Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Hosur Road, Bangalore, 560029, India.
Neuroradiology. 2010 Nov;52(11):977-85. doi: 10.1007/s00234-010-0661-1. Epub 2010 Feb 20.
Proton ((1)H) magnetic resonance spectroscopy (MRS) changes are noted in Wilson's disease (WD). However, there are no studies regarding membrane phospholipid abnormality using (31)P MRS in these patients. We aimed to analyze the striatal spectroscopic abnormalities using (31)P and (1)H MRS in WD.
Forty patients of WD (treated, 29; untreated,11) and 30 controls underwent routine MR image sequences and in vivo 2-D (31)P and (1)H MRS of basal ganglia using an image-selected technique on a 1.5-T MRI scanner. Statistical analysis was done using Student's t test.
The mean durations of illness and treatment were 6.2 ± 7.4 and 4.8 ± 5.9 years, respectively. MRI images were abnormal in all the patients. (1)H MRS revealed statistically significant reduction of N-acetyl aspartate (NAA)/choline (Cho) and NAA/creatine ratios in striatum ((1)H MRS) of treated patients compared to controls. The mean values of phosphomonoesters (PME) (p < 0.0001), phosphodiesters (PDE) (p < 0.0001), and total phosphorus (TPh) (p < 0.0001) were elevated in patients compared to controls. Statistically significant elevated levels of ratio of PME/PDE (p = 0.05) observed in the striatum were noted in treated patients as compared to controls in the (31)P MRS study. The duration of illness correlated well with increased PME/PDE [p < 0.001], PME/TPh [p < 0.05], and PDE/TPh [p < 0.05] and decreased NAA/Cho [p < 0.05] ratios. There was correlation of MRI score and reduced NAA/Cho ratio with disease severity. The PME/PDE ratio (right) was elevated in the treated group [p < 0.001] compared to untreated group.
There is reduced breakdown and/or increased synthesis of membrane phospholipids and increased neuronal damage in basal ganglia in patients with WD.
在威尔逊病（WD）中可以观察到质子（1H）磁共振波谱（MRS）的变化。然而，目前还没有关于这些患者使用 31P MRS 检测膜磷脂异常的研究。我们旨在分析 WD 患者使用 31P 和 1H MRS 的纹状体光谱异常。
40 例 WD 患者（治疗组 29 例，未治疗组 11 例）和 30 例对照者在 1.5T MRI 扫描仪上使用图像选择技术进行常规磁共振图像序列和基底节区的体内 2D（31）P 和 1H MRS。使用学生 t 检验进行统计分析。
所有患者的 MRI 图像均异常。1H MRS 显示，与对照组相比，治疗组患者纹状体的 N-乙酰天冬氨酸（NAA）/胆碱（Cho）和 NAA/肌酸比值显著降低。与对照组相比，磷酸单酯（PME）（p<0.0001）、磷酸二酯（PDE）（p<0.0001）和总磷（TPh）（p<0.0001）的平均水平在患者中升高。与对照组相比，治疗组患者纹状体中 PME/PDE 比值（p=0.05）显著升高。与对照组相比，病程与 PME/PDE 比值升高[P<0.001]、PME/TPh [P<0.05]和 PDE/TPh [P<0.05]呈正相关，与 NAA/Cho 比值降低呈负相关。MRI 评分与 NAA/Cho 比值与疾病严重程度相关。与未治疗组相比，治疗组的 PME/PDE 比值（右侧）升高（p<0.001）。
WD 患者基底节区膜磷脂分解减少和/或合成增加，神经元损伤增加。
