Tourbah A, Stievenart J L, Iba-Zizen M T, Lubetzki C, Baumann N, Eymard B, Moser H W, Lyon-Caen O, Cabanis E A
Service de Neuroradiologie, Centre Hospitalier National d'Ophtalmologie des XV-XX, Paris, France.
Arch Neurol. 1997 May;54(5):586-92. doi: 10.1001/archneur.1997.00550170062015.
To describe the changes in the results of magnetic resonance imaging and spectroscopy occurring in the normal-appearing white matter of patients with adult adrenoleukodystrophy and to present evidence of a particular change that may serve as a marker for the follow-up of the disease.
Neurologic, magnetic resonance imaging, and localized proton spectroscopic examinations were performed in 11 patients with adult adrenoleukodystrophy and compared with 11 sex- and age-matched controls.
Eleven patients with adult adrenoleukodystrophy participated in a trial of dietary therapy with glyceryl trioleate and glyceryl trierucate (Lorenzo's oil) in the Fédération de Neurologie and the Institut National de la Santé et de la Recherche Médicale, Unité 134, at the Hôpital de la Salpêtrière in Paris, France.
The results of magnetic resonance imaging of the white matter were normal in 2 patients and showed areas of mild symmetrical hypersignals on T2-weighted images and fluid attenuated inversion recovery sequences, localized in the posterior white matter in 9 patients. The results of spectroscopy indicated that the peak of the area of choline-containing compounds was increased at long echo times in patients with adult adrenoleukodystrophy, which may reflect very long-chain fatty acid accumulation in this disease. The peak of the area of myo-inositol-containing compounds was increased at short echo times in patients with adult adrenoleukodystrophy, which may indicate a rise in this metabolite concentration. The N-acetylaspartate-creatine amplitude ratio was significantly decreased in patients with motor deficit. The significance of this finding remains to be established.
The results of localized proton magnetic resonance spectroscopy show abnormalities in the cerebral white matter of patients with adult adrenoleukodystrophy, which may contribute to the understanding of the pathophysiologic characteristics of the disease. Although changes in the results of spectroscopy found in this disease are not specific, the increase of choline-containing compounds may reflect the accumulation of very long-chain fatty acids in the central nervous system. Localized proton magnetic resonance spectroscopy may prove a valuable technique, in addition to magnetic resonance imaging, for noninvasive investigation of patients with adult adrenoleukodystrophy undergoing future clinical trials.
描述成年肾上腺脑白质营养不良患者正常表现白质的磁共振成像和波谱分析结果的变化，并提供一种可能作为该疾病随访标志物的特定变化的证据。
对11例成年肾上腺脑白质营养不良患者进行神经学、磁共振成像和局部质子波谱检查，并与11例性别和年龄匹配的对照者进行比较。
11例成年肾上腺脑白质营养不良患者参与了在法国巴黎萨尔佩特里埃医院神经学联合会和国家健康与医学研究所第134单元进行的甘油三油酸酯和甘油三芥酸酯（洛伦佐油）饮食疗法试验。
2例患者白质的磁共振成像结果正常，9例患者在T2加权图像和液体衰减反转恢复序列上显示轻度对称性高信号区域，位于后白质。波谱分析结果表明，成年肾上腺脑白质营养不良患者在长回波时间时含胆碱化合物区域的峰升高，这可能反映了该疾病中极长链脂肪酸的积累。成年肾上腺脑白质营养不良患者在短回波时间时含肌醇化合物区域的峰升高，这可能表明该代谢物浓度升高。运动功能缺损患者的N-乙酰天门冬氨酸-肌酸振幅比显著降低。这一发现的意义尚待确定。
局部质子磁共振波谱分析结果显示成年肾上腺脑白质营养不良患者脑白质存在异常，这可能有助于理解该疾病的病理生理特征。虽然该疾病中波谱分析结果的变化不具有特异性，但含胆碱化合物的增加可能反映了中枢神经系统中极长链脂肪酸的积累。除磁共振成像外，局部质子磁共振波谱分析可能是一种有价值的技术，用于对未来临床试验中的成年肾上腺脑白质营养不良患者进行无创研究。
