Meoded A, Poretti A, Yoshida S, Huisman T A G M
Division of Pediatric Radiology, The Johns Hopkins School of Medicine, Baltimore, Maryland 21287, USA.
Neuropediatrics. 2011 Feb;42(2):82-5. doi: 10.1055/s-0031-1279784. Epub 2011 Jun 14.
Vanishing white matter disease (VWM) is one of the most frequent inherited childhood white matter disorders. We present the brain and spinal cord disease progression on serial conventional magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) in a 4-year-old boy. Consecutive MRI examinations demonstrated a progression of the signal abnormalities in the cerebral white matter. Globally, apparent diffusion coefficient (ADC) values as well as axial and radial diffusivity increased over time, while fractional anisotropy (FA) values decreased. Involvement of the cervical posterior spinal tracts and mild global spinal cord atrophy was found. In conclusion, serial MRI and DTI studies may help to better understand the selective injury of the myelin and axons in VWM disease. These data may help in monitoring disease progression. Our data also show that complete neuroimaging work-up in VWM should also include the spinal cord.
消失性白质病（VWM）是儿童期最常见的遗传性白质疾病之一。我们展示了一名4岁男孩在系列传统磁共振成像（MRI）和扩散张量成像（DTI）上脑和脊髓疾病的进展情况。连续的MRI检查显示脑白质信号异常有进展。总体而言，表观扩散系数（ADC）值以及轴向和径向扩散率随时间增加，而分数各向异性（FA）值降低。发现颈后脊髓束受累及轻度全脊髓萎缩。总之，系列MRI和DTI研究可能有助于更好地理解VWM疾病中髓鞘和轴突的选择性损伤。这些数据可能有助于监测疾病进展。我们的数据还表明，VWM的完整神经影像学检查也应包括脊髓。
