Imaging the neural correlates of neuropathic pain and pleasurable relief associated with inherited erythromelalgia in a single subject with quantitative arterial spin labelling

Pain. 2012 May;153(5):1122-1127. doi: 10.1016/j.pain.2011.12.012. Epub 2012 Feb 24.


We identified a patient with severe inherited erythromelalgia secondary to an L858F mutation in the voltage-gated sodium channel Na(v)1.7. The patient reported severe ongoing foot pain, which was exquisitely sensitive to limb cooling. We confirmed this heat hypersensitivity using quantitative sensory testing. Additionally, we employed a novel perfusion imaging technique in a simple block design to assess her baseline erythromelalgia pain vs cooling relief. Robust activations of key pain, pain-affect, and reward-related centres were observed. This combined approach allowed us to confirm the presence of a temperature-sensitive channelopathy of peripheral neurons and to investigate the neural correlates of tonic neuropathic pain and relief in a single subject.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Brain / physiopathology*
  • Erythromelalgia / genetics
  • Erythromelalgia / physiopathology*
  • Female
  • Functional Neuroimaging / methods*
  • Humans
  • NAV1.7 Voltage-Gated Sodium Channel
  • Neuralgia / genetics
  • Neuralgia / physiopathology*
  • Pleasure / physiology*
  • Sensory Thresholds / physiology
  • Sodium Channels / genetics
  • Spin Labels


  • NAV1.7 Voltage-Gated Sodium Channel
  • SCN9A protein, human
  • Sodium Channels
  • Spin Labels