Copper deficiency myelopathy: A report of two cases

Abstract

Context: Copper deficiency myelopathy represents an often underdiagnosed, acquired neurological syndrome, clinically characterized by posterior column dysfunction. The main causes of copper deficiency are bariatric surgery, increased consumption of zinc, and malabsorption. However, even after a careful history taking and extensive laboratory researches, the etiology of copper deficiency remains undetermined in a significant percentage of cases. Patients affected by copper deficiency myelopathy usually present with sensory ataxia due to dorsal column dysfunction and sometimes with mild leg spasticity. In such patients, spinal cord magnetic resonance imaging (MRI) may show hyperintense lesions in T2-weighted sequences involving the posterior columns of cervical and thoracic cord. These MRI findings are not distinguishable from those of subacute combined degeneration associated with vitamin B12 deficiency. Findings: Here, we describe two patients with gait ataxia and sensory symptoms in which a diagnosis of copper deficiency myelopathy was made. Both patients showed a significant clinical, neuroradiological, and neurophysiological improvement after proper supplementation therapy. Conclusion: The patients herein described underline the importance to include serum copper and ceruloplasmin levels as part of the myelopathy diagnostic workup, especially in the cases of otherwise unexplained subacute myelopathy involving the posterior columns. Since copper deficiency myelopathy is a progressive syndrome, early diagnosis is mandatory in order to promptly provide a proper supplementation therapy and, thus, prevent an irreversible neurological damage.

Copper Deficiency Myelopathy After Upper Gastrointestinal Surgery

“…Plantone et al described 2 cases of women with gait ataxia (1 mild and 1 moderate) aged 50 and 53 years with MRI dorsal column findings similar to those seen in our case, with low copper levels but with no cause for the copper deficiency found. Interestingly, oral suplementation at 8 mg/d of copper led to significant clinical and radiologic improvement . Kumar et al described 3 cases of copper deficiency myelopathy; for the 1 man (72 years old, who had undergone partial gastrectomy at age 26), MRI findings showed hyperintense signal change on T2 weighting in the dorsal columns.…”

“…Interestingly, oral suplementation at 8 mg/d of copper led to significant clinical and radiologic improvement. 15 Kumar et al described 3 cases of copper deficiency myelopathy; for the 1 man (72 years old, who had undergone partial gastrectomy at age 26), MRI findings showed hyperintense signal change on T2 weighting in the dorsal columns. He had a vitamin B12 deficiency, but symptoms of ascending paraesthesia, increased limb weaknes, and ataxia progressed, despite replacement.…”

An Atypical Case of Subacute Combined Degeneration With Concomitant Copper Deficiency

“…Copper deficiency reported to have a range of neurological manifestations such as myelopathy, peripheral neuropathy (7). Patients may present with sensory ataxia due to copper deficiency myelopathy which represents an often underdiagnosed, acquired neurological syndrome (24). Similar to subacute combined degeneration, copper deficiency myelopathy is characterized by posterior column dysfunction.…”

PPIs as possible risk factor for copper deficiency myelopathy

“…We described two patients affected by copper deficiency myelopathy (CDM) that showed a significant clinical, neuroradiological, and neurophysiological improvement after proper supplementation therapy [5] and we recently diagnosed three further cases of CDM (unpublished data). Therefore, based on our data, we would like to discuss one important issue in relation to the etiology of CDM.…”