What is the recommended treatment and dosing for copper deficiency myelopathy?

Treatment and Dosing for Copper Deficiency Myelopathy

For copper deficiency myelopathy, initiate treatment with 4-8 mg of copper daily, which is substantially higher than standard supplementation doses, and consider intravenous administration in severe cases or when oral therapy fails to restore levels. 1

Severity-Based Treatment Approach

Severe Copper Deficiency (Plasma Copper <8 μmol/L)

• Repletion measures must be taken immediately when plasma copper values are <8 μmol/L, regardless of CRP elevation 1
• Treatment requires 4-8 mg copper daily, which is the therapeutic dose for deficiency states 1
• In cases of myelopathy with severe neurological symptoms, consider intravenous copper repletion for rapid correction, as oral supplementation alone may result in only partial neurological recovery 2
• The route of administration should be determined by severity of deficit 1

Moderate Copper Deficiency (Plasma Copper <12 μmol/L with CRP >20 mg/L)

• Copper administration should be considered when plasma concentrations are <12 μmol/L with elevated CRP >20 mg/L 1
• In chronic conditions, oral administration may be considered first 1
• For mild deficiency, consider two Forceval daily (multivitamin containing copper) for 3 months and recheck levels 1

Critical Monitoring and Zinc Considerations

The Zinc-Copper Interaction

• Always check both zinc and copper levels simultaneously when considering copper replacement, as high zinc is a common cause of copper deficiency 1
• Maintain a ratio of 8-15 mg zinc to 1 mg copper when supplementing both minerals 1
• High-dose zinc supplementation can induce copper deficiency by blocking intestinal copper absorption 3
• If severe zinc deficiency is present with normal or borderline copper levels, treat zinc first but monitor copper closely, as copper levels may fall further 1

Laboratory Monitoring

• Copper status must be determined by measuring plasma copper simultaneously with CRP, as inflammation falsely elevates copper levels 1
• In patients with myelopathy of unclear etiology, copper levels should be measured as part of the diagnostic workup 1
• Follow-up copper levels should be checked after 3 months of treatment 1

Special Populations and Risk Factors

Post-Bariatric Surgery Patients

• Copper levels should be measured in all patients following bariatric surgery or other abdominal surgeries that exclude the duodenum 1
• With severe copper deficiency in this population, refer for specialist advice 1
• Standard post-bariatric supplementation provides only 1-3 mg copper daily, which is insufficient for treating established deficiency 1, 4

Patients with Established Myelopathy

• The most common risk factors include prior upper gastrointestinal surgery (36.2% of cases), excessive zinc consumption (19.9%), and malabsorption syndromes 5, 3
• Copper deficiency myelopathy presents with sensory ataxia, dorsal column dysfunction, and lower limb spasticity, closely mimicking B12 deficiency 6, 7
• MRI may show hyperintense T2 signal in posterior cervical and thoracic cord, sometimes with characteristic inverted "V" appearance 5, 3

Treatment Outcomes and Prognosis

Expected Response

• Copper supplementation typically leads to hematological normalization (correction of anemia, neutropenia) within weeks 3, 2
• Neurological improvement is variable and often incomplete, with only 24% of cases showing improvement and only 5.1% recovering to baseline 5
• Treatment prevents further neurological deterioration but may not reverse established damage 3, 7

Critical Timing

• Early diagnosis is mandatory to prevent irreversible neurological damage, as copper deficiency myelopathy is progressive 6
• Delays in diagnosis result in worse outcomes, as neurological recovery is typically partial even with appropriate treatment 3, 7

Common Pitfalls to Avoid

• Do not use standard supplementation doses (1-3 mg daily) to treat established deficiency—these are prophylactic doses only 1, 4
• Do not supplement copper without checking zinc levels first, as the interaction between these minerals is bidirectional 1
• Do not assume normal copper levels if CRP is elevated, as ceruloplasmin (which carries copper) is an acute phase reactant 1
• Do not delay treatment waiting for specialist referral in severe cases—initiate copper replacement immediately while arranging follow-up 1
• Do not stop monitoring after initial improvement, as ongoing supplementation and monitoring are required to prevent recurrence 3