Paroxysmal Autonomic Instability with Dystonia after Severe Traumatic Brain Injury

Background: Paroxysmal autonomic instability with dystonia (PAID) syndrome, a subset of dysautonomia, is characterized by paroxysms of marked agitation, diaphoresis, hyperthermia, hypertension, tachycardia and tachypnea accompanied by hypertonia and extensor posturing.

Case Report: We report a 52-year-old man who was severely brain injured and developed spastic tetraparesis with cognitive impairment. During his intensive care unit stay and rehabilitation period, he presented with paroxysmal episodes of dystonic posturing accompanied by dysautonomia.

Discussion: Our case raises awareness of PAID, a life-threatening condition which can mimic many others and poses significant challenges in the acute management and rehabilitation of patients.

Highlights:

• PAID is characterized by paroxysms of marked agitation, diaphoresis, hyperthermia, hypertension, tachycardia and tachypnea accompanied by hypertonia and extensor posturing.
• It usually presents in patients with severe brain injury primarily due to trauma or hypoxia resulting in diffuse axonal or brainstem injury.
• PAID is also associated with tuberculous meningitis, interpeduncular tuberculoma, pneumococcal meningoencephalitis, intracerebral hemorrhage and paraneoplastic limbic encephalopathy.
• Differential diagnosis of PAID include neuroleptic malignant syndrome, malignant hyperthermia, sepsis, thyroid storm, pheochromocytoma, autonomic epileptic seizures, sepsis and impending cerebral herniation.