Baram T Z, Goldman A M, Percy A K
Neurology. 1986 Jan;36(1):111-5. doi: 10.1212/wnl.36.1.111.
In three patients with Krabbe disease (galactosylceramide lipidosis), CT and MRI patterns progressed with the evolution of the disease. At first, discrete and symmetric dense areas on CT were found in deep gray matter of hemispheres and brainstem, and also in periventricular and capsular white matter. MRI showed decreased T1 values with normal or slightly decreased T2 values in those areas and large symmetric "plaque-like" lesions with high T1 and T2 values in white matter of the centrum semiovale. Later, both CT and MRI revealed diffuse reduction in gray matter and, more profoundly, in white matter mass. These findings may alert clinicians to the possibility of Krabbe disease in infants with progressive encephalopathy.
在3例克拉伯病(半乳糖神经酰胺脂质沉积症)患者中,CT和MRI表现随疾病进展而变化。起初,CT显示半球深部灰质、脑干以及脑室周围和囊下白质出现散在、对称的致密区。MRI显示这些区域T1值降低,T2值正常或略有降低,半卵圆中心白质出现大的对称“斑片状”T1和T2高信号病变。后来,CT和MRI均显示灰质弥漫性减少,白质减少更为明显。这些发现可能提醒临床医生,对于患有进行性脑病的婴儿,要警惕克拉伯病的可能性。