Warmth and nociceptive evoked potentials in cold-induced sweating syndrome type 1

Muscle Nerve. 2016 Jun;54(1):100-3. doi: 10.1002/mus.24976. Epub 2016 Feb 26.


Introduction: Cold-induced sweating syndrome type 1 (CISS1), is a rare, severe, autosomal recessive disease. It is characterized by morphological alterations and profuse sweating when ambient temperature is <22 °C. Although some individuals with CISS1 have decreased pain perception, no study has been conducted to evaluate thermal and pain sensations in these patients. The aim of this study was to assess the function of the nociceptive Aδ-fibers and warmth C-fibers by using CO2 laser-evoked potentials (LEPs) in patients affected by CISS1.

Methods: Four patients were studied. Laser pulses were applied to the skin of the right hand and the perioral region at painful intensity to record Aδ-LEPs, and at non-painful intensity to obtain C-LEPs. Fifteen healthy subjects were studied for control purposes.

Results: No significant difference in latencies or amplitudes of either Aδ- or C-LEPs was found between the 2 groups.

Conclusion: Cutaneous nociceptive and warmth pathway functions are normal in CISS1. Muscle Nerve 54: 100-103, 2016.

Keywords: Aδ-fibers; C-fibers; CISS1; cold-induced sweating; laser-evoked potentials.

MeSH terms

  • Adolescent
  • Adult
  • Death, Sudden
  • Facies
  • Female
  • Hand Deformities, Congenital / physiopathology*
  • Humans
  • Hyperhidrosis / physiopathology*
  • Laser-Evoked Potentials / physiology*
  • Male
  • Nociception / physiology*
  • Reaction Time / physiology
  • Skin / innervation
  • Temperature*
  • Trismus / congenital*
  • Trismus / physiopathology

Supplementary concepts

  • Crisponi syndrome