title: Motor neuron disease associated with copper deficiency.
abstract: 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.
authors.affiliation: Department of Neurology, Washington University School of Medicine, Campus Box 8111, 660 South Euclid Avenue, St. Louis, Missouri 63110, USA.
authors.forename: Conrad C
authors.initials: CC
authors.lastname: Weihl
authors.forename: Glenn
authors.initials: G
authors.lastname: Lopate
mesh_terms: D000328:Adult; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D003300:Copper / Q000097:blood / Q000172:deficiency*; D004568:Electrodiagnosis; D005260:Female; D005942:Gluconates / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D016896:Treatment Outcome
chemical_list: D005942:Gluconates; D003300:Copper