Vasconcellos E, Smith M
Department of Neurology, Vanderbilt University Medical Center, Nashville, Tennessee 37212, USA.
Pediatr Neurol. 1998 Aug;19(2):151-2. doi: 10.1016/s0887-8994(98)00033-2.
Krabbe disease is characterized by abnormal breakdown and turnover of myelin, leading to extensive demyelination in both the peripheral and central nervous systems. A 7-month-old infant with early-onset Krabbe disease had deceptively normal head images, but spinal MRI demonstrated abnormal gadolinium enhancement of the lumbosacral sacral nerve roots and cauda equina such as that seen in Guillain-Barré syndrome. Abnormal enhancement in spinal MRI has not been previously described in patients with leukodystrophies.
克拉伯病的特征是髓鞘异常分解和更新,导致外周和中枢神经系统广泛脱髓鞘。一名患有早发性克拉伯病的7个月大婴儿的头部影像看似正常,但脊髓磁共振成像(MRI)显示腰骶神经根和马尾神经钆增强异常,类似于格林-巴利综合征所见。以前在脑白质营养不良患者中尚未描述过脊髓MRI的异常增强。