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The neurodegenerative syndrome of copper deficiency has been recognized for some time in ruminant animals, in which it is commonly known as "swayback" The disease involves a nutritional deficiency in the trace element copper. Copper is ubiquitous and daily requirement is low making acquired copper deficiency very rare. Copper deficiency can manifest in parallel with vitamin B12 deficiency and zinc toxicity. The most common cause of copper deficiency is a remote gastrointestinal surgery, such as gastric bypass surgery, due to malabsorption of copper. Menkes disease is a variation of copper deficiency involving a wide variety of symptoms . Menkes disease is a congenital genetic disorder that is often fatal. Copper is involved in normalized function of many enzymes, such as cytochrome c oxidase, which is complex IV in mitochondrial electron transport chain, ceruloplasmin, Cu/Zn superoxide dismutase, and in amine oxidases. These enzyme catalyze reactions for oxidative phosphorylation, iron transportation, antioxidant and free radical scavenging and neutralization, and neurotransmitter synthesis, respectively . A regular diet contains a variable amount of copper, but may provide 5 mg/day , of which only 20-50% are absorbed. . The diet of the elderly have shown to have lower copper amounts than the recommended daily intake . Dietary copper can be found in whole grain cereals, legumes, oysters, organ meats (livers), cherries, dark chocolate, fruits, leafy green vegetables, nuts, poultry, prunes, and soybeans products like tofu . The deficiency in copper can cause many hematological manifestations, such as myelodysplasia, anemia, leukopenia (low white blood cell count) and neutropenia (low count of neutrophils, a type of white blood cell that is often called "the first line of defense" for the immune system). Copper deficiency has long been known for as a cause of myelodysplasia(when a blood profile has indicators of possible future leukemia development), but it was until recently in 2001 that copper deficiency was associated with neurological manifestations. Some neurological manifestations can be sensory ataxia (irregular muscle coordination), peripheral neuropathy (damage in the peripheral nerves) and myelopathy (disease of the spinal cord) . |
