Copper deficiency myelopathy

Arch Neurol. 2004 May;61(5):762-6. doi: 10.1001/archneur.61.5.762.


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.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Ceruloplasmin / deficiency*
  • Copper / blood
  • Copper / deficiency*
  • Evoked Potentials, Somatosensory
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Metabolic Diseases / blood
  • Metabolic Diseases / etiology
  • Metabolic Diseases / physiopathology
  • Middle Aged
  • Peripheral Nervous System Diseases / blood
  • Peripheral Nervous System Diseases / etiology
  • Peripheral Nervous System Diseases / physiopathology
  • Spinal Cord / pathology
  • Spinal Cord / physiopathology
  • Spinal Cord Diseases / blood
  • Spinal Cord Diseases / etiology*
  • Spinal Cord Diseases / physiopathology


  • Copper
  • Ceruloplasmin