Liaw S B, Shen E Y, Hsu C H, Hong H Y, Kao H A, Ho M Y, Huang F Y
Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan, R.O.C.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 1990 Sep-Oct;31(5):288-98.
During the more than five years from January 1984 to June 1989, twenty-four patients with definite or probable cystic periventricular leukomalacia (PVL) were diagnosed by cranial ultrasonography at Mackay Memorial Hospital. The 24 patients were divided into two groups. Group A comprise two boys and four girls who received longitudinal sonographic follow-ups for leukomalacia. Of these six patients, five were premature and all suffered from severe perinatal insults. In each case, sequences of developmental cystic PVL were observed by serially scanning the brain. High echogenicity was discovered during the initial stages (2 to 7 days) in the periventricular area, and cystic formations were observed between the age of 18 and 60 days. Clinically, only one patient developed normally; four had severe motor dysfunction and poor motor development; and one was lost during follow-up, Group B was composed of 18 patients who visited the out-patient clinic for psychomotor retardation evaluation, and were found through ultrasound to have or possibly have cystic PVL formations at various stages. The clinical work-up revealed that 12 had spastic quadriplegia; 2 had hemiplegia; 3 had spastic displegia; and 1 case had hypotonic cerebral palsy. In infants, PVL is considered to be a much more reliable and important prognostic predictor than intraventricular hemorrhage. Consequently, it is crucial that physicians should screen patients at high risk for PVL, especially those with perinatal insults.
在1984年1月至1989年6月的五年多时间里,台北马偕纪念医院通过头颅超声诊断出24例明确或可能患有脑室周围白质软化症(PVL)的患者。这24例患者被分为两组。A组包括2名男孩和4名女孩,他们接受了白质软化症的纵向超声随访。在这6例患者中,5例为早产儿,均遭受了严重的围产期损伤。在每例患者中,通过对脑部进行连续扫描观察到了进行性发展的囊性PVL序列。在初始阶段(2至7天),在脑室周围区域发现高回声,在18至60天龄之间观察到囊性结构形成。临床上,只有1例患者发育正常;4例有严重的运动功能障碍和运动发育不良;1例在随访期间失访。B组由18例因精神运动发育迟缓评估前来门诊就诊的患者组成,通过超声检查发现他们在不同阶段有或可能有囊性PVL形成。临床检查显示,12例有痉挛性四肢瘫;2例有偏瘫;3例有痉挛性双瘫;1例有低张型脑瘫。在婴儿中,PVL被认为是比脑室内出血更可靠、更重要的预后预测指标。因此,医生筛查PVL高危患者,尤其是那些有围产期损伤的患者至关重要。