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Review
. 2010 Jun;257(6):869-81.
doi: 10.1007/s00415-010-5511-x. Epub 2010 Mar 16.

VSports手机版 - Copper deficiency myelopathy

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Review

Copper deficiency myelopathy (VSports注册入口)

Stephan R Jaiser (VSports在线直播) et al. J Neurol. 2010 Jun.

Abstract

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 VSports手机版. .

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"VSports" Figures

Fig. 1
Fig. 1
Age at presentation. Female and male cases are plotted in black and white, respectively
Fig. 2
Fig. 2
Serum copper and caeruloplasmin. a In two cases, serum copper was reported to be low with no numerical value given (not shown). Typical reference ranges have a lower limit of normal of 0.75 μg/mL (range 0.65–0.85). b No readings were reported in 15 cases (not shown). The mean lower limit of normal was 21.5 mg/dL (range 14–26, excluding an outlier of 7.9)
Fig. 3
Fig. 3
Serum zinc against serum copper in the 45 cases where both were reported. Different symbols represent different aetiologies
Fig. 4
Fig. 4
Sagittal and axial T2-weighted 3T MR images of the cervical cord. A typical high signal lesion is demonstrated in the dorsal columns (arrowheads)

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