Objective:
To highlight the importance of early diagnosis and treatment of a rare, reversible cause of progressive spinal cord disorder from copper deficiency
Background:
Copper deficiency myelopathy (CDM) is a treatable, but underrecognized cause of progressive spinal cord degeneration. Copper is necessary in many enzymes critical for myelin maintenance in the dorsal column and corticospinal tracts. In addition, its deficiency can cause cytopenia in multiple cell lines due to its function as a cofactor in hematopoiesis. Patients with excess zinc ingestion, malabsorption such as Crohn’s disease, or gastric bypass surgery history are at risk for this condition.
Results:
A 30-year-old with irritable bowel syndrome on a laxative treatment presented with a two-month history of intermittent muscle spasm, bilateral lower extremity paresthesia, and generalized weakness. Neurological exam showed upper motor neuron signs (spasticity, hyperreflexia, clonus, Hoffman’s sign), and spastic gait.
MRI brain and cervical spine, and EMG were unremarkable. Autoimmune myelopathy panel was negative. Infectious workup, including HIV, syphilis, and HTLV I/II, was unrevealing. Serum copper was low (74 µg/dL, normal 80–158 µg/dL), but serum zinc and vitamin B12 levels were within normal limits.
The patient improved with 2mg of oral copper supplement and cessation of laxative use. A year later, the patient became pregnant and was monitored with monthly copper, zinc, folate, and ceruloplasmin levels. She required increased copper dose to 6mg during her 1st trimester, and stopped copper supplements in her 2nd trimester, with complete resolution of symptoms.
Conclusions:
Treatment can range from 2-8mg oral copper in mild CDM, to 4mg intravenous copper for 6 days in severe CDM. If CDM is left untreated, it can cause permanent dorsal column degeneration and bone marrow dyscrasias. Close follow up with appropriate dosing of copper supplementation is important in patients with CDM, especially in pregnancy, as normal ranges of copper and ceruloplasmin can double.
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