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- Neeraj Kumar, Brian Crum, Ronald C Petersen, Steven A Vernino, and J Eric Ahlskog.
- Department of Neurology, Mayo Clinic, Mayo Foundation, Rochester, Minn, USA. kumar.neeraj@mayo.edu
- Arch Neurol Chicago. 2004 May 1; 61 (5): 762-6.
BackgroundIn humans, Menkes disease is the well-recognized neurological disorder due to inherited copper deficiency. Myelopathy due to acquired copper deficiency is not a well-recognized entity in humans, although myelopathy due to copper deficiency is well documented in some animal species.PatientsWe describe 3 patients who developed a progressive spastic-ataxic gait with proprioceptive deficits. All patients had a severe reduction in serum ceruloplasmin and copper levels.ResultsAll patients had evidence of posterior column dysfunction clinically and on somatosensory evoked potential studies. Two had a signal change in the posterior column on magnetic resonance imaging of the spinal cord.ConclusionPatients presenting with otherwise unexplained myelopathies should have their serum ceruloplasmin level measured.
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