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Neeraj Kumar, MD; Brian Crum, MD; Ronald C. Petersen, MD, PhD; Steven A. Vernino, MD, PhD; J. Eric Ahlskog, PhD, MD

Arch Neurol. 2004;61:762-766.

Background  In 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.

Patients  We 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.

Results  All 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.

Conclusion  Patients presenting with otherwise unexplained myelopathies should have their serum ceruloplasmin level measured.

From the Department of Neurology, Mayo Clinic, Mayo Foundation, Rochester, Minn.

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