Program Director Harlem Hospital/Columbia University, United States
Introduction: Paroxysmal autonomic instability with dystonia (PAID) syndrome is a rare condition that presents in severe brain injuries caused by trauma or hypoxia. This syndrome is associated with damage to the axonal or brainstem, and is rarely seen in cases of intracranial hemorrhage, tumor, or hydrocephalus, primarily due to damage to the cortical and subcortical areas that control vegetative functions, leading to dysregulation of the autonomic nervous system.
Description: A 49-year-old female was admitted to the ICU following a cardiac arrest secondary to a massive pulmonary embolism, hypoxic hypercarbic respiratory failure and hypoxic-ischemic brain injury, acute tubular necrosis (ATN) necessitating dialysis. Post cardiac arrest, she exhibited high sedation requirements due to autonomic instability, making it challenging to wean off propofol, dexmedetomidine, and fentanyl. After tracheostomy and percutaneous endoscopic gastrostomy (PEG) tube placement, attempts to discontinue propofol and fentanyl resulted in severe hypertension (>180/100), tachycardia (>144), tachypnea (34-44), diaphoresis, hyperthermia (103.8), agitation, and dystonic movements. Initially it was suspected to be due to endotracheal tube (ETT) placement, sepsis, or electrolyte imbalances, all of which were ruled out. Her episodes resolved with fentanyl pushes, benzodiazepines, and propofol, consistent with PAID due to underlying hypoxic brain injury. She was started on clonidine , propranolol, and these were gradually titrated up to discontinue IV sedation. Bromocriptine for fever, along with Tylenol, external cooling as needed, Gabapentin, fentanyl patch, and as-needed morphine were prescribed. Eventually, with the titration of medication, she was able to be off propofol and dexmedetomidine on day 36 of admission. Once PAID was well controlled, she was transferred to rehab for rehabilitation.
Discussion: PAID syndrome is characterized by elevated temperature (>101.3°F), tachycardia (>130 beats/min), tachypnea (>40 breaths/min), agitation, diaphoresis, and dystonia (rigidity or decerebrate posture) with cyclic episodes occurring at least once a day for three days. Other potential causes such as sepsis, delirium tremens, meningitis, neuroleptic malignant syndrome, thyroid storm, and malignant hyperthermia must be ruled out.
