title: Clinical and electrodiagnostic findings in copper deficiency myeloneuropathy.
abstract: BACKGROUND
  Copper deficiency is an increasingly recognised cause of neurological impairment. This retrospective review highlights clinical and electrodiagnostic findings in patients diagnosed at our institution with copper deficiency.
  
  
  METHODS
  Clinical, radiographic and electrodiagnostic findings were reviewed in patients with evidence of copper deficiency. Patients with other potential causes of myelopathy or neuropathy were excluded.
  
  
  RESULTS
  The predominant clinical feature in all six patients was a sensory ataxia, resulting in marked gait unsteadiness. Nerve conduction studies and needle EMG were performed in all patients and revealed a mild to moderate distal, axonal, sensorimotor peripheral neuropathy. Median and tibial somatosensory evoked potentials were abnormal in all five patients in which it was performed, showing impaired conduction in central or proximal peripheral somatosensory pathways.
  
  
  CONCLUSIONS
  This pattern of electrodiagnostic findings suggests that impairment in somatosensory pathways demonstrated by somatosensory evoked potential testing is the main cause of the sensory ataxia in patients with copper deficiency.
authors.affiliation: Department of Neurology, Mayo Clinic, Scottsdale, AZ 85259, USA. goodman.brent@mayo.edu
authors.forename: B P
authors.initials: BP
authors.lastname: Goodman
authors.forename: E P
authors.initials: EP
authors.lastname: Bosch
authors.forename: M A
authors.initials: MA
authors.lastname: Ross
authors.forename: C
authors.initials: C
authors.lastname: Hoffman-Snyder
authors.forename: D D
authors.initials: DD
authors.lastname: Dodick
authors.forename: B E
authors.initials: BE
authors.lastname: Smith
mesh_terms: D000368:Aged; D003300:Copper / Q000172:deficiency*; D004568:Electrodiagnosis; D004576:Electromyography; D005073:Evoked Potentials, Somatosensory / Q000502:physiology; D005260:Female; D020233:Gait Disorders, Neurologic / Q000503:physiopathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000503:physiopathology; D009431:Neural Conduction / Q000502:physiology; D009460:Neurologic Examination; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D012189:Retrospective Studies; D013118:Spinal Cord Diseases / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology
chemical_list: D003300:Copper