Abstract
The purpose of this study was to quantitatively assess cardiovagal, adrenergic and
postganglionic sudomotor functions in a patient with chorea-acanthocytosis. We describe
a 25-year-old woman with acanthocytosis, lip and tongue biting, chorea like movements
and increased levels of serum creatine phosphokinase. The patient presented with orthostatic
hypotension. The heart period response to deep breathing was moderately decreased
and the quantitative sudomotor axon reflex test (QSART) was mildly abnormal in the
lower extremities. The composite autonomic score (CASS) for this patient was 6 points
which indicates moderate autonomic failure. Although a few previous reports have described
autonomic dysfunction associated with chorea-acanthocytosis and have indicated that
impairment is primarily sympathetic, our case study suggests that there is also parasympathetic
dysfunction. We conclude that there is widespread autonomic involvement in chorea-acanthocytosis
and that autonomic studies are useful in monitoring their course.
Keywords
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Article info
Publication history
Accepted:
May 11,
2001
Received in revised form:
May 11,
2001
Received:
February 26,
2001
Identification
Copyright
© 2002 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
