heart, cold stress and CMT

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From the Archives of Physical and Medical Rehabilitation. 1994

Jul;75(7):787-91.

Abnormal responses to cold stress in Charcot-Marie-Tooth I syndrome.

LL, Vieth RF, FS.

Department of Pediatric Neurology, Ohio State University College of

Medicine, Columbus.

In Charcot-Marie-Tooth syndrome (CMT, Hereditary Motor Sensory

Neuropathy), patient complaints of cold intolerance are common but their

peripheral responses to cold have not been documented. Using digital

plethysmography, a simple test of vascular reactivity with 1 minute cold

stress, 20 unrelated adult CMT patients showed a significantly increased

average heart rate and decreased average arterial oxygen saturation

following cold when compared to 50 age-matched normal controls. There

did not appear to be a unique or characteristic CMT vascular reaction to

cold stress in CMT patients because their abnormal peripheral vascular

responses were variable. Variability in CMT neuropathic responses to

cold is consistent with the known irregular segmental demyelination of

CMT peripheral nerves as well as abnormal CMT sweating patterns. Though

understanding the precise patterns of CMT patient peripheral

nerve disturbances remains difficult, awareness of CMT patient's

abnormal responses to cold may facilitate CMT patient care and

rehabilitation.