先天性强直性肌营养不良中的脑室扩张。

Cerebral ventricular dilation in congenital myotonic dystrophy.

作者信息

Regev R, de Vries L S, Heckmatt J Z, Dubowitz V

出版信息

J Pediatr. 1987 Sep;111(3):372-6. doi: 10.1016/s0022-3476(87)80456-0.

Abstract

Ultrasonography or computed tomography scanning of the brain was performed in 10 infants with congenital myotonic dystrophy between the age of 1 day and 2 months, and showed intracranial abnormalities in all. Ventricular dilation was diagnosed in eight (80%), subarachnoid hemorrhage in one, and white matter infarcts in one. The common finding of ventricular dilation is probably related to developmental brain abnormality dating back to fetal life, because it was already present in three infants scanned on the first day of life. Neonatal asphyxia was present in seven infants, associated with intraventricular hemorrhage in two. The relationship between these changes and mental retardation, which is a common feature in this disease, is unclear.

摘要

对10名年龄在1天至2个月之间的先天性肌强直性营养不良婴儿进行了脑部超声检查或计算机断层扫描，结果显示所有婴儿均存在颅内异常。8例（80%）诊断为脑室扩张，1例为蛛网膜下腔出血，1例为白质梗死。脑室扩张这一常见发现可能与可追溯至胎儿期的脑部发育异常有关，因为在出生第一天接受扫描的3名婴儿中就已出现这种情况。7名婴儿存在新生儿窒息，其中2名伴有脑室内出血。这些变化与该疾病常见特征智力发育迟缓之间的关系尚不清楚。