Format

Send to

Choose Destination
Muscle Nerve. 2011 Nov;44(5):737-40. doi: 10.1002/mus.22159.

Subclinical autonomic dysfunction in spinobulbar muscular atrophy (Kennedy disease).

Author information

1
Department of Neuroscience, University of Rome Tor Vergata, 00133 Rome, Italy. camillarocchi@gmail.com

Abstract

INTRODUCTION:

Spinobulbar muscular atrophy (SBMA) is an inherited adult-onset motor neuron disease caused by the expansion of a polyglutamine tract within the androgen receptor. Autonomic nervous system involvement (ANS) is not considered part of SBMA. The aim of this study was to assess autonomic cardiovascular function in 5 SBMA patients.

METHODS:

Five quantitative autonomic function tests (AFTs) were performed in 5 SBMA patients. Plasma noradrenaline (NA) concentration in patients and in 5 healthy subjects was also measured.

RESULTS:

AFTs were abnormal in 4 of the 5 patients, and plasma NA concentration was significantly reduced in patients with respect to controls.

CONCLUSION:

The impairment of cardiovascular responses to AFTs in addition to reduced plasma NA concentration observed in our patients suggests subclinical involvement of the ANS in Kennedy disease.

PMID:
22006688
DOI:
10.1002/mus.22159
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Wiley
Loading ...
Support Center