Copper deficiency myelopathy.
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.


METHODS
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.


CONCLUSIONS
Patients presenting with otherwise unexplained myelopathies should have their serum ceruloplasmin level measured.
Kumar
Neeraj
N
Department of Neurology, Mayo Clinic, Mayo Foundation, Rochester, Minn, USA. kumar.neeraj@mayo.edu
Crum
Brian
B
Petersen
Ronald C
RC
Vernino
Steven A
SA
Ahlskog
J Eric
JE
D000368:Aged; D002570:Ceruloplasmin / Q000172:deficiency*; D003300:Copper / Q000097:blood / Q000172:deficiency*; D005073:Evoked Potentials, Somatosensory; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008659:Metabolic Diseases / Q000097:blood / Q000209:etiology / Q000503:physiopathology; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000097:blood / Q000209:etiology / Q000503:physiopathology; D013116:Spinal Cord / Q000473:pathology / Q000503:physiopathology; D013118:Spinal Cord Diseases / Q000097:blood / Q000209:etiology* / Q000503:physiopathology
D003300:Copper; D002570:Ceruloplasmin