Department of Internal Medicine, Division of Neurology, Hyogo College of Medicine, 1-1, Mukogawa-cho, Nishinomiya, Hyogo 663-8501, Japan.
J Neurol Sci. 2009 Oct 15;285(1-2):146-8. doi: 10.1016/j.jns.2009.06.026. Epub 2009 Jul 12.
Recent studies have suggested that the elevation of intracellular chloride contributes to excitotoxic cell death in motor neuron and can be related to the pathogenesis of amyotrophic lateral sclerosis (ALS). We investigated whether chloride levels in cerebrospinal fluid (CSF) and serum were lower in ALS patients than in control patients with other neurological diseases (OND). We also examined the relationship between chloride levels and clinical ALS phenotypes. We measured chloride levels (CSF and serum) in 27 ALS patients and 33 age- and gender-matched OND controls admitted to our hospital for diagnosis. The CSF chloride levels were lower in ALS patients (117 [range 102-130] mmol/L) than in OND controls (126 [range 114-134] mmol/L) (P<0.0001). However, no significant difference was found in their serum chloride levels (P>0.05). There was no significant difference in CSF chloride levels among the sub-groups of ALS patients classified according to their age, gender, duration of illness, clinical state and type of onset (P>0.05). CSF chloride levels already significantly decreased in ALS patients at the time of diagnosis. We conclude that the elevation of intracellular chloride would cause the reduction of chloride in CSF and be related to the pathogenesis of ALS.
最近的研究表明，细胞内氯离子的升高与运动神经元的兴奋性细胞死亡有关，并且可能与肌萎缩侧索硬化症（ALS）的发病机制有关。我们研究了脑脊液（CSF）和血清中的氯离子水平在 ALS 患者中是否低于其他神经系统疾病（OND）的对照组患者。我们还检查了氯离子水平与 ALS 临床表型之间的关系。我们测量了 27 名 ALS 患者和 33 名年龄和性别匹配的 OND 对照组患者的脑脊液（CSF）和血清中的氯离子水平，这些患者因诊断而被收入我院。与 OND 对照组（126 [范围 114-134] mmol/L）相比，ALS 患者的 CSF 氯离子水平较低（117 [范围 102-130] mmol/L）（P<0.0001）。然而，血清氯离子水平没有显著差异（P>0.05）。根据年龄、性别、疾病持续时间、临床状态和发病类型对 ALS 患者进行分组后，CSF 氯离子水平没有显著差异（P>0.05）。在诊断时，ALS 患者的 CSF 氯离子水平已经显著降低。我们的结论是，细胞内氯离子的升高会导致 CSF 中氯离子的减少，与 ALS 的发病机制有关。
