Marino S, De Luca M, Dotti M T, Stromillo M L, Formichi P, Galluzzi P, Mondelli M, Bramanti P, Federico A, De Stefano N
Department of Neurological and Behavioral Sciences, University of Siena, Siena, Italy.
Neurology. 2007 Sep 18;69(12):1261-9. doi: 10.1212/01.wnl.0000276945.92950.69.
Cerebral involvement is usually absent in pure adrenomyeloneuropathy (AMN). Recently, nonconventional MR studies have reported brain abnormalities in patients with pure AMN, providing evidence that occult cerebral involvement may occur in this disease. It remains unclear, however, whether these brain abnormalities reflect centripetal extension of spinal cord long-tract axonopathy or can be the expression of a pathologic process largely involving the brain.
Conventional MRI and proton MR spectroscopic imaging (1H-MRSI) data of four patients with pure AMN were compared to those of four men with spinal cord injury (SCI) and 10 age-matched healthy men (HM). Resonance intensity areas of N-acetylaspartate (NAA) and choline were calculated as ratios to creatine (Cr) in voxels located in white matter (WM) regions. Functional MRI (fMRI) data during simple motor task were obtained in a separate session in three patients with AMN and three age-matched HM.
Conventional MRI examinations were normal in all patients. On 1H-MRSI, NAA/Cr values were lower in all WM regions of patients with AMN than in those of patients with SCI (p < 0.05) and HM (p < 0.01). In contrast, patients with SCI showed NAA/Cr values lower than HM only in the periventricular WM (p = 0.04). At fMRI, patients with AMN showed a more pronounced activation than HM in all movement-associated cortical regions contralateral to the hand moved and an exclusive voxel activation of the primary motor, somatosensory, and posterior parietal cortices ipsilateral to the hand moved.
CNS damage in pure adrenomyeloneuropathy is not confined exclusively to spinal cord and seems to primarily involve the brain.
在单纯性肾上腺脊髓神经病（AMN）中通常不存在脑受累情况。最近，非传统的磁共振成像（MR）研究报告了单纯性AMN患者存在脑异常，这为该病可能存在隐匿性脑受累提供了证据。然而，目前尚不清楚这些脑异常是反映脊髓长束轴索性病变的向心性扩展，还是主要累及脑的病理过程的表现。
将4例单纯性AMN患者的传统MRI和质子磁共振波谱成像（1H-MRSI）数据与4例脊髓损伤（SCI）男性患者以及10例年龄匹配的健康男性（HM）的数据进行比较。计算位于白质（WM）区域的体素中N-乙酰天门冬氨酸（NAA）和胆碱的共振强度面积与肌酸（Cr）的比值。在单独的一次检查中，对3例AMN患者和3例年龄匹配的HM进行了简单运动任务期间的功能MRI（fMRI）数据采集。
所有患者的传统MRI检查均正常。在1H-MRSI上，AMN患者所有WM区域的NAA/Cr值均低于SCI患者（p<0.05）和HM（p<0.01）。相比之下，SCI患者仅在脑室周围WM区域的NAA/Cr值低于HM（p = 0.04）。在fMRI检查中，AMN患者在与手部运动对侧的所有运动相关皮质区域的激活比HM更明显，并且在与手部运动同侧的初级运动、躯体感觉和顶叶后皮质区域出现了独特的体素激活。
单纯性肾上腺脊髓神经病中的中枢神经系统损伤并非仅局限于脊髓，似乎主要累及脑。
