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Copper deficiency myeloneuropathy

INTRODUCTION

Copper deficiency-associated myelopathy has been well described in various animal species, in particular ruminants, in which it is called swayback or enzootic ataxia. Acquired copper deficiency has only recently been recognized to cause a myelopathy in humans [1,2]. The neurologic manifestations of copper deficiency are usually, but not always, accompanied by the typical hematologic derangements of anemia and leukopenia.

This topic discusses the neurologic manifestations of acquired copper deficiency. The physiologic and biochemical functions of dietary copper and the dietary requirements of copper are discussed in detail separately. (See "Overview of dietary trace minerals", section on 'Copper'.)

A brief overview of Menkes disease, a congenital x-linked disorder of severe copper deficiency, is also discussed separately. (See "Overview of dietary trace minerals", section on 'Menkes disease'.)

EPIDEMIOLOGY

The age range of reported cases of copper deficiency myeloneuropathy is 30 to 82 years [3,4]. More cases in women than men are reported.

PATHOPHYSIOLOGY

The mechanism underlying neurologic damage in individuals with copper deficiency is uncertain. Copper is a component of enzymes that have a critical role in the structure and function of the nervous system [5-10]. It permits electron transfer in key enzymatic pathways. These include cytochrome-c-oxidase for electron transport and oxidative phosphorylation, copper/zinc superoxide dismutase for antioxidant defense, tyrosinase for melanin synthesis, dopamine beta-hydroxylase for catecholamine biosynthesis, lysyl oxidase for crosslinking of collagen and elastin, peptidylglycine alpha-amidating monooxygenase for neuropeptide and peptide hormone processing, monoamine oxidase for serotonin synthesis, and ceruloplasmin for brain iron homeostasis.

              

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Literature review current through: Apr 2013. | This topic last updated: Dec 19, 2011.
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