Copper Deficiency Myelopathy: Symptoms, Risks, and Care

Copper is an essential mineral crucial for nerve function, red blood cell production, and immune health. A deficiency can lead to serious neurological complications, including copper deficiency myelopathy, which affects the spinal cord and causes motor and sensory impairments. Though rare, it is often misdiagnosed, delaying treatment. Early detection and proper management are key to preventing irreversible damage.

Contributing Factors

Copper deficiency myelopathy results from disrupted copper absorption or excessive loss. Malabsorption syndromes, particularly those linked to gastrointestinal surgeries, are a major cause. Procedures like gastric bypass or extensive small bowel resection impair copper uptake by altering the duodenum and proximal jejunum, where copper is primarily absorbed. A study in The American Journal of Clinical Nutrition found that Roux-en-Y gastric bypass patients often experience a decline in serum copper levels, sometimes developing neurological symptoms within months or years post-surgery.

Chronic gastrointestinal disorders like celiac disease and Crohn’s disease also contribute to copper deficiency. Persistent inflammation and mucosal damage reduce the intestine’s ability to absorb trace minerals. Research in Gastroenterology highlights that untreated celiac disease frequently leads to multiple micronutrient deficiencies, including copper depletion, which can cause progressive spinal cord degeneration.

Dietary insufficiency, though less common in developed nations, remains a risk, particularly for those on restrictive diets. Excessive zinc intake—whether from supplements or high-zinc foods like oysters and red meat—can also induce copper deficiency. Zinc competes with copper for absorption by upregulating metallothionein, a protein that binds copper and prevents its circulation. A case series in Neurology documented myelopathy in patients who used zinc-containing denture creams for prolonged periods.

Genetic disorders such as Menkes disease can impair copper metabolism. This X-linked condition results in defective ATP7A transport proteins responsible for copper distribution. While Menkes disease typically presents in infancy, milder variants can lead to late-onset neurological symptoms resembling acquired copper deficiency myelopathy. Genetic screening has shown that mutations in copper transport genes can predispose individuals to neurological deterioration, even with adequate dietary intake.

Signs And Symptoms

Copper deficiency myelopathy primarily presents as a progressive neurological disorder, often resembling subacute combined degeneration from vitamin B12 deficiency. Early symptoms include sensory disturbances in the lower extremities, such as numbness, tingling, or a “pins and needles” sensation, typically starting in the feet and progressing upward. This pattern reflects the vulnerability of the dorsal columns, which control proprioception and vibration sensation. A case series in Brain found that diminished vibratory sense in the ankles and knees often precedes widespread sensory impairment.

As the condition advances, gait abnormalities become more pronounced. Many patients develop spastic ataxia, a mix of stiffness and coordination difficulties affecting mobility. The corticospinal tract, which governs voluntary motor control, is particularly susceptible to copper depletion, leading to hyperreflexia and lower limb spasticity. A study in Neurology found that over 80% of patients with copper deficiency myelopathy displayed exaggerated deep tendon reflexes and a positive Babinski sign, indicating upper motor neuron involvement. These motor deficits can progress to the point where assistive devices like walkers or wheelchairs are needed.

Some individuals also experience autonomic dysfunction, including bladder urgency, incontinence, or bowel irregularities, due to spinal cord involvement. Research in Clinical Autonomic Research suggests that longstanding copper deficiency may lead to orthostatic hypotension, which causes dizziness upon standing and increases fall risk, particularly in older adults.

In some cases, optic neuropathy accompanies myelopathy, leading to visual disturbances like blurred vision or reduced color perception. The optic nerve, like the spinal cord, requires copper for proper myelination. A review in Ophthalmology noted cases where patients with myelopathy also exhibited optic disc pallor, indicating chronic optic nerve damage. While vision loss is not universal, its presence alongside spinal cord dysfunction should prompt consideration of copper deficiency.

Diagnostic Procedures

Diagnosing copper deficiency myelopathy involves clinical evaluation, laboratory testing, and neuroimaging to differentiate it from similar conditions like vitamin B12 deficiency, multiple sclerosis, and hereditary ataxias. A neurological exam assesses sensory deficits, reflex abnormalities, and upper motor neuron signs. Patients often exhibit decreased vibratory sensation, spasticity, and an ataxic gait, prompting further investigation.

Laboratory tests confirm copper deficiency. Serum copper and ceruloplasmin levels are primary markers, with low concentrations of both indicating deficiency. Serum copper levels below 70 µg/dL and ceruloplasmin levels under 20 mg/dL typically warrant concern. Zinc levels may also be measured, as excessive zinc intake can deplete copper by inducing metallothionein expression, which binds copper and prevents absorption. If zinc toxicity is suspected, discontinuing external zinc sources is necessary.

Spinal cord MRI provides further diagnostic clarity. T2-weighted MRI sequences often show hyperintense signals in the dorsal columns of the cervical and thoracic spinal cord, resembling imaging abnormalities seen in vitamin B12 deficiency. Unlike structural spinal cord diseases, copper deficiency myelopathy typically lacks contrast enhancement or mass effect, reinforcing its metabolic nature. In ambiguous cases, additional imaging such as brain MRI or nerve conduction studies may help rule out other diagnoses.

Dietary And Supplement Measures

Restoring copper levels involves dietary adjustments and supplementation. For mild deficiencies, increasing consumption of copper-rich foods can be effective. Organ meats, especially beef liver, are the most concentrated sources, providing over 12,000 µg of copper per 100 grams—far exceeding the Recommended Dietary Allowance (RDA) of 900 µg per day for adults, per the National Institutes of Health (NIH). Other sources include shellfish like oysters and lobster, which contain 2,000 to 4,800 µg per 100 grams, as well as nuts, seeds, and legumes.

For significant deficiencies or impaired absorption, oral copper supplementation is often necessary. Copper sulfate and copper gluconate are commonly used, with doses ranging from 2 to 4 mg per day. A clinical review in The American Journal of Hematology reported that patients with anemia and neuropathy from copper deficiency showed improvement with 2 mg of elemental copper daily, though neurological recovery was slower than hematologic correction. To minimize gastrointestinal discomfort, copper supplements are best taken with food and divided into smaller doses throughout the day.

In cases where oral supplementation is ineffective—such as in patients with malabsorption syndromes or post-gastric bypass—parenteral copper administration may be needed. Intravenous copper histidinate or copper chloride is administered under medical supervision, with dosing tailored to individual needs. A case study in Clinical Nutrition described a patient with severe copper deficiency myelopathy who received intravenous copper at 2 mg per day for five days, followed by weekly maintenance doses, leading to partial neurological recovery over months. Long-term monitoring of serum copper levels is essential to ensure adequate repletion and prevent recurrence.

Neurological Rehabilitation

Rehabilitation focuses on improving motor function, sensory deficits, and overall quality of life. While copper repletion can halt neurological deterioration, many individuals experience lingering deficits, making rehabilitation crucial. Recovery depends on factors such as deficiency duration, spinal cord involvement, and treatment promptness.

For patients with gait abnormalities, structured physical therapy strengthens muscles, enhances coordination, and reduces spasticity. Gait retraining, balance drills, and proprioceptive exercises help restore mobility, while assistive devices like ankle-foot orthoses or walkers may be necessary for persistent motor deficits.

Occupational therapy addresses fine motor difficulties and sensory impairments affecting daily activities. Task-specific exercises improve hand coordination and grip strength, while sensory re-education techniques, including vibration therapy and textured surface exposure, help retrain tactile processing. Functional electrical stimulation (FES) has also been explored for lower limb weakness, enhancing neuromuscular activation and walking efficiency.

While full neurological recovery is not always possible, a well-structured rehabilitation plan tailored to individual needs can maximize independence and improve overall well-being.