Copper deficiency myelopathy mimicking cervical spondylitic myelopathy: a systematic review of the literature with case report

• Published in: The spine journal 2024-11, Vol.24 (11), p.2026-2034
• Main Authors: Chen, Jeffrey W., Zeoli, Tyler, Hughes, Natasha C., Lane, Amanda, Berkman, Richard A.
• Format: Article
• Language: English
• Subjects: Adult; Aged; Cervical spondylitic myelopathy; Cervical Vertebrae - surgery; Copper - blood; Copper - deficiency; Copper deficiency myelopathy; CSM; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Myelopathy; Spinal Cord Diseases - blood; Spinal Cord Diseases - diagnosis; Spinal Cord Diseases - etiology; Spinal Cord Diseases - surgery; Spondylosis - complications; Spondylosis - diagnosis
• ISSN: 1529-9430; 1878-1632; 1878-1632
• DOI: 10.1016/j.spinee.2024.06.018

•Copper deficiency myelopathy (CDM) is a rare disease of progressive dorsal column dysfunction resulting in sensory ataxia, weakness, and spasticity.•Patients presenting to a spine surgeon with myelopathy that progress in spite of adequate surgical decompression, or myelopathy concomitant with cytopenia, require further investigation for copper deficiency myelopathy.•Clues to copper deficiency include a distinct MRI T2 hyperintense inverted “V” signal in the dorsal columns in a patient with risk factors for copper deficiency such as gastric bypass, bariatric surgery, or excessive zinc exposure.•Hematological abnormalities resolve rapidly with replenishment of copper, while neurological deficits only stabilize or partially improve Appropriate copper supplementation therapy may lead to a reversal of T2 signal-intensity changes in the dorsal columns. Copper deficiency myelopathy (CDM) is a rare disease that can present with spastic quadriparesis and sensory ataxia. As a result, it can precisely mimic cervical spondylitic myelopathy (CSM). Copper deficiency may be seen following gastric bypass surgery, malabsorption syndromes such as celiac disease, and with excessive exogenous zinc intake. We present a systematic review of the literature for CDM and an illustrative case. Provide a systematic review of CDM to highlight the importance of recognizing the consideration of CDM in patients presenting to a spine surgeon with myelopathy that progress despite adequate surgical decompression, or myelopathy concomitant with cytopenia, thus requiring further workup. Retrospective medical record review and systematic review of the literature. PubMed and Ovid-Embase database search was conducted in July 2022. Self-reported measures include PRISMA flow diagram for retrospective review; Physiological measures include retrospective review of MRI imaging of cervical spine; alternate demographic and laboratory value data extracted via literature review. A PubMed and Ovid-Embase database search was conducted in July 2022 searching for “copper deficiency myelopathy (MeSH)” from 2000 to 2022 via PRISMA guidelines. Following title and abstract review, the following data was extracted from full text: age, sex, etiology, hematological values upon presentation (mean corpuscular volume, white blood count, platelet count, and hemoglobin level), metal serum studies (serum copper, ceruloplasmin, and zinc), 24-hour collection of copper and zinc, and distinct radiographic findings on MRI. A