Paroxysmal Autonomic Instability with Dystonia, a Rare Syndrome and a Rehabilitation Emergency - A Case Report

Abstract

Introduction: Paroxysmal autonomic instability with dystonia is a relatively rare complication of multiple central nervous system (CNS) lesions. It remains poorly understood, despite its unfavorable clinical picture. It is associated with significant morbidity and mortality, increased length of stay in rehabilitation centers and unfavorable functional outcome. As it is difficult to differentiate from other conditions that also put patient`s lives at risk it is necessary for doctors to be aware for this syndrome. Persistent autonomic dysfunction may result in secondary brain injury and may be fatal.

Case Report: Five years old, male, victim of prolonged cardiac arrest due to near drowning, resulting in severe anoxic encephalopathy with neuromotor and cognitive-perceptual sequelae. Admitted in a rehabilitation center a month later, in a minimally consciousness state, he started several episodes of dystonia in descerebrate posture associated with tachycardia, diaphoresis, and persistent crying, triggered by different stimuli. This syndrome was a real clinical challenge, interfering with rehabilitation.

Discussion: This rare entity remains seldom considered in clinical practice and is associated with significant morbidity and mortality, extensive use of diagnostic tests and inadequate therapy in the rehabilitation context of these patients. The prompt recognition and treatment are crucial to prevent secondary brain injury.