Pou-Serradell A, Ugarte-Elola A C
Department of Neurology, Hospital Nuestra Señora del Mar, Autonomous University of Barcelona, Spain.
Neurofibromatosis. 1989;2(4):218-26.
Cerebral ventricular dilation and/or frank hydrocephalus unrelated to brain tumors is not a rare feature of von Recklinghausen neurofibromatosis (NF-1). In our experience, it was observed in 23% of patients consulting for neurological problems. From 1984 to 1989, 30 patients with documented NF-1 were referred to for cranial or spinal magnetic resonance imaging (MRI). In 9 of these patients, clinical features or previous computed tomographic scanning revealed hydrocephalus or ventriculomegaly. One patient had biventricular hydrocephalus (enlarged lateral ventricles), 7 had triventricular hydrocephalus (aqueductal stenosis) and 1 had tetraventricular hydrocephalus (obstruction of the foramina of Magendie and Luschka). MRI showed anomalies in all 9 patients. The importance of MRI for the diagnosis, control and treatment of the hydrocephalus as well as for understanding the embryologic pathogenesis of these conditions is emphasized.
与脑肿瘤无关的脑室扩张和/或明显脑积水是冯雷克林霍增氏神经纤维瘤病（NF-1）的一个常见特征。根据我们的经验，在因神经系统问题就诊的患者中，有23%观察到这种情况。1984年至1989年期间，30例确诊为NF-1的患者接受了头颅或脊髓磁共振成像（MRI）检查。其中9例患者的临床特征或之前的计算机断层扫描显示有脑积水或脑室扩大。1例患者为双侧脑室积水（侧脑室扩大），7例为三脑室积水（导水管狭窄），1例为四脑室积水（马让迪孔和路施卡孔梗阻）。MRI显示所有9例患者均有异常。强调了MRI在脑积水的诊断、监测和治疗以及理解这些病症的胚胎发病机制方面的重要性。
