Acquired copper deficiency myelopathy

• Published in: Revue neurologique Vol. 166; no. 6-7; pp. 639 - 643
• Main Authors: Videt-Gibou, D, Belliard, S, Rivalan, J, Ménard, D, Edan, G
• Format: Journal Article
• Language: French
• Published: France 01-06-2010
• Subjects: Adult; Copper - deficiency; Copper - therapeutic use; Enteral Nutrition - adverse effects; Female; Gait Ataxia - etiology; Humans; Iron Overload - complications; Magnetic Resonance Imaging; Malabsorption Syndromes - complications; Male; Middle Aged; Muscle Spasticity - etiology; Nephrotic Syndrome - etiology; Nephrotic Syndrome - therapy; Spinal Cord - pathology; Zinc - adverse effects
• ISSN: 0035-3787
• DOI: 10.1016/j.neurol.2009.10.017

The hematological manifestations of acquired copper deficiency are well known. But the neurological manifestations have only been recognised in the past few years. The most common neurological manifestation in adults is a myeloneuropathy with prominent sensory ataxia and spastic gait. Electrophysiological tests reveal an axonal sensorimotor peripheral neuropathy. Spinal MRI shows an augmented T2 signal involving the dorsal column. The causes of acquired copper deficiency include gastric surgery, excessive zinc ingestion, and malabsorption but in most cases, the cause remains unclear. Early recognition and treatment may prevent neurological deterioration but improvement seems to be slight and inconstant.
We report two new cases of acquired copper deficiency myeloneuropathy associated with a nephrotic syndrome and, in one case, with a major iron overload syndrome. Biological abnormalities disappeared under copper supplementation. A significant neurological improvement with disappearance of ataxia occurred in one patient who received copper supplementation eight months after symptom onset.
Nephrotic syndrome might be another complication of acquired copper deficiency. Delayed treatment is not necessarily associated with a deleterious neurological prognosis. Significant neurological improvement under copper supplementation is possible.