Magnetic resonance imaging and magnetic resonance spectroscopy in a patient with amyotrophic lateral sclerosis and frontotemporal dementia.
Magnetic resonance imaging and magnetic resonance spectroscopy in a patient with amyotrophic lateral sclerosis and frontotemporal dementia.
复制标题
肌萎缩侧索硬化症和额颞叶痴呆患者的磁共振成像和磁共振波谱。
作者：
H. Šarac;M. Žagar;D. Vranješ;N. Henigsberg;E. Bilic;G. Pavliša
磁共振成像(MRI)和磁共振波谱(MRS)已在单一神经退行性疾病中进行了研究，但尚未在ALS合并FTD(ALS-FTD)等混合性疾病中进行检测。据我们所知，这项研究是第一次尝试证明MRI异常与运动皮质、额叶白质和皮质脊髓束的MR波谱代谢物改变之间的关系，该患者被诊断为ALS可能有上运动神经元症状(ALS-PUMNS)和FTD。患者行头颅MRI和MRS检查，根据峰面积测量值计算N-乙酰天冬氨酸(NAA)/肌酸(Cr)、胆碱/肌酸(Choline/Cr)、肌醇(Ml)/肌酸(Ml)和谷氨酰胺(Glx)/肌酸(Cr)的比值。获得了右侧运动皮质、额叶白质和皮质脊髓束的波谱。MRI检查包括中央沟扩大、皮质脊髓束高信号和额叶萎缩。光谱显示NAA/Cr和GLX/Cr比值降低，而Cho/Cr比值显著升高。MRI表现为中央沟增大，皮质脊髓束高信号，双侧额叶萎缩。当前患者的质子光谱代谢变化与先前报道的仅在ALS中的MRS代谢变化完全相关。令人惊讶的是，除了额叶白质，几乎所有测量的感兴趣体素都发现了正常的m1(胶质标记)值。这些发现不同于之前仅在ALS或FTD中的发现。总之，这些发现支持ALS、FTD和ALS-FTD可能代表单一病理连续体的不同表现的概念。
Magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) have been investigated in a single neurodegenerative disease manifesting as either amyotrophic lateral sclerosis (ALS) or frontotemporal dementia (FTD) alone, but have not been examined in combined disorders such as ALS with FTD (ALS-FTD). To our knowledge, this study is the first attempt to demonstrate relationship between MRI abnormalities and MR spectroscopic metabolite changes of the motor cortex, frontal white matter and corticospinal tract in a patient with the diagnosis of ALS with probable upper motor neuron signs (ALS-PUMNS) and FTD. Patient presented underwent MRI of the brain and MRS. The ratio of N-acetylaspartate (NAA) to creatine (Cr), choline to Cr, myo-inositol (ml) to Cr and glutamate-glutamine (Glx) to Cr were derived from peak area measurement. Spectra from the right motor cortex, frontal white matter and corticospinal tract were obtained. MR images were evaluated for sulcus centralis enlargement, corticospinal tract hyperintensity and frontal lobes atrophy. Spectra showed reduced NAA/Cr and Glx/Cr ratio, yet the ratio of Cho/Cr exhibited significant elevation. MR images revealed sulcus centralis enlargement, high signal intensity of corticospinal tract and atrophy of both frontal lobes. Proton spectroscopic metabolic changes in a current patient fully correlate with previously reported MRS metabolic changes in ALS alone. Surprisingly, normal ml (glial marker) values have been found in almost all measured voxels of interest except in the frontal white matter. These findings differ from the previous findings in ALS or FTD alone. In conclusion, these findings support the concept that ALS, FTD and ALS-FTD may represent different manifestations of a single pathological continuum.