Motor neuron disease associated with copper deficiency

Conrad C. Weihl, Glenn Lopate

Research output: Contribution to journalArticlepeer-review

68 Scopus citations


Copper deficiency in humans is a rare cause of myeloneuropathy that usually presents with a spastic ataxic gait, hyperreflexia, and distal sensory loss similar to that seen in patients with subacute combined degeneration. We describe three copper-deficient patients, two of whom were referred with a presumptive diagnosis of amyotrophic lateral sclerosis, who had progressive asymmetric weakness or electrodiagnostic findings of proximal and distal denervation suggestive of lower motor neuron disease. Copper replacement resulted in stabilization or mild improvement in weakness. The clinical spectrum of human copper deficiency should include lower motor neuron disease in addition to a syndrome of spastic ataxia.

Original languageEnglish
Pages (from-to)789-793
Number of pages5
JournalMuscle and Nerve
Issue number6
StatePublished - Dec 2006


  • Amyotrophic lateral sclerosis
  • Copper deficiency
  • Motor neuron disease
  • Myelodysplastic syndrome
  • Myelopathy
  • Spinal cord disease


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