Journal
MUSCLE & NERVE
Volume 44, Issue 5, Pages 737-740Publisher
WILEY-BLACKWELL
DOI: 10.1002/mus.22159
Keywords
androgen receptors; autonomic dysfunction; autonomic function tests; Kennedy disease; plasma noradrenaline
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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. Muscle Nerve 44: 737-740, 2011
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