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Myelopolyneuropathy and Pancytopenia Due to Copper Deficiency and High Zinc Levels of Unknown Origin
Further Support for Existence of a New Zinc Overload Syndrome
Peter Hedera, MD;
John K. Fink, MD;
Paula L. Bockenstedt, MD;
George J. Brewer, MD
Arch Neurol. 2003;60:1303-1306.
Objective To describe a patient with idiopathic zinc overload without an identifiable source and secondary copper deficiency causing myelopolyneuropathy and pancytopenia.
Design Case report.
Patient and Results A 46-year-old man presented with severe bone marrow suppression and subsequently developed progressive myelopathy with sensory ataxia. No identifiable cause of myelopathy was detected, and his neuroimaging findings were unremarkable. Plasma analysis demonstrated a low copper level and an increased zinc level (<10 µg/dL [<12.6-18.9 µmol/L] and 184 µg/dL [28.2 µmol/L], respectively; normal range for both, 80-120 µg/dL [12.6-18.9 µmol/L and 12.3-18.4 µmol/L, respectively) and a low level of ceruloplasmin. There was no evidence for an external source of zinc. Daily oral supplementation with 2 mg resulted in the prompt reversal of hematologic abnormalities, improved but still subnormal plasma copper levels, and normalization of ceruloplasmin values. The patient's neurologic condition deteriorated further, with worsening of myelopathy and development of polyneuropathy. Analyses of plasma copper and zinc levels demonstrated persisting hyperzincemia and subnormal copper levels during 4 years of follow-up. Increased copper supplementation to 8 mg/d partially reversed his neurologic signs. A clinical investigation of 6 siblings and 1 surviving parent did not identify family members with similar abnormalities.
Conclusions Persistent hyperzincemia without an identifiable external source appears to be a primary metabolic defect, while copper deficiency is a secondary phenomenon, causing hematologic and neurologic abnormalities. Two unrelated patients with similar idiopathic hyperzincemia and hypocupremia have been recently described. This suggests the existence of a new metabolic disorder with idiopathic zinc overload.
From the Departments of Neurology (Drs Hedera and Fink), Internal Medicine (Drs Bockenstedt and Brewer), and Human Genetics (Dr Brewer), University of Michigan, and the Geriatric Research Education and Clinical Center, Ann Arbor Veterans Affairs Medical Center (Dr Fink), Ann Arbor, Mich. Dr Hedera is now affiliated with the Department of Neurology, Vanderbilt University, Nashville, Tenn.
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