Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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January 28, 2016
Copper Deficiency Myelopathy
- Background:
- Copper deficiency in humans is rare, as copper has a low daily requirement and is present in many foods.
- Gastric surgery, excess zinc intake, and malabsorption are causes of copper deficiency.
- Zinc is known to induce the synthesis of metallothionein in enterocytes; copper binds tightly to metallothionein, which prevents its absorption.
- Severe deficiency can lead to spastic gait and sensory ataxia. Other complications include anemia, peripheral neuropathy, and CNS demyelination.
- Clinical Information:
- This patient reported using denture cream containing zinc for over 20 years and was treated for giardiasis several weeks before her symptoms started.
- Copper levels were undetectable in her blood and low in her urine. Vitamin B12 and methylmalonic acid levels were within normal limits. CBC also showed pancytopenia.
- Physical exam showed symmetric bilateral upper and lower extremity weakness causing gait disturbance, as well as deficits in proprioception and vibratory sensation.
- Lab abnormalities and physical exam findings gradually improved with treatment.
- Key Diagnostic Features:
- MRI of the spine typically shows increased T2 signal in the dorsal columns of the cervical and thoracic cord at multiple levels.
- Brain MRI findings of scattered T2-hyperintense white matter lesions that do not enhance or restrict diffusion are nonspecific and are likely incidental in this patient.
- Differential Diagnosis:
- Spine MRI findings:
- Vitamin B12 deficiency (subacute combined degeneration)
- Nitrous oxide toxicity
- HIV vacuolar myelopathy
- Herpes virus infection
- Neurosyphilis (tabes dorsalis)
- Multiple sclerosis
- Acute disseminated encephalomyelitis (ADEM)
- Brain MRI findings:
- Chronic microvascular ischemia
- Migraines
- Multiple sclerosis
- Small vessel vasculitis
- Spine MRI findings:
- Treatment:
- Intravenous or oral copper supplementation, treating the underlying cause of malabsorption, and cessation of high zinc intake. Proper treatment prevents further neurologic compromise but improvement of neurologic signs and symptoms can be variable.
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