, Volume 48, Issue 2, pp 78–83 | Cite as

Imaging features of copper deficiency myelopathy: a study of 25 cases

  • Neeraj KumarEmail author
  • J. Eric Ahlskog
  • Christopher J. Klein
  • John D. Port
Diagnostic Neuroradiology


Acquired copper deficiency presents with a spastic gait and sensory ataxia. Spinal cord magnetic resonance imaging (MRI) in patients with copper deficiency myelopathy may show increased T2 signal, most commonly in the dorsal midline cervical and thoracic cord. These imaging findings may be reversible with normalization of serum copper. The clinical and imaging picture is very similar to the subacute combined degeneration seen in patients with vitamin B12 deficiency. Neuroradiologists should consider this possibility when a long segment of symmetric dorsal spinal cord T2-hyperintensity is identified.


Copper deficiency Myelopathy Magnetic resonance Cord signal 


  1. 1.
    Bottomley SS (2004) Sideroblastic anemias. In: Greer JP, Foerster J, Lukens JN, Rodgers GM, Paraskevas F, Glader B (eds) Wintrobe's clinical hematology, 11th edn. Lippincott, Philadelphia, pp 1011–1033Google Scholar
  2. 2.
    Schleper B, Stuerenburg HJ (2001) Copper deficiency-associated myelopathy in a 46-year-old woman. J Neurol 248:705–706PubMedCrossRefGoogle Scholar
  3. 3.
    Gregg XT, Reddy V, Prchal JT (2002) Copper deficiency masquerading as myelodysplastic syndrome. Blood 100:1493–1495PubMedCrossRefGoogle Scholar
  4. 4.
    Prodan CI, Holland NR, Wisdom PJ, Burstein SA, Bottomley SS (2002) CNS demyelination associated with copper deficiency and hyperzincemia. Neurology 59:1453–1456PubMedCrossRefGoogle Scholar
  5. 5.
    Kumar N, Gross JB Jr, Ahlskog JE (2003) Myelopathy due to copper deficiency. Neurology 61:273PubMedGoogle Scholar
  6. 6.
    Hedera P, Fink JK, Bockenstedt PL, Brewer GJ (2003) Myelopolyneuropathy and pancytopenia due to copper deficiency and high zinc levels of unknown origin: further support for existence of a new zinc overload syndrome. Arch Neurol 60:1303–1306PubMedCrossRefGoogle Scholar
  7. 7.
    Kumar N, McEvoy KM, Ahlskog JE (2003) Myelopathy due to copper deficiency following gastrointestinal surgery. Arch Neurol 60:1782–1785PubMedCrossRefGoogle Scholar
  8. 8.
    Greenberg SA, Briemberg HR (2004) A neurological and hematological syndrome associated with zinc excess and copper deficiency. J Neurol 251:111–114PubMedCrossRefGoogle Scholar
  9. 9.
    Kumar N, Crum B, Petersen RC, Vernino S, Ahlskog JE (2004) Copper deficiency myelopathy. Arch Neurol 61:762–766PubMedCrossRefGoogle Scholar
  10. 10.
    Kumar N, Low PA (2004) Myeloneuropathy and anemia due to copper malabsorption. J Neurol 251:747–749PubMedCrossRefGoogle Scholar
  11. 11.
    Kumar N, Gross JB, Ahlskog JE (2004) Copper deficiency myelopathy produces a clinical picture like subacute combined degeneration. Neurology 63:33–39PubMedGoogle Scholar
  12. 12.
    Kumar N, Ahlskog EA, Gross JB (2004) Acquired hypocupremia after gastric surgery. Clin Gastroenterol Hepatol 2:1074–1079PubMedCrossRefGoogle Scholar
  13. 13.
    Jubb KVF, Huxtable CR (1993) The nervous system. In: Jubb KVF, Kennedy PC, Palmer N (eds) Pathology of domestic animals, 4th edn. Academic Press, San Diego, pp 267–439Google Scholar
  14. 14.
    Tan N, Urich H (1983) Menkes' Disease and Swayback—a comparative study of two copper deficiency syndromes. J Neurol Sci 62:95–113PubMedCrossRefGoogle Scholar
  15. 15.
    Penrith ML, Tustin RC, Thornton DJ, Burdett PD (1996) Swayback in a blesbok (Damaliscus dorcas phillipsi) and a black wildebeest (Connochaetes gnou). J S Afr Vet Assoc 67:93–96PubMedGoogle Scholar
  16. 16.
    Barlow RM (1963) Further observations on swayback, Part 1 (Transitional pathology). J Comp Pathol 73:51–60PubMedGoogle Scholar
  17. 17.
    Alleyne T, Joseph J, Lalla A, Sampson V, Adogwa A (1998) Cytochrome-c oxidase isolated from the brain of swayback-diseased sheep display unusual structure and uncharacteristic kinetics. Mol Chem Neuropathol 34:233–247PubMedCrossRefGoogle Scholar
  18. 18.
    Kumar N, Ahlskog JE (2004) Myeloneuropathy due to copper deficiency or zinc excess? (letter) Arch Neurol 61:604–605PubMedCrossRefGoogle Scholar
  19. 19.
    Hedera P, Brewer GJ (2004) Myeloneuropathy due to copper deficiency or zinc excess? Arch Neurol 61:605 (letter)CrossRefGoogle Scholar
  20. 20.
    Katsaros VK, Glocker FX, Hemmer B, Schumacher M (1998) MRI of spinal cord and brain lesions in subacute combined degeneration. Neuroradiology 40:716–719PubMedCrossRefGoogle Scholar
  21. 21.
    Locatelli ER, Laureno R, Ballard P, Mark AS (1999) MRI in vitamin B12 deficiency myelopathy. Can J Neurol Sci 26:60–63PubMedGoogle Scholar
  22. 22.
    Misra UK, Kalita J, Das A (2003) Vitamin B12 deficiency neurological syndromes: a clinical, MRI and electrodiagnostic study. Electromyogr Clin Neurophysiol 43:57–64PubMedGoogle Scholar
  23. 23.
    Larner AJ, Zeman AZ, Allen CM, Antoun NM (1997) MRI appearances in subacute combined degeneration of the spinal cord due to vitamin B12 deficiency. J Neurol Neurosurg Psychiatry 62:99–100PubMedCrossRefGoogle Scholar
  24. 24.
    Beltramello A, Puppini G, Cerini R, El-Dalati G, Manfredi M, Roncolato G et al (1998) Subacute combined degeneration of the spinal cord after nitrous oxide anaesthesia: role of magnetic resonance imaging. J Neurol Neurosurg Psychiatry 64:563–564PubMedGoogle Scholar
  25. 25.
    Wolansky LJ, Goldstein G, Gozo A, Lee HJ, Sills I, Chatkupt S (1995) Subacute combined degeneration of the spinal cord: MRI detection of preferential involvement of the posterior columns in a child. Pediatr Radiol 25:140–141PubMedCrossRefGoogle Scholar
  26. 26.
    Kuker W, Hesselmann V, Thron A, de Simone A (1997) MRI demonstration of reversible impairment of the blood-CNS barrier function in subacute combined degeneration of the spinal cord. J Neurol Neurosurg Psychiatry 62:298–299PubMedGoogle Scholar
  27. 27.
    Karantanas AH, Markonis A, Bisbiyiannis G (2000) Subacute combined degeneration of the spinal cord with involvement of the anterior columns: a new MRI finding. Neuroradiology 42:115–117PubMedCrossRefGoogle Scholar
  28. 28.
    Berger JR, Quencer R (1991) Reversible myelopathy with pernicious anemia: clinical/MR correlation. Neurology 41:947–948PubMedGoogle Scholar
  29. 29.
    Tajima Y, Mito Y, Owada Y, Moriwaka F, Tashiro K (1994) MR appearance of subacute combined degeneration of the spinal cord. Jpn J Psychiatry Neurol 48:611–614PubMedGoogle Scholar
  30. 30.
    Bassi SS, Bulundwe KK, Greeff GP, Labuscagne JH, Gledhill RF (1999) MRI of the spinal cord in myelopathy complicating vitamin B12 deficiency: two additional cases and a review of the literature. Neuroradiology 41:271–274PubMedCrossRefGoogle Scholar
  31. 31.
    Sobue G, Yasuda T, Kumazawa K, Yamamoto K, Mitsuma T (1995) MRI demonstrates dorsal column involvement of the spinal cord in Sjogren's syndrome-associated neuropathy. Neurology 45:592–593PubMedGoogle Scholar
  32. 32.
    Yasuda T, Sobue G, Hirose Y, Mimura M, Yanagi T (1994) MR of acute autonomic and sensory neuropathy. Am J Neuroradiol 15:114–115PubMedGoogle Scholar
  33. 33.
    Kieburtz KD, Giang DW, Schiffer RB, Vakil N (1991) Abnormal vitamin B12 metabolism in human immunodeficiency virus infection. Association with neurological dysfunction. Arch Neurol 48:312–314PubMedGoogle Scholar
  34. 34.
    Konagaya M, Matsumoto A, Takase S, Mizutani T, Sobue G, Konishi T et al (2004) Clinical analysis of longstanding subacute myelo-optico-neuropathy: sequelae of clioquinol at 32 years after its ban. J Neurol Sci 218:85–90PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • Neeraj Kumar
    • 1
    • 3
    Email author
  • J. Eric Ahlskog
    • 1
  • Christopher J. Klein
    • 1
  • John D. Port
    • 2
  1. 1.Department of NeurologyMayo ClinicRochesterUSA
  2. 2.Department of RadiologyMayo ClinicRochesterUSA
  3. 3.RochesterUSA

Personalised recommendations