Autonomic dysfunction in presymptomatic spinocerebellar ataxia type-2
Article first published online: 25 FEB 2011
© 2011 John Wiley & Sons A/S
Acta Neurologica Scandinavica
Volume 125, Issue 1, pages 24–29, January 2012
How to Cite
Montes-Brown, J., Machado, A., Estévez, M., Carricarte, C. and Velázquez-Pérez, L. (2012), Autonomic dysfunction in presymptomatic spinocerebellar ataxia type-2. Acta Neurologica Scandinavica, 125: 24–29. doi: 10.1111/j.1600-0404.2011.01494.x
- Issue published online: 21 DEC 2011
- Article first published online: 25 FEB 2011
- Accepted for publication January 7, 2011
- autonomic nervous system diseases;
- heart rate;
- heart rate variability;
- spectral analysis;
- spinocerebellar ataxias;
- trinucleotide repeats
Montes-Brown J, Machado A, Estévez M, Carricarte C, Velázquez-Pérez L. Autonomic dysfunction in presymptomatic spinocerebellar ataxia type-2. Acta Neurol Scand: 2012: 125: 24–29. © 2011 John Wiley & Sons A/S.
Objectives – To explore and quantify possible abnormalities in the autonomic cardiovascular regulation in presymptomatic stage of type 2 spinocerebellar ataxia (PS-SCA2).
Materials & methods – Heart rate variability (HRV) for 5-min series of RR intervals was analyzed in 48 PS-SCA2. Autonomic testing included resting recording, standing, Valsalva maneuver, and deep breathing. The results were compared with a group of sex- and age-matched controls.
Results – Time-and-frequency domain HRV indices were significantly different between PS-SCA2 and control groups. Using two standard diagnostic procedures were identified 4 (8.33%) subjects with severe and 8 (16.66%) subjects with early cardiac autonomic neuropathy in PS-SCA2. CAG index significantly correlated with age (−0.35) and HR (0.31).
Conclusions – Our results confirm the presence of cardiovascular autonomic dysfunction in PS-SCA2 subjects.