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偏瘫型脑瘫。病因与预后。

Hemiplegic cerebral palsy. Aetiology and outcome.

作者信息

Uvebrant P

机构信息

Department of Pediatrics II, East Hospital, Göteborg, Sweden.

出版信息

Acta Paediatr Scand Suppl. 1988;345:1-100. doi: 10.1111/j.1651-2227.1988.tb14939.x.

Abstract

Hemiplegic cerebral palsy (CP) was studied in a retrospective population-based series of 169 cases from the South-western Swedish health care region covering the birth years 1969-78. The purpose was to analyse the prevalence, aetiology and neuro-developmental outcome in children born preterm and at term, and to correlate pathogenetic periods, aetiological factors and clinical parameters to neuroradiology. The prevalence at the ages 6-15 years was 0.66 per 1000. Postnatally acquired hemiplegia, mainly postinfectious, iatrogenic or posttraumatic, constituted 11%. Among term children with congenital hemiplegia (pre and perinatally derived) the aetiology was considered prenatal, mainly circulatory brain lesions and maldevelopments, in 42%, combined pre and perinatal in 9%, perinatal (cerebral haemorrhage, hypoxia) in 16% and untraceable in 34%. The corresponding distribution among preterm children was 29%, 47%, 25% and 6%, respectively. The rate of preterm birth among congenital cases was 24%. Birth asphyxia was shown to be a poor indicator of pathogenetic period, whereas a cascade of postpartum complications suggested perinatal brain damage. Clinical follow-up of 152 children revealed that 50% had mild, 31% moderate and 19% severe motor dysfunction. Stereognostic sense was impaired in 44% of the children (astereognosia in 20%). Additional impairments (mental retardation, epilepsy, impaired vision, hearing and speech, severe behavioural/perceptual problems) were present in 42%. Term children with congenital hemiplegia tended to be more severely affected than preterm children. The resulting total handicap was considered mild in 40%, moderate in 44% and severe in 16%. The prevalence of severe total handicap was highest among postnatal cases. Computerised tomography (CT), performed in 109 congenital cases, was normal in 26%, showed unilateral ventricular enlargement in 36% and revealed cortical/subcortical cavities in 20%. In the remaining 18% CT findings were classified as "other". With the classification so far used, correlations between CT findings and aetiologies were unsatisfactory and disappointing. In contrast, CT findings showed a strong correlation with clinical degree of severity and magnitude of associated handicap. As a rule, normal CT implied mild disability and unilateral ventricular enlargement moderate, whereas cortical/subcortical cavities were frequently associated with severe handicap, including mental retardation and epilepsy.

摘要

我们对瑞典西南部医疗保健地区1969 - 1978年出生的169例偏瘫型脑瘫(CP)患者进行了一项基于人群的回顾性研究。目的是分析早产和足月出生儿童的患病率、病因及神经发育结局,并将发病时期、病因因素和临床参数与神经放射学进行关联。6 - 15岁儿童的患病率为每1000人中有0.66例。产后获得性偏瘫,主要是感染后、医源性或创伤后所致,占11%。在先天性偏瘫(产前和围产期所致)的足月儿童中,病因被认为是产前的,主要是脑循环病变和发育异常,占42%;产前和围产期合并因素占9%;围产期(脑出血、缺氧)占16%;病因不明的占34%。早产儿童中的相应分布分别为29%、47%、25%和6%。先天性病例中的早产率为24%。出生窒息被证明是发病时期的一个不良指标,而后一系列产后并发症提示围产期脑损伤。对152名儿童的临床随访显示,50%有轻度运动功能障碍,31%有中度运动功能障碍,19%有重度运动功能障碍。44%的儿童存在实体觉受损(20%为触觉失认)。42%的儿童存在其他障碍(智力发育迟缓、癫痫、视力、听力和言语受损、严重行为/感知问题)。先天性偏瘫的足月儿童往往比早产儿童受影响更严重。最终的总体残疾程度被认为轻度的占40%,中度的占44%,重度的占16%。重度总体残疾的患病率在产后病例中最高。对109例先天性病例进行了计算机断层扫描(CT),26%的结果正常,36%显示单侧脑室扩大,20%显示皮质/皮质下空洞。其余18%的CT结果被归类为“其他”。按照目前使用的分类方法,CT结果与病因之间的相关性并不理想且令人失望。相比之下,CT结果与临床严重程度和相关残疾程度密切相关。通常,CT结果正常意味着轻度残疾,单侧脑室扩大意味着中度残疾,而皮质/皮质下空洞往往与重度残疾相关,包括智力发育迟缓和癫痫。

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