Ballard R, Tien R D, Nohria V, Juel V
Department of Radiology, Duke University Medical Center, Durham, NC 27710.
Pediatr Radiol. 1994;24(4):266-7. doi: 10.1007/BF02015453.
Chédiak-Higashi syndrome (CHS) is a rare autosomal recessive disorder postulated to result from lack of regulation of fusion of the primary lysosomes. In this report we present the MR and CT features of the brain in a patient with known CHS. These findings include diffuse atrophy of the brain with diffuse periventricular decreased density identified with CT, as well as increased signal on the T2-weighted images and lack of enhancement on the T1-weighted images in the periventricular and corona radiata regions.
切-东综合征（CHS）是一种罕见的常染色体隐性疾病，据推测是由于初级溶酶体融合缺乏调控所致。在本报告中，我们展示了一名已知患有CHS患者的脑部磁共振成像（MR）和计算机断层扫描（CT）特征。这些表现包括CT显示的弥漫性脑萎缩及脑室周围密度弥漫性降低，以及在T2加权图像上脑室周围和放射冠区域信号增强，在T1加权图像上无强化。
