Miller S P, Shevell M I, Patenaude Y, O'Gorman A M
Division of Pediatric Neurology, Department of Neurology and Neurosurgery, Montreal Children's Hospital, McGill University, Montreal, Quebec, Canada.
Pediatr Neurol. 2000 Aug;23(2):155-9. doi: 10.1016/s0887-8994(00)00172-7.
The spectrum of neuromotor abnormalities of term children with periventricular leukomalacia (PVL) has never been specifically defined. We report 12 term children with PVL to delineate its long-term clinical correlates. A retrospective review of a standardized computer database and files of a single pediatric neurologist during a 7-year period was performed. The imaging studies were reviewed independently by two neuroradiologists. The mean age of the patients at the initial neurologic assessment was 24. 4 months (range 5-60); nine were males. The reason for the assessment was developmental delay in 10 (83.3%), seizure in one, and attention-deficit-hyperactivity disorder in one. Three children (25%) had normal motor examinations, three (25%) were hypotonic, three (25%) had spastic diplegia, two (16.7%) had spastic quadriplegia, and one (8.3%) had fine-motor abnormalities. Nine children (75%) had developmental delay (severe global delay in six), and two children (16.7%) had epilepsy; electroencephalograms were abnormal in six (50%). PVL was mild in five (41.7%), moderate in two (16.6%), and severe in five (41.7%) children. Four of eight children with global developmental delay had enlargement of cerebral sulci in addition to white matter changes. PVL in term children presents with a spectrum of neurologic abnormalities, particularly developmental delay and heterogeneous motor findings not limited to classic spastic diplegia. The clinician should consider the diagnosis of PVL in the context of term children with developmental delay and motor abnormalities, even in the absence of perinatal difficulties.
足月儿脑室周围白质软化症(PVL)的神经运动异常谱尚未有明确界定。我们报告12例足月儿PVL病例,以阐述其长期临床关联。对一个标准化计算机数据库及一位儿科神经科医生7年间的病例档案进行了回顾性研究。影像学检查由两位神经放射科医生独立复查。患者初次神经学评估时的平均年龄为24.4个月(范围5 - 60个月);9例为男性。评估原因包括发育迟缓10例(83.3%)、癫痫1例、注意缺陷多动障碍1例。3例患儿(25%)运动检查正常,3例(25%)肌张力低下,3例(25%)为痉挛性双瘫,2例(16.7%)为痉挛性四肢瘫,1例(8.3%)有精细运动异常。9例患儿(75%)有发育迟缓(6例为严重全面发育迟缓),2例患儿(16.7%)有癫痫;6例(50%)脑电图异常。5例患儿(41.7%)PVL为轻度,2例(16.6%)为中度,5例(41.7%)为重度。8例全面发育迟缓患儿中有4例除白质改变外还伴有脑沟增宽。足月儿PVL表现为一系列神经学异常,尤其是发育迟缓以及不限于典型痉挛性双瘫的多种运动表现。即使没有围产期问题,临床医生在遇到发育迟缓和运动异常的足月儿时也应考虑PVL的诊断。