Copper deficiency myeloneuropathy

Neeraj Kumar MD (Dr. Kumar of the Mayo Clinic College of Medicine has no relevant financial relationships to disclose.)
Louis H Weimer MD, editor. (Dr. Weimer of Columbia University has received consulting fees from Roche.)
Originally released September 15, 2009; last updated January 27, 2017; expires January 27, 2020

Overview

The commonest neurologic manifestation of acquired copper deficiency is that of a myelopathy. A peripheral neuropathy of variable severity is commonly associated with the myelopathy. Anemia and neutropenia are well-recognized hematologic manifestations of acquired copper deficiency. Copper deficiency myeloneuropathy may be present without hematological manifestations. The commonly identified causes of acquired copper deficiency include a prior history of gastric surgery, excessive zinc ingestion, and malabsorption. Copper and vitamin B12 deficiency may coexist. Estimation of serum copper levels should be a part of the work-up in patients with a myelopathy or myeloneuropathy, particularly in patients with a high risk of developing copper deficiency. In this article, the author discusses the topic of acquired copper deficiency, with a special emphasis on its neurologic manifestations.

Key points

 

• The commonest neurologic manifestation of acquired copper deficiency is that of a myelopathy. A peripheral neuropathy of variable severity is commonly associated with the myelopathy.

 

• Anemia and neutropenia are well-recognized hematologic manifestations of acquired copper deficiency. Copper deficiency myeloneuropathy may be present without hematological manifestations.

 

• The commonly identified causes of acquired copper deficiency include a prior history of gastric surgery, excessive zinc ingestion, and malabsorption.

 

• Copper and vitamin B12 deficiency may coexist.

 

• Estimation of serum copper levels should be a part of the work-up in patients with a myelopathy or myeloneuropathy, particularly in patients with a high risk of developing copper deficiency.

Historical note and terminology

Copper deficiency-associated myelopathy has been well described in various animal species. Often seen in ruminants, it has been called swayback or enzootic ataxia. Only in recent years have the neurologic manifestations of acquired copper deficiency in humans been recognized (Prodan and Holland 2000; Schleper and Stuerenburg 2001; Gregg et al 2002; Prodan et al 2002; Prodan et al 2006; Hedera et al 2003; Kumar et al 2003a; Kumar et al 2003b; Kumar et al 2004a; Kumar et al 2004b; 2004c; 2005; 2006; Greenberg and Briemberg 2004; Kumar and Low 2004; Rowin and Lewis 2005; Willis et al 2005; Everett et al 2006; Kumar 2006; Shook et al 2006; Sorenson 2006; Weihl and Lopate 2006; Yaldizli et al 2006; Juhasz-Pocsine et al 2007; Spinazzi et al 2007; Halfdanarson et al 2008; Kelkar et al 2008; Nations et al 2008; Goodman et al 2009a; Goodman et al 2009b; Hedera et al 2009; Naismith et al 2009; Spain et al 2009; Zara et al 2009; Afrin 2010; Jaiser and Winston 2010; Videt-Gibou et al 2010; Inaba et al 2011; Kumar et al 2011; Gabreyes et al 2013). The neurologic syndrome due to acquired copper deficiency may be present without the hematological manifestations (Kumar et al 2003a; Kumar et al 2003b; Kumar et al 2004a; Kumar et al 2004b; Kumar et al 2004c; Kumar 2006). Copper deficiency since birth is seen in Menkes disease. Comparative neuropathological studies have shown similarities between Menkes disease and swayback.

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