Copper deficiency: an unusual case of myelopathy with neuropathy

This version was published on 1 November 2008

Ann Clin Biochem 2008;45:616-617
doi:10.1258/acb.2008.008122
© 2008 Association for Clinical Biochemistry

 

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We read with interest the report of a case of myelopathy and neuropathy due to copper deficiency.1 Although this is the 55th reported case since the original description of myelopathy due to acquired copper deficiency in humans,2 it is likely that the condition remains under-recognized. In the North of England alone, five cases of copper deficiency myelopathy (CDM) have been diagnosed since the local index case raised awareness in late 2006.3

The most common cause of CDM is previous upper gastrointestinal surgery, which was implicated in 47% of reported cases (review in preparation by authors). In the past, such surgery was typically performed for peptic ulcer disease, but with the advent of proton pump inhibitors and increasing levels of obesity, bariatric surgery is likely to take over as the leading risk factor.4 The second most common cause of CDM is hyperzincaemia. In addition to the causes listed by the authors, denture cream can be a source of spurious zinc excess which has recently been associated with four cases of CDM and is easily missed by the unaware.5Although primary dietary copper deficiency could potentiallygive rise to CDM, such an association has not been reportedto date.

Bertfield et al. (Ann Clin Biochem 2008;45:434–435) address the pathogenesis of the haematological manifestations, but not that of the neurological syndrome. It has been suggested that copper deficiency causes myelopathy through dysfunction of cytochrome oxidase, which is known to be copper-dependent.6 However, this enzyme is not cobalamin-dependent, so this hypothesis does not readily explain the marked phenotypic parallels between CDM and subacute combined degeneration of the cord due to cobalamin deficiency (SCD). We have recently proposed that CDM and SCD share dysfunction of the methylation cycle as their final common pathway, which would explain their clinical and radiological similarity.7

This is the first case in which copper supplementation was givenintramuscularly. A parenteral route was chosen because the patient‘would be unable to absorb oral supplements’. Althoughthe absorptive surface for copper is reduced, oral supplementationhas been effective in many similar cases so such an approachmay not be necessary.

Finally, Bertfield et al. assert that ‘treatment withcopper may prevent further neurological deterioration’.This is an understatement, since the neurological deficit ofall reported cases did stabilize or improve on adequate copperreplacement. Timely recognition and treatment are crucial inpreventing potentially irreversible neurological disability.


Gavin P Winston1 and
Stephan R Jaiser2


1 National Hospital for Neurology and Neurosurgery, Queen Square, London;
2 Department of Neurology, Newcastle General Hospital, Newcastle upon Tyne, UK




REFERENCES

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REFERENCES


  1. Bertfield DL, Jumma O, Pitceathly RD, Sussman JD. Copper deficiency: an unusual case of myelopathy with neuropathy. Ann Clin Biochem 2008;45:434–5[Abstract/Free Full Text]
  2. Schleper B, Stuerenburg HJ. Copper deficiency-associated myelopathy in a 46-year-old woman. J Neurol 2001;248:705–6[Medline]
  3. Jaiser SR, Duddy M. Copper deficiency masquerading as subacute combined degeneration of the cord and myelodysplastic syndrome. Adv Clin Neurosci Rehabil 2007;7:20–1
  4. Juhasz-Pocsine K, Rudnicki SA, Archer RL, Harik SI. Neurologic complications of gastric bypass surgery for morbid obesity. Neurology 2007;68:1843–50[Abstract/Free Full Text]
  5. Nations SP, Boyer PJ, Love LA et al. Denture cream. An unusual source of excess zinc, leading to hypocupremia and neurologic disease. Neurology 2008 (Epub ahead of print, doi:10.1212/01.wnl.0000312375.79881.94)
  6. Kumar N. Copper deficiency myelopathy (human swayback). Mayo Clin Proc 2006;81:1371–84[Abstract/Free Full Text]
  7. Winston GP, Jaiser SR. Copper deficiency myelopathy and subacute combined degeneration of the cord – Why is the phenotype so similar? Med Hypotheses 2008;71:229–36[Medline]

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