" Background and purpose: The aim of this study was to investigate the role of the autonomic nervous system in Amyotrophic Lateral Sclerosis using a multimodal approach. NMS were studied with the NMS assessment scale for Parkinson's disease and an autonomic subscale was calculated. Cardioautonomic innervation at rest and while standing was assessed by different parameters of heart rate variability. NMS in general were more frequent in Amyotrophic Lateral Sclerosis patients and correlated inversely with the Amyotrophic Lateral Sclerosis functional rating scale whereas the autonomic subscore of NMSScale did not differ between both groups and was not related to functional impairment. Cardioautonomic assessment solely revealed an increased heart rate at rest in Amyotrophic Lateral Sclerosis patients, whereas the other HRV parameters did not differ from controls. Using a multimodal approach the authors found evidence for a rather mild cardio-sympathetic overactivity in Amyotrophic Lateral Sclerosis patients. Overall, autonomic dysfunction seems to be subtle and is not related to the functional state of Amyotrophic Lateral Sclerosis patients.