, CBFV increased significantly during the first 6 hours of life and continued to increase until 16 hours of age. Thereafter, CBFV remained relatively constant. This increase in CBFV was primarily the result of increased diastolic flow. Three infants who had evidence of intraventricular haemorrhage on cranial ultrasound, had similar CBFV compared with the infants with no evidence of haemorrhage. Two infants died and both demonstrated areas of periventricular leukomalacia at autopsy. These infants had a prolonged period of low CBFV. These measurements provide normal data for ventilated, preterm infants. As previously suggested in term infants, the initial rise in CBFV may be secondary to closure of the ductus although a generalized decrease in peripheral vascular resistance could also be a contributing factor. Fluctuations in CBFV rather than individual readings are probably more important in the genesis of IVH. An episode of significantly reduced CBFV is a poor prognostic sign.     

Immunohistochemical Localization of an Hiv Epitope in Cerebral Aneurysmal Arteriopathy in Pediatric Acquired Immunodeficiency Syndrome (AIDS)

A 6-year-old boy with acquired immunodeficiency syndrome (AIDS) developed aphasia and quadriplegia 3 months before his death. Cerebral vascular ectasia and multiple cerebral infarcts were noted on premortem radiological studies. Postmortem evaluation revealed diffuse aneurysmal dilatation of the circle of Willis associated with fresh and organizing thrombi, destruction of the elastic lamina, and marked intimal fibroplasia. Multiple cerebral infarcts and subacute AIDS encephalitis with basal ganglia calcification were also present. Immunohistochemistry with a monoclonal antibody (anti-gp41) to human immunodeficiency virus (HIV) demonstrated positively stained cells in the arterial wall of the circle of Willis and in the cerebral parenchyma. Double immunostaining demonstrated that gp41-positive cells in the circle of Willis were also positive for a macrophage marker or leukocyte-common antigen, but not with an endothelial marker. Some macrophages or microglia in the cerebrum were also colabeled with anti-gp41. These results suggest that HIV may be directly involved in vascular pathology associated with pediatric AIDS.     

儿童烟雾病的临床特点

目的探讨儿童烟雾病的临床特点。方法对儿童烟雾病4例的临床资料结合文献进行分析。结果儿童烟雾病早期表现为一过性短暂性脑缺血发作,多次发作后呈持续性神经损伤征候,主要为偏瘫、肢体感觉障碍、癫发作、言语障碍、不自主运动与精神表现等。磁共振血管成像(MRA)所见与数字减影血管成像(DSA)相似,可显示脑血管狭窄或闭塞及异常血管网等改变,且为无创性检查,可作为儿童烟雾病首选辅助检查。结论儿童烟雾病临床症状不典型,神经症状无特异性,故易误诊,诊断应全面收集病史,详尽的体格检查,恰当选择辅助检查。     

新生大鼠缺氧缺血性脑损伤时细胞凋亡抑制蛋白1的变化

目的探讨在新生大鼠缺氧缺血性脑损伤(HIBD)细胞凋亡抑制蛋白1(cIAPl)的变化。方法建立新生大鼠HIBD动物模型，采用RT-PCR技术检测缺氧缺血(HI)后不同时间点缺血侧脑组织中cIAP1基因表达的变化。结果正常组中有cIAPl基因表达，HI组cIAPl基因表达明显下调，且随HI后时间的延长，cIAPl基因表达在HI后6h开始下调，24h下调最明显，48-72h有所回升。结论脑缺氧缺血引起的神经细胞凋亡可能与clAPl基因表达下调有关。