Copper deficiency myelopathy.
Copper deficiency myelopathy.
复制标题
DOI：
10.1007/s00415-010-5511-x
复制
发表时间：
2010-06
影响因子：
6
通讯作者：
Winston GP
中科院分区：
文献类型：
--
作者：
Jaiser SR;Winston GP
获得性铜缺乏症被认为是一种罕见的原因贫血和中性粒细胞减少症超过半个世纪。铜缺乏性脊髓病（CDM）仅在过去十年中被描述，并且代表了非压迫性脊髓病的可治疗原因，其非常类似于由于维生素B12缺乏引起的亚急性联合变性。在这里，55例文献报道的人口统计学，病因学，血液学和生化参数，脊柱成像，治疗和结果进行了审查。铜代谢紊乱的病理生理进行了讨论。CDM最常出现在第五和第六个十年，更常见于女性（F：M = 3.6：1）。危险因素包括既往上消化道手术史、锌超载和吸收不良综合征，所有这些都会损害上消化道的铜吸收。20%的病例未确定病因。在一些不被认为是原发性锌超载的病例中检测到高锌水平，在这种情况下，锌对铜缺乏状态的贡献仍不清楚。在78%的患者中发现了细胞减少症，尤其是贫血，并且骨髓增生异常综合征可能在过去被误诊。47%的脊髓MRI异常，通常显示颈髓和胸髓后部的高T2信号。在临床上相容的情况下，CDM可以通过存在一种或多种风险因素和/或血细胞减少来提示。低血清铜和铜蓝蛋白水平证实了诊断，与威尔逊病相反，尿铜水平通常较低。治疗包括补充铜和改变任何风险因素，并导致血液学正常化和神经系统改善或稳定。由于任何神经系统恢复都是部分的，并且随着减肥胃肠道手术的使用越来越多，CDM的病例数将继续增加，因此临床警惕仍然是最大限度地减少神经系统后遗症的关键。提出了治疗和预防的建议。
Acquired copper deficiency has been recognised as a rare cause of anaemia and neutropenia for over half a century. Copper deficiency myelopathy (CDM) was only described within the last decade, and represents a treatable cause of non-compressive myelopathy which closely mimics subacute combined degeneration due to vitamin B12 deficiency. Here, 55 case reports from the literature are reviewed regarding their demographics, aetiology, haematological and biochemical parameters, spinal imaging, treatment and outcome. The pathophysiology of disorders of copper metabolism is discussed. CDM most frequently presented in the fifth and sixth decades and was more common in women (F:M = 3.6:1). Risk factors included previous upper gastrointestinal surgery, zinc overload and malabsorption syndromes, all of which impair copper absorption in the upper gastrointestinal tract. No aetiology was established in 20% of cases. High zinc levels were detected in some cases not considered to have primary zinc overload, and in this situation the contribution of zinc to the copper deficiency state remained unclear. Cytopenias were found in 78%, particularly anaemia, and a myelodysplastic syndrome may have been falsely diagnosed in the past. Spinal MRI was abnormal in 47% and usually showed high T2 signal in the posterior cervical and thoracic cord. In a clinically compatible case, CDM may be suggested by the presence of one or more risk factors and/or cytopenias. Low serum copper and caeruloplasmin levels confirmed the diagnosis and, in contrast to Wilson’s disease, urinary copper levels were typically low. Treatment comprised copper supplementation and modification of any risk factors, and led to haematological normalisation and neurological improvement or stabilisation. Since any neurological recovery was partial and case numbers of CDM will continue to rise with the growing use of bariatric gastrointestinal surgery, clinical vigilance will remain the key to minimising neurological sequelae. Recommendations for treatment and prevention are made.
登录
查看更多内容
影响因子：
1.1
作者：
Bennetts, H. W.;Chapman, F. E.
通讯作者：
Chapman, F. E.
影响因子：
--
作者：
Kumar, N;McEvoy, KM;Ahlskog, JE
通讯作者：
Ahlskog, JE
影响因子：
12.6
作者：
Kumar, Neeraj;Ahlskog, J. Eric;Gross, John B., Jr.
通讯作者：
Gross, John B., Jr.
影响因子：
6
作者：
Kumar, N;Low, PA
通讯作者：
Low, PA
影响因子：
9.9
作者：
Kumar, N;Gross, JB;Ahlskog, JE
通讯作者：
Ahlskog, JE