Wang C, Melberg A, Weis J, Månsson J-E, Raininko R
Department of Radiology, Uppsala University, Uppsala, Sweden.
Acta Neurol Scand. 2007 Oct;116(4):268-72. doi: 10.1111/j.1600-0404.2007.00867.x.
Adult-onset Krabbe disease is an uncommon form of leukodystrophy. Its magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) manifestations are not well documented.
To describe early MR findings in adult-onset Krabbe disease.
A 28-year-old woman who had spastic paraparesis and a 5-year history of gait problems underwent MRI of the brain and cervical spine. Proton MRS was performed at 1.5 T using a short echo time. Metabolites were analyzed in the areas of MR signal abnormalities and normal-appearing brain. Six healthy volunteers were examined as controls.
MRI revealed changes in the upper corticospinal tracts, splenium and, minimally, adjacent to the atria of the lateral ventricles. MRS showed decreased creatine, choline, N-acetylaspartate and glutamate and probably elevated lactate in the upper corticospinal tract but not in the normal-appearing frontal lobe. The spinal cord was thin. Laboratory tests verified Krabbe disease.
These results indicate early involvement of the upper corticospinal tract in adult-onset Krabbe disease. The cases reported earlier had imaging changes indicating a more advanced disease or no MR findings. Thinning of the spinal cord is a new finding in Krabbe disease.
成人型克拉伯病是一种罕见的脑白质营养不良形式。其磁共振成像（MRI）和磁共振波谱（MRS）表现尚无充分记录。
描述成人型克拉伯病的早期磁共振成像表现。
一名28岁患有痉挛性截瘫且有5年步态问题病史的女性接受了脑部和颈椎的MRI检查。使用短回波时间在1.5T下进行质子MRS检查。在磁共振信号异常区域和外观正常的脑区分析代谢物。选取6名健康志愿者作为对照进行检查。
MRI显示皮质脊髓束上部、胼胝体以及侧脑室房部附近有轻微改变。MRS显示皮质脊髓束上部肌酸、胆碱、N-乙酰天门冬氨酸和谷氨酸减少，乳酸可能升高，但在外观正常的额叶未见此情况。脊髓变细。实验室检查确诊为克拉伯病。
这些结果表明成人型克拉伯病早期累及皮质脊髓束上部。早期报道的病例有影像学改变，提示病情更严重或无磁共振成像表现。脊髓变细是克拉伯病的一项新发现。
