Cardiac neural regulation involvement in patients with amyotrophic lateral sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease characterized by motor and extra-motor neuron loss. Aim of this study was to attempt a better characterization of the cardiac neural regulation in ALS using the heart rate variability analysis. Cardiac neural regulation was altered in all patients, i.e. they showed a low RR variance and an abnormal response to orthostasis. Interestingly, at rest patients could be divided in those with lower and those with higher sympathetic activity than normal. In both cases the response to orthostasis was blunted. These findings suggest that an impairment of the autonomic nervous system is present in ALS with different patterns of cardiac regulation imbalance among patients. It has to be established if such differences depend upon time course of the disease, different clinical entities, or other anatomic, pathological and clinical variables.