BACKGROUND: In humans, Menkes disease is the well-recognized neurological disorder due to inherited copper deficiency. Myelopathy due to acquired copper deficiency is not a well-recognized entity in humans, although myelopathy due to copper deficiency is well documented in some animal species. PATIENTS: We describe 3 patients who developed a progressive spastic-ataxic gait with proprioceptive deficits. All patients had a severe reduction in serum ceruloplasmin and copper levels. RESULTS: All patients had evidence of posterior column dysfunction clinically and on somatosensory evoked potential studies. Two had a signal change in the posterior column on magnetic resonance imaging of the spinal cord. CONCLUSION: Patients presenting with otherwise unexplained myelopathies should have their serum ceruloplasmin level measured.
BACKGROUND: In humans, Menkes disease is the well-recognized neurological disorder due to inherited copper deficiency. Myelopathy due to acquired copper deficiency is not a well-recognized entity in humans, although myelopathy due to copper deficiency is well documented in some animal species. PATIENTS: We describe 3 patients who developed a progressive spastic-ataxic gait with proprioceptive deficits. All patients had a severe reduction in serum ceruloplasmin and copper levels. RESULTS: All patients had evidence of posterior column dysfunction clinically and on somatosensory evoked potential studies. Two had a signal change in the posterior column on magnetic resonance imaging of the spinal cord. CONCLUSION:Patients presenting with otherwise unexplained myelopathies should have their serum ceruloplasmin level measured.
Authors: Farhard Khimani; Ryan Livengood; Olukemi Esan; Jeffrey A Vos; Vivek Abhyankar; Ludwig Gutmann; William Tse Journal: Am J Stem Cells Date: 2013-06-30
Authors: Marco Spinazzi; Franca De Lazzari; Bruno Tavolato; Corrado Angelini; Renzo Manara; Mario Armani Journal: J Neurol Date: 2007-04-06 Impact factor: 4.849