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Clinical Autonomic Research
Published in: Clinical Autonomic Research 3/2018

Open Access 01-06-2018 | Short Communication

Autonomic function testing in spinocerebellar ataxia type 2

Authors: Elisabetta Indelicato, Alessandra Fanciulli, Jean Pierre Ndayisaba, Wolfgang Nachbauer, Roberta Granata, Julia Wanschitz, Michaela Wagner, Elke R. Gizewski, Werner Poewe, Gregor K. Wenning, Sylvia Boesch

Published in: Clinical Autonomic Research | Issue 3/2018

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Abstract

Purpose

To assess whether autonomic failure belongs to the clinical spectrum of spinocerebellar ataxia type 2 (SCA2), an autosomal dominant genetic disorder showing progressive cerebellar and brainstem dysfunction.

Methods

We evaluated cardiovascular autonomic function in 8 patients with SCA2 and 16 age- and gender-matched healthy controls. Other autonomic domains were examined through standardized questionnaires and by testing the skin sympathetic reflex.

Results

Patients with SCA2 showed normal responses to cardiovascular autonomic function tests, with the exception of lower baroreflex sensitivity upon standing compared to controls. In questionnaires, 7 out of 8 patients reported bladder disturbances, while 3 out of 6 tested patients had no skin sympathetic reflex.

Conclusions

We did not observe clinically overt cardiovascular autonomic failure in patients with SCA2. Other autonomic domains (i.e., bladder and sudomotor function) may be affected in the disease.
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Metadata
Title
Autonomic function testing in spinocerebellar ataxia type 2
Authors
Elisabetta Indelicato
Alessandra Fanciulli
Jean Pierre Ndayisaba
Wolfgang Nachbauer
Roberta Granata
Julia Wanschitz
Michaela Wagner
Elke R. Gizewski
Werner Poewe
Gregor K. Wenning
Sylvia Boesch
Publication date
01-06-2018
Publisher
Springer Berlin Heidelberg
Published in
Clinical Autonomic Research / Issue 3/2018
Print ISSN: 0959-9851
Electronic ISSN: 1619-1560
DOI
https://doi.org/10.1007/s10286-018-0504-4

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