Copper deficiency myeloneuropathy

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Copper deficiency myeloneuropathy is rare but increasingly recognized as a cause of neurological impairment, presenting similarly to subacute combined degeneration of the cord secondary to vitamin B12 deficiency.

Clinical presentation

Patients typically present with a proprioceptive loss, due to dorsal column involvement, which manifests as a sensory ataxia .

Copper deficiency is not common and may be present in a few clinical circumstances:

  • malabsorption syndromes
  • gastric bypass surgery
  • zinc toxicity: there is a competitive gastrointestinal absorption between copper and zinc

Pathology

The serum copper and ceruloplasmin levels are low.

Radiographic features

MRI is the modality of choice for assessing suspected cases.

MRI

Spinal cord imaging demonstrates symmetric high T2 signal in the dorsal columns .

In some patients, findings suggestive of demyelination have been reported .

Treatment and prognosis

Copper supplementation can arrest further neurological deterioration and even reverse symptoms and imaging findings .

Differential diagnosis