Severe Ataxia, Myelopathy, and Peripheral Neuropathy Due to Acquired Copper Deficiency in a Patient With History of Gastrectomy

• Published in: JPEN. Journal of parenteral and enteral nutrition Vol. 30; no. 5; pp. 446 - 450
• Main Authors: Tan, Jeremias C., Burns, David L., Jones, H. Royden
• Format: Journal Article
• Language: English
• Published: Silver Spring, MD SAGE Publications 01.09.2006; ASPEN; American Society for Parenteral and Enteral Nutrition
• Subjects: Aged; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Ataxia - etiology; Biological and medical sciences; Bone marrow, stem cells transplantation. Graft versus host reaction; Ceruloplasmin - metabolism; Copper - blood; Copper - deficiency; Copper - therapeutic use; Emergency and intensive care: metabolism and nutrition disorders. Enteral and parenteral nutrition; Gastrectomy - adverse effects; Hematologic and hematopoietic diseases; Humans; Intensive care medicine; Male; Medical sciences; Peripheral Nervous System Diseases - etiology; Platelet diseases and coagulopathies; Postgastrectomy Syndromes - blood; Postgastrectomy Syndromes - diagnosis; Postgastrectomy Syndromes - drug therapy; Postoperative Complications - blood; Postoperative Complications - physiopathology; Spinal Cord Diseases - etiology; Trace Elements - blood; Trace Elements - deficiency; Trace Elements - therapeutic use; Transfusions. Complications. Transfusion reactions. Cell and gene therapy; Treatment Outcome; Human; Nervous system diseases; Nutrition; Deficiency; Peripheral neuropathy; Enteral administration; Parenteral administration; Cerebral disorder; Feeding; Central nervous system disease; Ataxia; Peripheral nerve disease; Neurological disorder
• ISSN: 0148-6071; 1941-2444
• DOI: 10.1177/0148607106030005446

Background: In animal studies, copper absorption has been demonstrated to occur in the proximal gut via duodenal enterocytes. Acquired copper deficiency is known as “swayback” in ruminant animals and Menkes' disease in humans. Copper is an essential micronutrient necessary for the hematologic and neurologic systems. Acquired copper deficiency in humans has been described, causing a syndrome similar to the subacute combined degeneration of vitamin B12 deficiency. Methods: This is a single case report. Our patient developed a neurologic constellation of ataxia, myelopathy, and peripheral neuropathy similar to vitamin B12 deficiency many years after gastrectomy for severe peptic ulcer disease. The patient was maintained for decades with enteral feedings via jejunostomy that provided the recommended dietary allowance (RDA) for copper. Results: Copper deficiency was suspected, identified, and treated. Over 3 months of follow-up, serum copper levels increased from 4 μg/dL to 20 μg/dL (70–150 μg/dL), and ceruloplasmin increased from 6 mg/dL to 8 mg/dL (14–58 mg/dL). During this short time of follow-up, the patient has had no further progression of his neurologic symptoms. Conclusions: Ataxia and myelopathy secondary to acquired copper deficiency are rare complications of major gastric resection. This is quite similar to the syndrome of vitamin B12 deficiency. Vitamin B12 repletion does not improve symptoms. Bariatric procedures such as gastric bypass surgery result in a similar functional anatomy of the proximal gut and may place more patients at increased risk. Early recognition and therapy with oral or parenteral copper may lead to a decrease in both neurologic and hematologic consequences. Severe neurologic symptoms secondary to acquired copper deficiency are being recognized in patients undergoing gastric surgery. With increased use of restrictive and malabsorptive operations for obesity, more patients may be at risk for micronutrient deficiency and late neurologic complications.