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.