Autonomic dysfunction in presymptomatic spinocerebellar ataxia type-2.

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.

Heart rate variability in type 2 spinocerebellar ataxia.

OBJECTIVES: To explore cardiovascular autonomic regulation in Spinocerebellar ataxia type 2 (SCA2) patients, using heart rate variability (HRV) analysis and neurophysiologic autonomic reflex tests, and determine relations and causal related factors of dysautonomia in SCA2. MATERIALS AND METHODS: Heart rate variability indices for 5 min series of RR intervals were analyzed in 97 SCA2 patients, assessed quantitatively for somatic and autonomic nervous system complaints applying the International Cooperative Ataxia Rating Scale and Scales for Outcomes in Parkinson's disease (SCOPA-AUT), respectively. Autonomic testing included: resting control, standing, Valsalva maneuver and deep breathing. RESULTS: Mean RR, long- and short-term variability indices and spectral density power (LF, HF) indices were lower in the patients group, whereas LF/HF ratio and LF (nu) were higher. Highly differences between groups were observed for seven diagnostic autonomic test indices. Significant correlations were found between different clinical and demographic indices and between clinical indices and some HRV indices. CONCLUSIONS: We confirm the presence of cardiovascular autonomic dysfunction in a large group of SCA2 patients.