Copper deficiency: an unusual case of myelopathy with neuropathy

Bertfield, Deborah; Jumma, Odai; Pitceathly, Robert D S; Sussman, Jon

doi:10.1258/acb.2008.007218

Cited by 8 publications

(8 citation statements)

References 5 publications

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“…A total of 55 cases were found in the literature [1, 2, 4, 12, 13, 16, 18, 19, 21, 24, 28–30, 38, 39, 41, 42, 44–47, 58, 63, 67, 71, 74, 77, 88, 89]. Age at presentation ranged from 30 to 82 years with a peak in the fifth and sixth decades (mean 54 years, median 53 years; Fig.…”

Section: Resultsmentioning

confidence: 99%

“…The remaining patients received intravenous (4%, 2 cases) [71, 88], oral then intravenous (2%, 1 case) [38, 39, 44] or intramuscular supplements (2%, 1 case) [4]; in 10 cases (18%) the route of administration was not specified [13, 16, 29, 38, 39, 41, 44, 58]. Doses were usually equivalent to 2 mg/day of elemental copper, though administration of up to 9 mg/day was reported (ignoring extreme outliers which are likely to reflect reporting errors [89]).…”

Section: Resultsmentioning

confidence: 99%

“…Doses were usually equivalent to 2 mg/day of elemental copper, though administration of up to 9 mg/day was reported (ignoring extreme outliers which are likely to reflect reporting errors [89]). A variety of copper salts were used, including copper acetate [63], copper chloride [88], copper citrate [28], copper gluconate [2, 12, 38, 44, 45, 74, 89], copper histidine [4] and copper sulphate [1, 12, 38, 41, 44, 47, 67, 71, 77]. No adverse effects were reported.…”

Section: Resultsmentioning

confidence: 99%

“…Whilst neurological complications of gastrectomy for peptic ulcer disease have long been recognised, the underlying aetiology often remained obscure [38, 39]. The association between a remote history of upper gastrointestinal surgery, copper deficiency and myelopathy was first described in 2001 [71], and has since been increasingly reported following both non-bariatric [2, 4, 12, 13, 28, 38, 39, 41, 44, 47, 63, 74, 77, 88] and bariatric intervention [16, 29, 38, 39, 44, 47]. …”

Section: Discussionmentioning

confidence: 99%

“…An additional 30 cases of myelopathy or myeloneuropathy have now been reported [1, 2, 4, 12, 13, 16, 18, 19, 21, 24, 28–30, 58, 63, 67, 71, 74, 77, 88, 89]. Here we review the published cases with regards to their demographics, aetiology, laboratory indices, spinal imaging, treatment and outcome.…”

Section: Introductionmentioning

confidence: 99%

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Copper deficiency myelopathy

2010

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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.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Copper deficiency myelopathy

2010

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Nutritional and Alcoholic Neuropathies

Shields

2013

Neuromuscular Disorders in Clinical Practice

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Short-Term Cuprizone Feeding Induces Selective Amino Acid Deprivation with Concomitant Activation of an Integrated Stress Response in Oligodendrocytes

et al. 2013

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Cuprizone [bis(cyclohexylidenehydrazide)]-induced toxic demyelination is an experimental approach frequently used to study de- and re-myelination in the central nervous system. In this model, mice are fed with the copper chelator cuprizone which leads to oligodendrocyte apoptosis and subsequent microgliosis, astrocytosis, and demyelination. The underlying mechanisms of cuprizone-induced oligodendrocyte death are still unknown. We analysed differences in amino acid levels after short-term cuprizone exposure (i.e., 4 days). Furthermore, an amino acid response (AAR) pathway activated in oligodendrocytes after cuprizone intoxication was evaluated. Short-term cuprizone exposure resulted in a selective decrease of alanine, glycine, and proline plasma levels, which was paralleled by an increase of apoptotic cells in the liver and a decrease of alanine aminotransferase in the serum. These parameters were paralleled by oligodendrocyte apoptosis and the induction of an AAR with increased expression of the transcription factors ATF-3 and ATF-4 (activating transcription factor-3 and -4). Immunohistochemistry revealed that ATF-3 is exclusively expressed by oligodendrocytes and localized to the nuclear compartment. Our results suggest that cuprizone-induced liver dysfunction results in amino acid starvation and in consequence to the activation of an AAR. We propose that this stress response modulates oligodendrocyte viability in the cuprizone animal model.

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Copper deficiency: an unusual case of myelopathy with neuropathy

Abstract: Copper deficiency is an unusual but potentially treatable cause of myelopathy. It should be considered in patients with poor nutrition or a history of gastrointestinal surgery. We report the case of an 82-year-old man who presented with sensory symptoms and gait ataxia.

Cited by 8 publications

References 5 publications

Copper deficiency myelopathy

Copper deficiency myelopathy

Nutritional and Alcoholic Neuropathies

Short-Term Cuprizone Feeding Induces Selective Amino Acid Deprivation with Concomitant Activation of an Integrated Stress Response in Oligodendrocytes

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