Unexplained neuropathy is a rare condition, but doctors should suspect it and measure serum copper levels in patients with myelopathy, peripheral neuropathy, or optic neuropathy, said Juan J. Vilchez, MD, chief, department of neurology, Hospital Universitario La Fé, Valencia, Spain. Patients can improve with prompt treatment, he said.

In their poster, Dr. Vilchez and colleagues described a 52-year-old man who first came to their hospital in 2003 with progressive gait and visual impairment that started approximately 1 year earlier.

Although he could walk unassisted, the patient's symptoms mostly affected the lower body and included spastic paraparesis, impaired proprioception and vibration sensation, exaggerated knee jerk reflexes, and bilateral extensor plantar responses. He also suffered from impotence and constipation.

Extensive tests of endocrine and immunological function were normal or negative, but evoked potential studies showed evidence of abnormal conduction. Abnormal findings also were seen on electromyography and muscle biopsy, which showed changes consistent with mitochondrial myopathy.

The patient had no personal or familial history of any neurological disorder.

The patient continued to deteriorate, until by 2005 he could walk only with bilateral support. In June of that year he was found to have low serum levels of copper and ceruloplasmin. His doctors started him on a course of 3 mg/day oral copper supplements, well above the normal U.S. Recommended Daily Allowance for adults of 900 mcg. The patient improved steadily and within 7 months was able to walk independently again.

Copper is essential to the function of several enzymes, including cytochrome C oxidase, which figures in mitochondrial respiration, lysyl oxidase, important in collagen and elastin crosslinking, and ceruloplasmin, which is necessary for iron metabolism, the authors said.

Copper deficiency has long been associated with anemia and neutropenia, but its neurological consequences have only recently been recognized. It is available in a wide variety of foods, including fruits, nuts, legumes, avocadoes, and shellfish, but deficiencies may occur in adults who have celiac disease or are on very restrictive diets.

The reasons for this patient's copper deficiency were never identified.


[Presentation title: Copper Deficiency Associated Reversible Opticomyeloneuropathy. Poster 197]

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