Benkirane Adam, Warlop Thibault, Ivanoiu Adrian, Baret Pierre, Wiame Elsa, Haufroid Vincent, Duprez Thierry, Hantson Philippe
Department of Neurology, Cliniques Universitaires St-Luc, Brussels, Belgium.
Institute of Neurosciences, Université Catholique de Louvain, Brussels, Belgium.
Front Neurol. 2023 Jan 4;13:1063803. doi: 10.3389/fneur.2022.1063803. eCollection 2022.
Copper deficiency is an acquired condition that can lead to neurologic dysfunctions, such as myelopathy, motor neuron impairment, polyneuropathy, cognitive impairment, and optic nerve neuropathy. Associated biological findings are low serum copper and ceruloplasmin levels with low copper urinary excretion. We report the case of a previously healthy 59-year-old man who presented a complex neurological picture starting with symptoms and radiological signs consistent with degenerative myelopathy in the presence of persisting low serum copper and ceruloplasmin despite oral and intravenous copper supplementation. Over time, his symptoms evolved into a motor neuron disease evocating an amyotrophic lateral sclerosis (ALS) phenotype. The potential role of copper deficiency is discussed, together with the difficulties in biomonitoring copper supplementation.
铜缺乏是一种后天获得性疾病，可导致神经功能障碍，如脊髓病、运动神经元损伤、多发性神经病、认知障碍和视神经神经病。相关生物学表现为血清铜和铜蓝蛋白水平降低，尿铜排泄减少。我们报告了一例59岁既往健康男性的病例，该患者出现复杂的神经系统症状，最初的症状和影像学表现与退行性脊髓病一致，尽管口服和静脉补充铜，但血清铜和铜蓝蛋白水平持续偏低。随着时间的推移，他的症状演变成一种运动神经元疾病，呈现出肌萎缩侧索硬化（ALS）表型。本文讨论了铜缺乏的潜在作用，以及生物监测铜补充的困难。
