Cold face test demonstrates parasympathetic cardiac dysfunction in familial dysautonomia

M. J. Hilz, B. Stemper, P. Sauer, U. Haertl, Wolfgang Singer, F. B. Axelrod

Research output: Contribution to journalArticle

51 Citations (Scopus)

Abstract

In familial dysautenomia (FD)., i.e., Riley-Day syndrome, parasympathetic dysfunction has not been sufficiently evaluated. The cold face test is a noninvasive method of activating trigeminal brain stem cardiovagal and sympathetic pathways and can be performed in patients with limited cooperation. We performed cold face tests in 11 FD patients and 15 controls. For 60 s, cold compresses (0-1°C) were applied to the cheeks and forehead while we monitored heart rate, respiration, beat-to-beat radial artery blood pressure, and laser-Doppler skin blood flow at the first toe pulp. From these measurements heart rate variability parameters were calculated: root mean square of successive differences (RMSSD), coefficient of variation (CV), low- and high-frequency (LF and HF, respectively) power spectra of the electrocardiogram, and the LF transfer function gain between blood pressure and heart rate. All patients perceived cold stimulation and acknowledged discomfort. In controls, heart rate and skin blood flow decreased significantly during cold face test; in patients, both parameters decreased only briefly and not significantly. In controls, blood pressure, RMSSD, CV, and heart rate HF-power spectra increased but remained unchanged in patients. Respiration, as well as heart rate LF power spectra, did not change in either group. In controls, LF transfer function gain between blood pressure and heart rate indicated that bradycardia was not secondary to blood pressure increase. We conclude that the cold face test demonstrated that patients with FD have a reduced cardiac parasympathetic response, which implies efferent parasympathetic dysfunction.

Original languageEnglish (US)
JournalAmerican Journal of Physiology - Regulatory Integrative and Comparative Physiology
Volume276
Issue number6 45-6
StatePublished - Jun 1999
Externally publishedYes

Fingerprint

Familial Dysautonomia
Heart Rate
Blood Pressure
Skin
Radial Artery
Forehead
Cheek
Toes
Bradycardia
Respiratory Rate
Brain Stem
Electrocardiography
Respiration
Lasers

Keywords

  • Autonomic neuropathy
  • Hereditary sensory neuropathy
  • Parasympathetic cardiac dysfunction
  • Riley-Day syndrome
  • Sympathetic dysfunction
  • Trigeminal brain stem vagal pathways

ASJC Scopus subject areas

  • Physiology
  • Physiology (medical)

Cite this

Cold face test demonstrates parasympathetic cardiac dysfunction in familial dysautonomia. / Hilz, M. J.; Stemper, B.; Sauer, P.; Haertl, U.; Singer, Wolfgang; Axelrod, F. B.

In: American Journal of Physiology - Regulatory Integrative and Comparative Physiology, Vol. 276, No. 6 45-6, 06.1999.

Research output: Contribution to journalArticle

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