Ikeda Ken, Hirayama Takehisa, Takazawa Takanori, Kawabe Kiyokazu, Iwasaki Yasuo
Department of Neurology, Toho University Omori Medical Center, Japan.
Intern Med. 2012;51(12):1501-8. doi: 10.2169/internalmedicine.51.7465. Epub 2012 Jun 15.
Previous studies have reported distinct serological profiles of lipid, urate and ferritin in Western patients with amyotrophic lateral sclerosis (ALS). We aimed to examine the levels of these serological factors and their relationship to disease progression in Japanese ALS patients.
Ninety-two patients with definite or probable ALS who fulfilled the revised El Escorial criteria were analyzed for clinical and serological variables. Serological data at the time diagnosed with ALS were compared to those of 92 age/sex/body mass index-matched healthy controls.
Compared to controls, urate and creatinine (Cr) levels were decreased and ferritin levels were increased significantly in sera of male and female patients with ALS. Significant increases of serum total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C) and triglyceride levels were found in female ALS patients. The annual decline of ALS Functional Rating Scale-Revised (ALS-FRS) and forced vital capacity (FVC) were inversely correlated with serum TC, LDL-C, Cr and urate levels, and were positively correlated with serum ferritin levels. Multivariate analysis showed that the rapid worsening of annual ALS-FRS and FVC was associated with serum levels of TC, LDL-C, Cr, urate and ferritin.
The present study indicated that serum levels of TC, LDL-C, Cr, urate and ferritin were correlated with clinical deterioration in ALS patients. These results are similar to those in Western patients. Metabolic and nutritional conditions of lipid, urate and iron could contribute to disease progression in ALS patients. Further studies investigating high nutrition diets and iron chelation for the treatment of ALS are warranted.
既往研究报道了西方肌萎缩侧索硬化(ALS)患者脂质、尿酸和铁蛋白的不同血清学特征。我们旨在研究这些血清学因素在日本ALS患者中的水平及其与疾病进展的关系。
对92例符合修订的埃尔埃斯科里亚尔标准的确诊或疑似ALS患者的临床和血清学变量进行分析。将确诊为ALS时的血清学数据与92例年龄、性别、体重指数匹配的健康对照者的数据进行比较。
与对照组相比,男性和女性ALS患者血清中的尿酸和肌酐(Cr)水平降低,铁蛋白水平显著升高。女性ALS患者血清总胆固醇(TC)、低密度脂蛋白胆固醇(LDL-C)和甘油三酯水平显著升高。ALS功能评定量表修订版(ALS-FRS)和用力肺活量(FVC)的年度下降与血清TC、LDL-C、Cr和尿酸水平呈负相关,与血清铁蛋白水平呈正相关。多变量分析显示,ALS-FRS和FVC的年度快速恶化与血清TC、LDL-C、Cr、尿酸和铁蛋白水平有关。
本研究表明,血清TC、LDL-C、Cr、尿酸和铁蛋白水平与ALS患者的临床恶化相关。这些结果与西方患者的结果相似。脂质、尿酸和铁的代谢及营养状况可能促使ALS患者疾病进展。有必要进一步研究高营养饮食和铁螯合疗法对ALS的治疗作用。