Sugimoto T, Yasuhara A, Nishida N, Murakami K, Woo M, Kobayashi Y
Department of Pediatrics, Kansai Medical University Otokoyama Hospital, Kyoto, Japan.
Neuropediatrics. 1993 Feb;24(1):4-7. doi: 10.1055/s-2008-1071504.
Fifty-five patients with microcephaly (head circumference < -2SD) were identified. The 55 patients were divided into two groups, consisting of group 1 (34 cases) in which genetic causes were considered primary, and group 2 (21 cases) in which intrauterine and/or postnatal acquired factors were thought to be responsible. MRI abnormalities were present in 80% of the total series: 68 and 100% in groups 1 and 2, respectively. In group 1, migration abnormalities were the most prominent and mental retardation was the major neurological handicap, while in group 2, hydranencephaly and infarction secondary to brain circulatory derangements were the most frequently observed abnormalities, with severe multiple handicaps such as cerebral palsy, epilepsy and mental retardation seen. Head MRI was considered indispensable in the investigation of the causes of microcephaly and in determining the neurological prognosis of affected patients.
共识别出55例小头畸形患者(头围<-2标准差)。这55例患者被分为两组,第1组(34例),认为其主要病因是遗传因素;第2组(21例),认为其病因是宫内和/或出生后获得性因素。在整个系列中,80%的患者存在MRI异常:第1组和第2组分别为68%和100%。在第1组中,迁移异常最为突出,智力发育迟缓是主要的神经障碍,而在第2组中,最常观察到的异常是积水性无脑畸形和脑循环紊乱继发的梗死,还可见严重的多重障碍,如脑瘫、癫痫和智力发育迟缓。头颅MRI被认为在小头畸形病因调查以及确定受影响患者的神经预后方面不可或缺。