Document Detail

Motor neuron disease associated with copper deficiency.
MedLine Citation:
PMID:  16929546     Owner:  NLM     Status:  MEDLINE    
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.
Conrad C Weihl; Glenn Lopate
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Muscle & nerve     Volume:  34     ISSN:  0148-639X     ISO Abbreviation:  Muscle Nerve     Publication Date:  2006 Dec 
Date Detail:
Created Date:  2006-11-20     Completed Date:  2007-03-02     Revised Date:  2007-12-03    
Medline Journal Info:
Nlm Unique ID:  7803146     Medline TA:  Muscle Nerve     Country:  United States    
Other Details:
Languages:  eng     Pagination:  789-93     Citation Subset:  IM    
Department of Neurology, Washington University School of Medicine, Campus Box 8111, 660 South Euclid Avenue, St. Louis, Missouri 63110, USA.
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MeSH Terms
Amyotrophic Lateral Sclerosis / diagnosis*,  drug therapy,  etiology*
Copper / blood,  deficiency*
Gluconates / therapeutic use
Middle Aged
Motor Neuron Disease / diagnosis*,  drug therapy,  etiology*
Treatment Outcome
Grant Support
Reg. No./Substance:
0/Gluconates; 7440-50-8/Copper

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