Case of sensory ataxic ganglionopathy–myelopathy in copper deficiency

• Published in: Journal of the neurological sciences Vol. 277; no. 1; pp. 184 - 186
• Main Authors: Zara, Gabriella, Grassivaro, Francesca, Brocadello, Filippo, Manara, Renzo, Pesenti, Francesco Francini
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier B.V 15-02-2009; Elsevier
• Subjects: Adult; Ataxia - drug therapy; Ataxia - etiology; Ataxia - pathology; Ataxic myelopathy; Biological and medical sciences; Ceruloplasmin - deficiency; Ceruloplasmin - metabolism; Copper - blood; Copper - deficiency; Copper - therapeutic use; Female; Ganglia, Spinal - metabolism; Ganglia, Spinal - pathology; Ganglionopathy; Humans; Iron - blood; Iron - deficiency; Magnetic Resonance Imaging; Medical sciences; Neurology; Posterior Horn Cells - metabolism; Posterior Horn Cells - pathology; Tumors of the nervous system. Phacomatoses; Ganglionopathy; Ataxic myelopathy; Copper deficiency; Myelopathy; Nervous system diseases; Deficiency; Central nervous system disease; Copper; Spinal cord disease
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/j.jns.2008.10.017

Abstract Spinal cord involvement associated with severe copper deficiency has been reported in the last 8 years. Copper deficiency may produce an ataxic myelopathy. Clinical and neuroimaging findings are similar to the subacute combined degeneration seen in patients with vitamin B12 deficiency. Macrocytic, normocytic and microcytic anemia, leukopenia and, in severe cases, pancytopenia are well known hematologic manifestations. The most patients with copper deficiency myelopathy had unrecognized carency. Some authors suggested that early recognition and copper supplementation may prevent neurologic deterioration but clinical findings do not improve. We present a patient with copper deficiency, dorsal root ganglions and cervical dorsal columns involvement. Clinical status and neuroimaging improved after copper replacement therapy. Sensory neurons of dorsal root ganglia may be the most sensitive nervous pathway. In this case the early copper treatment allowed to improve neurologic lesions and to prevent further involvements.