Vanderbilt University School of Medicine, 1161 21st Avenue S, Nashville, TN 37232, USA; Department of Neurosurgery, Baylor College of Medicine, 7200 Cambridge St., Houston, TX 77030, USA.
Department of Neurological Surgery, Vanderbilt University Medical Center, 1500 21st Avenue S, Nashville, TN 37212, USA.
Spine J. 2024 Nov;24(11):2026-2034. doi: 10.1016/j.spinee.2024.06.018. Epub 2024 Jun 21.
Copper deficiency myelopathy (CDM) is a rare disease that can present with spastic quadriparesis and sensory ataxia. As a result, it can precisely mimic cervical spondylitic myelopathy (CSM). Copper deficiency may be seen following gastric bypass surgery, malabsorption syndromes such as celiac disease, and with excessive exogenous zinc intake. We present a systematic review of the literature for CDM and an illustrative case.
Provide a systematic review of CDM to highlight the importance of recognizing the consideration of CDM in patients presenting to a spine surgeon with myelopathy that progress despite adequate surgical decompression, or myelopathy concomitant with cytopenia, thus requiring further workup.
STUDY DESIGN/SETTING: Retrospective medical record review and systematic review of the literature.
PubMed and Ovid-Embase database search was conducted in July 2022.
Self-reported measures include PRISMA flow diagram for retrospective review; Physiological measures include retrospective review of MRI imaging of cervical spine; alternate demographic and laboratory value data extracted via literature review.
A PubMed and Ovid-Embase database search was conducted in July 2022 searching for "copper deficiency myelopathy (MeSH)" from 2000 to 2022 via PRISMA guidelines. Following title and abstract review, the following data was extracted from full text: age, sex, etiology, hematological values upon presentation (mean corpuscular volume, white blood count, platelet count, and hemoglobin level), metal serum studies (serum copper, ceruloplasmin, and zinc), 24-hour collection of copper and zinc, and distinct radiographic findings on MRI.
A total of 116 studies were included in this review which contained 198 cases of copper deficiency myelopathy. The mean age was 53.57±14.14 years, with the majority being females (63.8%). The most common etiology was prior gastric surgery (n=55, 36.2 %) followed by excessive zinc consumption from the use of zinc denture cream (n=39, 19.9%). The mean serum copper was 15.67±17.84 (normal=80.0-155.0) mcg/dL and mean ceruloplasmin was 6.43±5.25 (normal=16-45) mg/dL. In spite of appropriate treatment with copper supplementation, only 47 cases (24%) reported improvement in neurological status, and only 10 (5.1%) recovered to baseline. A hyperintense T2 signal abnormality resembling an inverted "v" in the dorsal columns was the most common radiographic abnormality.
Pertinent risk factors for copper deficiency myelopathy include prior upper gastrointestinal surgery, zinc excess, and malabsorption. Characteristic laboratory and imaging findings include cytopenia, low serum copper and ceruloplasmin, and distinct inverted "v" T2 signal hyperintensity in the dorsal columns. The neurologic deterioration with copper deficiency will progress in spite of decompressive surgery, and can be devastating and irreversible even with copper supplementation, reinforcing the importance of early detection. We thus recommend patients with myelopathy presenting with a history of gastric bypass, malabsorption syndromes, excessive zinc exposure, cytopenia, or imaging resembling an inverted "v" shaped hyperintense T2 MRI signal in the dorsal columns, should first undergo blood tests for copper, ceruloplasmin, and B12 levels prior to surgical consideration.
铜缺乏性脊髓病（CDM）是一种罕见的疾病，可表现为痉挛性四肢瘫痪和感觉性共济失调。因此，它可以精确地模拟颈椎脊髓病（CSM）。铜缺乏可能发生在胃旁路手术后、乳糜泻等吸收不良综合征以及过量摄入外源性锌后。我们对 CDM 的文献进行了系统回顾，并提供了一个说明性病例。
对 CDM 进行系统回顾，以强调在出现脊髓病的患者中认识到 CDM 的重要性，这些患者尽管进行了充分的手术减压，但病情仍在进展，或者脊髓病同时伴有细胞减少症，因此需要进一步检查。
研究设计/背景：回顾性病历审查和文献系统回顾。
2022 年 7 月在 PubMed 和 Ovid-Embase 数据库中进行了搜索，检索词为“铜缺乏性脊髓病（MeSH）”，时间范围为 2000 年至 2022 年，遵循 PRISMA 指南。在标题和摘要审查后，从全文中提取了以下数据：年龄、性别、病因、发病时的血液学值（平均红细胞体积、白细胞计数、血小板计数和血红蛋白水平）、金属血清研究（血清铜、铜蓝蛋白和锌）、24 小时铜和锌收集以及 MRI 上的独特放射学发现。
本综述共纳入 116 项研究，包含 198 例铜缺乏性脊髓病病例。平均年龄为 53.57±14.14 岁，大多数为女性（63.8%）。最常见的病因是既往胃手术（n=55，36.2%），其次是因使用锌牙托膏导致锌摄入过量（n=39，19.9%）。平均血清铜为 15.67±17.84（正常范围为 80.0-155.0）μg/dL，平均铜蓝蛋白为 6.43±5.25（正常范围为 16-45）mg/dL。尽管进行了适当的铜补充治疗，但只有 47 例（24%）报告神经状态改善，只有 10 例（5.1%）恢复到基线水平。最常见的放射学异常是类似于“倒 V”形的背柱高信号 T2 异常。
铜缺乏性脊髓病的相关危险因素包括上消化道手术、锌过量和吸收不良。特征性的实验室和影像学发现包括细胞减少症、血清铜和铜蓝蛋白降低，以及背柱 T2 信号倒置“V”形高信号。尽管进行了减压手术，铜缺乏引起的神经恶化仍会进展，即使补充铜也可能是毁灭性的和不可逆的，这强调了早期发现的重要性。因此，我们建议出现胃旁路手术、吸收不良综合征、过量锌暴露、细胞减少症或影像学表现为背柱高信号倒置“V”形的脊髓病患者，应在考虑手术前首先进行铜、铜蓝蛋白和 B12 水平的血液检查。
Zotero 插件