Autoregressive spectral analysis of heart rate variability (HRV) was performed in 29 patients with amyotrophic lateral sclerosis (ALS) and 33 age-matched healthy subjects to evaluate the involvement of the autonomic nervous system. HRV analysis provides a means to recognize low (LF) and high (HF) frequency components, respectively mediated by sympathetic and parasympathetic heart control. An increase in the mean heart rate at rest (P < 0.001), a decrease in standard deviation of R-R interval as well as in PNN50 (P < 0.001), and an increase in the LF/HF component ratio (P < 0.01) were found in the ALS patients, indicating a vagal-sympathetic imbalance. These alterations were not related to the clinical features and to the duration of the disease. Our results suggest a subclinical involvement of the autonomic nervous system in ALS, particularly affecting parasympathetic cardiovascular control.