Investigation on the element disorders in the pathogenesis of neurodegenerative diseases is restricted by the difficulties of quantifying a number of trace elements in small amount of tissues and obtaining fresh specimens of human central nerve tissues. We established a sensitive method to quantify multi-elements in trace level using inductively coupled plasma mass spectrometry (ICP-MS). Then, we investigated whether formalin-preserved samples, which are easier to obtain than the fresh ones, can be used to differentiate the element concentrations between the patients and the control. The concentrations of Cu, Hg and Se were found to be stable, While the concentrations of Mg, Mn and Zn decreased and those of Al, As, Cd, Co, Fe, Ni, Pb and V increased during preservation. Based on these results, we evaluated regional element concentrations in the spinal cord of formalinized and fresh samples of amyotrophic lateral sclerosis (ALS) patients. In the formalinized samples of the patients, Cu, Se, Mg, and Zn were higher in the anterior horn. In fresh samples of a familial ALS patient, the concentrations of Mn, Fe, Zn and Co were high in all 4 regions in the spinal cord. The high concentrations of Mn were notable, which was also higher in the brain frontal lobe and cerebellum.