铜缺乏性脊髓病临床分析

目的:探讨铜缺乏性脊髓病(CDM)的病因、临床表现及治疗方法。方法:回顾性分析7例铜缺乏性脊髓病患者的临床表现、辅助检查及治疗效果。结果:7例患者均有后索、侧索和周围神经损害,血清铜浓度降低;7例肌电图提示周围神经损害,体感诱发电位异常;2例MRI有胸髓改变。补铜治疗有效。结论:铜缺乏性脊髓病是以铜缺乏导致的后索、侧索及周围神经损害为主的疾病,早期诊断是治疗CDM的关键。

Clinical Analysis of Copper Deficiency Myelopathy

Aim： To explore etiological factors, the clinical manifestations and therapy of copper deficiency myelopathy（CDM）. Methods： The data of 7 CDM patients were retrospectively analyzed on the clinical manifestations, results of accessory examinations, therapy and conversion. Results： 7 patients were with damaged posterior funiculus and lateral funiculus of the spinal cord, peripheral nerves. Serum copper assay of 7 patients were lower than that of health persons. Examination of electromyogram in the 7 patients showed that there were abnormal in the peripheral nerves and abnormal somatosensory evoked potentials（SEP）. Among 7 patients received spine magnetic resonance imaging （MRI）, there were 2 with T1 abnormal signal and long T2 abnormal signal, 2 with no reinforcement in enhancement scanning. Combining copper therapy can imporve CDM evidently. Conclusion： CDM is a disease characterized mainly with posterior funiculus, lateral funiculus and peripheral nerves damage due to copper deficiency. Early diagnosis and therapy is the key.