Severe ataxia, myelopathy, and peripheral neuropathy due to acquired copper deficiency in a patient with history of gastrectomy.
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 B(12) deficiency.


METHODS
This is a single case report. Our patient developed a neurologic constellation of ataxia, myelopathy, and peripheral neuropathy similar to vitamin B(12) 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 microg/dL to 20 microg/dL (70-150 microg/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 B(12) deficiency. Vitamin B(12) 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.
Tan
Jeremias C
JC
Gastroenterology, Lahey Clinic Medical Center, Burlington, Massachusetts 01805, USA.
Burns
David L
DL
Jones
H Royden
HR
D000368:Aged; D001259:Ataxia / Q000209:etiology; D002570:Ceruloplasmin / Q000378:metabolism; D003300:Copper / Q000097:blood / Q000172:deficiency* / Q000627:therapeutic use*; D005743:Gastrectomy / Q000009:adverse effects*; D006801:Humans; D008297:Male; D010523:Peripheral Nervous System Diseases / Q000209:etiology; D011178:Postgastrectomy Syndromes / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D011183:Postoperative Complications / Q000097:blood / Q000503:physiopathology; D013118:Spinal Cord Diseases / Q000209:etiology; D014131:Trace Elements / Q000097:blood / Q000172:deficiency* / Q000627:therapeutic use; D016896:Treatment Outcome
D014131:Trace Elements; D003300:Copper; D002570:Ceruloplasmin