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Purpose: 1) To determine the clinical expression and consequences of autonomic dysregulation in patients with diffuse axonal injury (DAI), and 2) to study the use of the "paroxysmal sympathetic hyperactivity assessment measure" (PSH-AM)., Methods: Patients clinically diagnosed with autonomic dysregulation were selected from a cohort involving 116 patients with DAI. We studied the incidence of autonomic features, treatment, and outcome. In addition a systematic review was performed., Results: Autonomic dysregulation was diagnosed in 19 of 116 (16.4%). Lower age (OR 0.95) and higher DAI grade (OR 7.2) were risk factors for autonomic dysregulation. Autonomic dysregulation was associated with an unfavourable outcome (OR 5.6) and a longer ICU and hospital stay. On the PSH-AM 57.9% (n = 11) scored a probable paroxysmal sympathetic hyperactivity (PSH), 36.8% (n = 7) scored possible, and 5.2% (n = 1) scored unlikely. The review yielded 30 articles. The incidence of autonomic dysregulation after TBI varied from 7.7-32.6% (mean 13.5%). TBI patients with autonomic dysregulation had a longer ICU stay and poorer outcome., Conclusion: Patients with DAI and autonomic dysregulation had a longer ICU stay and a poorer outcome compared to patients without autonomic dysregulation. The PSH-AM is a potential valuable tool to determine the likelihood of autonomic dysregulation., (Copyright © 2018 Elsevier Inc. All rights reserved.)