Autonomic dysfunction in presymptomatic spinocerebellar ataxia type-2

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.