Subacute combined degeneration of the cord

Case contributed by Christine Goh
Diagnosis certain

Presentation

Reduced sensation arms and legs.

Patient Data

Age: 54
Gender: Male

Abnormal T2 hyperintensity along the dorsal columns from C2 level extending to the C5.  The pattern of dorsal column involvement is highly suggestive of subacute combined degeneration of the cord.

The remainder of the cord, and visualized portions of the brainstem appear unremarkable. The cord is not expanded. No nerve root thickening noted.

Laboratory investigations:

  • Plasma copper: 17.4 nmol/L (11-33)
  • ceruloplasmin: 0.2 g/L (0.20 - 0.60)
  • B12: <61 pmol/L (140-650)
  • serum folate: 37.4 nmol/L (>7.0)
  • macrocytic anemia

Case Discussion

Vitamin B12 deficiency causes impairment of normal DNA and fatty acid synthesis resulting in megaloblastic anemia, loss of proprioception and vibration sense with sensory disturbance.

MR imaging findings of subacute combined-degeneration of the cord is of long-segment, bilateral T2 hyperintensity of the dorsal columns giving rise to an “inverted V” or “rabbit-ear” appearance .  In severe cases, signal abnormality can extend proximally to involve the  brainstem and cerbellum.

Causes of B12 deficiency include pernicious anemia, ileal or gastric fundal resection and severely restricted diet.

Copper deficiency, most commonly related to reduced absorption after gastric surgery, can cause an indistinguishable clinical and radiological appearance.

Nitrous oxide administration can provoke symptomatic B12 deficiency in patients with previously subclinical deficiency due to its action in oxidizing active cobalamin into its inactive form.

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