背景:铜缺乏性脊髓病(CDM)是一种罕见的疾病,可伴有痉挛性四肢瘫痪和感觉共济失调。因此,它可以精确地模仿脊髓型颈椎病(CSM)。铜缺乏可以在胃旁路手术后看到,吸收不良综合征,如乳糜泻,以及过量的外源性锌摄入。我们对CDM的文献进行了系统的回顾,并举例说明了一个案例。
目的:提供对CDM的系统评价,以强调认识到对患有脊髓病的脊柱外科医生的患者考虑CDM的重要性,尽管有足够的手术减压,或脊髓病伴有血细胞减少症,因此需要进一步的工作。
方法:回顾性病历回顾和文献系统回顾患者样本:2022年7月进行了PubMed和Ovid-Embase数据库搜索。结果措施:自我报告的措施包括用于回顾性审查的PRISMA流程图;生理措施包括对颈椎MRI成像的回顾性审查;通过文献综述和实验室数据提取的替代人口统计学和实验室价值数据从2022年7月搜索的数据库中进行了:在标题和摘要评论之后,全文提取以下数据:年龄,性别,病因学,出现时的血液学值(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小时收集铜和锌,MRI上有明显的影像学表现。
结果:本综述共纳入116项研究,其中包含198例铜缺乏性脊髓病。平均年龄53.57±14.14岁,大多数是女性(63.8%)。最常见的病因是先前的胃手术(n=55,36.2%),其次是使用锌义齿乳膏消耗锌过量(n=39,19.9%)。平均血清铜是15.67±17.84(正常=80.0-155.0)mcg/dL,平均铜蓝蛋白是6.43±5.25(正常=16-45)mg/dL。尽管用铜补充剂进行了适当的治疗,只有47例(24%)报告神经系统状况改善,只有10人(5.1%)恢复到基线水平。类似于背柱中倒置的“v”的高强度T2信号异常是最常见的影像学异常。
结论:铜缺乏性脊髓病的相关危险因素包括先前的上消化道手术,锌过量,和吸收不良。特征性实验室和影像学表现包括血细胞减少症,低血清铜和铜蓝蛋白,背柱和明显的反向“v”T2信号高强度。尽管进行了减压手术,但缺乏铜的神经系统恶化仍将进展,即使补充铜,也可能是毁灭性的和不可逆转的,加强早期检测的重要性。因此,我们推荐有胃旁路手术史的脊髓病患者,吸收不良综合征,过量的锌暴露,血细胞减少,或成像类似于背柱中的倒置“V”形高强度T2MRI信号,应该首先接受铜的血液检查,铜蓝蛋白,和B12水平在手术前考虑。
BACKGROUND: 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.
OBJECTIVE: 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.
METHODS: Retrospective medical record review and systematic review of the literature PATIENT SAMPLE: PubMed and Ovid-Embase database search was conducted in July 2022 OUTCOME MEASURES: 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 METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.