Gibson Summer B, Kasarskis Edward J, Hu Nan, Pulst Stefan-M, Mendiondo Marta S, Matthews Dwight E, Mitsumoto Hiroshi, Tandan Rup, Simmons Zachary, Kryscio Richard J, Bromberg Mark B
a Department of Neurology , University of Utah , Salt Lake City , Utah , USA.
b Department of Neurology , University of Kentucky , Lexington , Kentucky , USA.
Amyotroph Lateral Scler Frontotemporal Degener. 2015;16(7-8):473-7. doi: 10.3109/21678421.2015.1062516. Epub 2015 Aug 27.
Our objective was to explore if creatine kinase (CK) levels correlate with survival in amyotrophic lateral sclerosis (ALS), and whether a correlation is independent of other well-studied predictors such as location of onset, gender, age, fat free mass, spasticity, cramps, and fasciculations. We analyzed data from 80 ALS patients from a 48-week non-interventional longitudinal multicenter nutrition study with long term follow-up. The overall mean CK was 214 ± 191.8 U/l (range 22-1992 U/l). Forty-five percent of patients had at least one high CK value (> 200 U/l), and about half maintained a high CK value, but there was no trend over the study period. Male gender and extremity onset were significantly associated with high CK. In univariate analysis, age, bioelectric impedance spectroscopy (BIS) fat free mass, spasticity, and fasciculations were not associated with CK level. There was an association between CK and muscle cramps (p < 0.001). In survival analysis, low CK (≤ 200 U/l) was associated with a longer overall survival (p = 0.02), when adjusting for location of onset, age, race, gender, BIS fat free mass, and study site. In conclusion, CK may be a useful marker for ALS survival, which has implications for clinical care and the design of future clinical trials.
我们的目的是探究肌酸激酶（CK）水平与肌萎缩侧索硬化症（ALS）患者生存率之间是否存在关联，以及这种关联是否独立于其他已充分研究的预测因素，如发病部位、性别、年龄、去脂体重、痉挛、抽筋和肌束震颤。我们分析了来自一项为期48周的非干预性纵向多中心营养研究且有长期随访的80例ALS患者的数据。CK的总体平均水平为214±191.8 U/l（范围为22 - 1992 U/l）。45%的患者至少有一次CK值升高（>200 U/l），约一半患者维持较高的CK值，但在研究期间没有明显趋势。男性和肢体发病与高CK水平显著相关。在单因素分析中，年龄、生物电阻抗光谱法（BIS）测量的去脂体重、痉挛和肌束震颤与CK水平无关。CK与肌肉抽筋之间存在关联（p<0.001）。在生存分析中，调整发病部位、年龄、种族、性别、BIS测量的去脂体重和研究地点后，低CK（≤200 U/l）与较长的总生存期相关（p = 0.02）。总之，CK可能是ALS患者生存的一个有用标志物，这对临床护理和未来临床试验的设计具有重要意义。
