Autonomic Storm in a Patient With Amyotrophic Lateral Sclerosis in a Home-Care Setting.

Abstract

Amyotrophic lateral sclerosis (ALS) can cause autonomic storms characterized by blood pressure fluctuations, particularly in the advanced stages of the disease and among ventilator-dependent patients. Although benzodiazepines are used to manage these symptoms, the optimal agent is unclear. We present the case of a 78-year-old home-care patient with ventilator-dependent ALS who developed autonomic storms. Clotiazepam did not adequately control the episodes; however, diazepam was associated with resolution of the episodes, followed by improved sleep and nutritional intake. This report highlights the importance of recognizing an autonomic storm in advanced ALS patients and the potential benefit of diazepam in home-care settings.