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Autonomic dysfunction in presymptomatic spinocerebellar ataxia type-2


Julio Montes Brown, General Hospital Dr. Ernesto Guevara de la Serna, Ave 2 Diciembre No 1 Las Tunas, Las Tunas, Cuba CP 75100
Tel./fax: (531) 376313


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.