Patten B M, Kurlander H M, Evans B
Acta Neurol Scand. 1982 Nov;66(5):594-9. doi: 10.1111/j.1600-0404.1982.tb03148.x.
Because some investigators have reported abnormal concentrations of amino acids (AA) in fluids and tissues of patients with motor neuron disease (MND), we examined the AA content of frozen anterior horn spinal cord tissue taken from seven patients dying of MND and compared the results with those found in 12 control patients. Ammonia (21 +/- 8.1 vs. 12.7 +/- 6.9 mumol/g, P = 0.036) and ornithine (0.41 +/- 0.3 vs. 0.16 +/- 0.09, P = 0.036) were elevated in spinal tissue of motor neuron disease patients. Correlation analysis showed ammonia levels inversely related to duration of illness (r = -0.714, P = 0.036). We concluded that metabolic abnormalities exist in MND. Ammonia and ornithine may be adversely affecting motor neuron function, or alternatively they could be metabolic markers of a more generalized energy-deficient state in motor neuron disease.
由于一些研究人员报告了运动神经元病（MND）患者的体液和组织中氨基酸（AA）浓度异常，我们检测了7例死于MND的患者的冷冻脊髓前角组织中的AA含量，并将结果与12例对照患者的结果进行了比较。运动神经元病患者脊髓组织中的氨（21±8.1对12.7±6.9μmol/g，P = 0.036）和鸟氨酸（0.41±0.3对0.16±0.09，P = 0.036）升高。相关性分析显示氨水平与病程呈负相关（r = -0.714，P = 0.036）。我们得出结论，MND存在代谢异常。氨和鸟氨酸可能对运动神经元功能产生不利影响，或者它们可能是运动神经元病中更普遍的能量缺乏状态的代谢标志物。
