Ellis C M, Simmons A, Andrews C, Dawson J M, Williams S C, Leigh P N
Department of Clinical Neurosciences, Institute of Psychiatry and King's Healthcare, London, UK.
Neurology. 1998 Oct;51(4):1104-9. doi: 10.1212/wnl.51.4.1104.
To evaluate neuronal dysfunction in the motor region subcortical white matter in ALS using volumetric localized proton magnetic resonance spectroscopy (1H-MRS).
Sixteen patients with E1 Escorial definite, probable, or possible ALS and eight healthy age-matched control subjects were studied. The ALS patients were divided into those with limb onset (n = 8) and those with bulbar onset (n = 8). Measurements of the metabolic ratios N-acetylaspartate (NAA)/creatine and phosphocreatine (Cr+PCr), NAA/choline (Cho), and Cho/(Cr+PCr) were correlated with clinical assessments.
We found no differences in the metabolic peak area ratios in the motor region when comparing the total ALS group and the control subjects. However, correlations were found between the NAA/(Cr+PCr) ratio and the E1 Escorial category (p = 0.03), the ALS severity scale (p = 0.01), and the Medical Research Council score (p = 0.06). No correlations were found between the NAA/(Cr+PCr) ratio and the Ashworth Spasticity Scale, reflex score, or disease duration (p > 0.16). Bulbar-onset patients had a lower NAA/(Cr+PCr) ratio in the motor region compared with limb-onset patients (p = 0.03).
In vivo 1H-MRS of the subcortical white matter in the motor region is unlikely to be sensitive enough to detect early disease changes in ALS because there is considerable overlap between the metabolic peak area ratios from patients with ALS and normal control subjects. However, changes in the NAA/(Cr+PCr) metabolic peak area ratios correlate with clinical measures of disease severity, and this measure may be useful in monitoring disease progression.
采用容积定位质子磁共振波谱(1H-MRS)评估肌萎缩侧索硬化症(ALS)患者运动区皮质下白质的神经元功能障碍。
对16例埃斯科里亚尔标准(E1)确诊、可能或疑似ALS的患者以及8名年龄匹配的健康对照者进行研究。将ALS患者分为肢体起病组(n = 8)和延髓起病组(n = 8)。代谢比率N-乙酰天门冬氨酸(NAA)/肌酸和磷酸肌酸(Cr+PCr)、NAA/胆碱(Cho)以及Cho/(Cr+PCr)的测量结果与临床评估相关联。
比较ALS总组与对照者时,我们发现运动区的代谢峰面积比率没有差异。然而,发现NAA/(Cr+PCr)比率与E1埃斯科里亚尔分类(p = 0.03)、ALS严重程度量表(p = 0.01)以及医学研究委员会评分(p = 0.06)之间存在相关性。未发现NAA/(Cr+PCr)比率与Ashworth痉挛量表、反射评分或疾病持续时间之间存在相关性(p > 0.16)。与肢体起病患者相比,延髓起病患者运动区的NAA/(Cr+PCr)比率更低(p = 0.03)。
运动区皮质下白质的活体1H-MRS不太可能足够敏感以检测出ALS早期疾病变化,因为ALS患者与正常对照者的代谢峰面积比率存在相当大的重叠。然而,NAA/(Cr+PCr)代谢峰面积比率的变化与疾病严重程度的临床指标相关,并且该指标可能有助于监测疾病进展。