Case of the Week

Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada

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December 15, 2014

Subacute Combined Degeneration

  • Subacute combined degeneration is an acquired myelopathy caused by vitamin B12 deficiency, and is a rare cause of demyelination of the dorsal columns of the spinal cord.
  • Clinical Presentation: Profound neurologic déficits, including progressive sensory abnormalities, ascending paresthesias, weakness, ataxia, loss of sphincter control, and gait impairment. Megaloblastic anemia is a common early symptom leading to the diagnosis; although, neurologic symptoms may occur in the absence of haematologic abnormalities.
  • Key Diagnostic Features:
    • Symmetric increased signal seen on T2 and STIR images within the dorsal (and less commonly, lateral) columns: "inverted V" sign in axial plane.
    • Mild mass effect and mild contrast enhancement may be present.
  • DDx:
    • Dorsal column myelopathy
    • HIV myelopathy
    • Nitrous oxide toxicity
    • Copper deficiency
    • Cyanide toxicity
    • Spinal cord infarction
    • Demyelination
    • Other causes of transverse myelitis
  • Rx: Vitamin B12 therapy
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