Paltiel H J, O'Gorman A M, Meagher-Villemure K, Rosenblatt B, Silver K, Watters G V
Radiology. 1987 Jan;162(1 Pt 1):115-8. doi: 10.1148/radiology.162.1.3786750.
Leigh disease, or subacute necrotizing encephalomyelopathy (SNE), is a familial, degenerative disorder characterized by lesions of the gray and white matter in the brain and spinal cord. Low attenuation in the putamina on computed tomography (CT) scans is considered to be characteristic of the disease. The authors used CT to study five patients, in three of whom the disease was confirmed histologically. In one of the patients with documented SNE, there was extensive gray matter lucency with normal basal ganglia. CT scans obtained in a second patient showed diffuse, diseased white matter with focal cortical extension and bilateral caudate involvement. In the other three cases, CT scans revealed the usual changes that occur in the basal ganglia. The appearance of SNE on CT scans thus reflects the variable and widespread distribution of the disease. The absence of radiologically detectable abnormalities in the basal ganglia should not deter one from the diagnosis of Leigh disease given in the appropriate clinical context.
Leigh病，即亚急性坏死性脑脊髓病（SNE），是一种家族性退行性疾病，其特征为脑和脊髓的灰质和白质病变。计算机断层扫描（CT）显示壳核低衰减被认为是该病的特征。作者使用CT对5例患者进行研究，其中3例经组织学确诊。在1例确诊为SNE的患者中，灰质广泛透亮，基底神经节正常。第2例患者的CT扫描显示弥漫性病变的白质，伴有局灶性皮质延伸和双侧尾状核受累。在其他3例中，CT扫描显示基底神经节出现常见变化。因此，SNE在CT扫描上的表现反映了该病的多变和广泛分布。在适当的临床背景下，基底神经节在放射学上未检测到异常不应排除Leigh病的诊断。
