Abstract
In Japan, quite a few patients with spinal muscular atrophy type 1 (SMA type 1) survive
with mechanical ventilation. Since a patient with SMA type 1 and continuous artificial
ventilation exhibited excessive perspiration and tachycardia, we examined the autonomic
functions in three cases of SMA type 1, undergoing mechanical ventilation. Two cases
exhibited the common sympathetic-vagal imbalance on R–R interval analysis involving
24-h Holter ECG recordings in addition to an abnormality in finger cold-induced vasodilatation.
Furthermore, one case showed blood pressure and heart rate fluctuation with the paroxysmal
elevation, and a high plasma concentration of norepinephrine during tachycardia. These
findings suggest that autonomic dysfunction should be examined in SMA type 1 patients
with long survival, although the pathogenesis remains to be clarified.
Keywords
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Article info
Publication history
Accepted:
February 21,
2005
Received in revised form:
February 3,
2005
Received:
November 22,
2004
Identification
Copyright
© 2005 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
