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  • Updated 02.26.2021
  • Released 09.15.2009
  • Expires For CME 02.26.2024

Copper deficiency myeloneuropathy

Introduction

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.

• A low copper level can also be seen in Wilson disease, a disorder of copper toxicity, or in carriers of the Wilson disease gene. In Wilson disease, copper deposited in tissues and copper excreted in urine is increased.

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 (64; 69; 19; 65; 63; 25; 45; 49; 39; 42; 46; 43; 36; 18; 48; 67; 81; 11; 36; 70; 71; 80; 84; 30; 73; 23; 32; 57; 15; 16; 26; 56; 72; 86; 01; 29; 79; 28; 35; 13). The neurologic syndrome due to acquired copper deficiency may be present without the hematological manifestations (45; 49; 39; 42; 46; 36). Copper deficiency since birth is seen in Menkes disease. Comparative neuropathological studies have shown similarities between Menkes disease and swayback.

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