不随意运动型脑瘫临床特点分析

目的探讨不随意运动型脑瘫的症状学分类、危险因素、神经影像学异常特征及其相互关系。方法回顾性研究我中心住院治疗的136例不随意运动型脑瘫患儿的临床特点。根据锥体外系损伤的神经病学机制，将不随意运动型脑瘫分为：张力障碍型、舞蹈-徐动型、徐动伴痉挛3种不同类型，以母亲调查问卷及会晤方式详细记录围产期脑损伤高危因素，由专科医师进行全面的神经科体格检查，Gesell发育评分法评价神经发育水平。结果124例（91.18％）存在明确的高危因素，其中窒息35例（25．74％）、病理性黄疸70例（51．47％）、窒息＋病理性黄疸11例（8．09％）、其他病因8例（5．88％）；舞蹈-徐动型脑瘫60例（44．12％），张力障碍型26例（19．12％），徐动-痉挛型50例（36．76％），窒息组徐动-痉挛型的比例明显高于舞蹈-徐动型和张力障碍型（X^2=46．3714，P〈0．05）；黄疸组主要类型为舞蹈-徐动型，其次是张力障碍型，徐动-痉挛型的比例最少（X^2=26．56，P〈0．001）；其他病因或病因不明组各亚类型之间无明显差异（X^2=2．7097，P〉0．05）；MRI异常率52．9％，窒息组异常率明显高于黄疸组（X^2=14．2331，P〈0．01），早产儿组异常率明显高于足月儿组（X^2=13．7185，P〈0.01）；脑室周围白质软化（PVL）28例（38.8％）、弥漫性脑萎缩20例（27．8％）、基底节区异常与颞叶萎缩18例（24．0％）。结论不随意运动型脑瘫的主要危险因素是病理性黄疸与窒息；临床症状表现与危险因素以及出生胎龄有关；MRI异常率低于其他脑瘫类型；主要影像学异常以PVL和弥漫型脑损伤为主，孤立的基底节区损害少见；对该类型脑瘫应合理选择检查方法，正确评价病因、临床症状特点和病理变化。

Clinical characteristics of dyskinetic cerebral palsy

ObjectiveTo study the risk factor, semeiology and neuroimaging abnormalities of dyskinetic cerebral palsy.MethodsA hospital-based study, 136 children with dyskinetic cerebral palsy were examined neurologically and their perinatal history was reviewed. Their cranial CT or MRI findings were studied. The association between the gestational ages, CP types and the radiological appearances were analyzed.Results124 cases (91.18%) were found obviously risk factors, including asphyxia (34 cases), pathological hyperbilirubinemia (70 cases), both asphyxia and hyperbilirubinemia (11 cases) and others (8 cases). According to the clinical and neurological features, 60 (44.12%) were chorea-athetoid cerebral palsy, 26 (19.12%) were dystonic cerebral palsy, and 50 (36.76%) were athetoid-spastic cerebral palsy. Those with asphyxia were mainly athetoid-spastic whereas cases with pathological hyperbilirubinemia were mainly chorea-athetoid cerebral palsy. The abnormal rates of cranial MRI scans was 52.9%, and it was higher in the group of asphyxia than pathological hyperbilirubinemia, preterm than term. The main findings on MRI scans were as follows: periventricular leucomalacia(PVL) 28 (38.8%), diffuse bilateral atrophy 20 (27.8%), focal abnormalities in the basal ganglia1 and/or temporal lobe 18 (24.0%).ConclusionMRI abnormalities of the brain were correlated with semeiologic subtypes, risk factors, and the gestational age at birth.