Archives of neurology · 2004
Copper deficiency myelopathy.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Kumar Neeraj, Crum Brian, Petersen Ronald C, Vernino Steven A, Ahlskog J Eric
Аннотация
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.
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.