title: Refractory Case of Paroxysmal Autonomic Instability With Dystonia Syndrome Secondary to Hypoxia.
abstract: Paroxysmal autonomic instability with dystonia (PAID) is a syndrome commonly related to traumatic brain injury (TBI) and rarely to anoxia associated with symptoms of dystonia, tachycardia, tachypnea, and diaphoresis. This is a case of a 20-year-old man who was stabbed in the heart. He underwent surgical repair of a ventricular septal defect and mitral valve replacement. Postoperatively, he developed dystonia with tachycardia and tachypnea consistent with PAID syndrome, secondary to prolonged hypoxia. Traditionally, this poorly understood syndrome is treated with morphine, clonazepam, and nonselective β-blockers. Second-line medications commonly used are baclofen, dantrolene, and gabapentin, which are aimed at the dystonia itself. In this case, both first- and second-line agents were ineffective. A 72-hour dexmedetomidine infusion resulted in complete resolution of symptoms. This is the first case of anoxia-induced PAID syndrome to be effectively treated with dexmedetomidine, which was previously used in a case induced by TBI.
authors: Department of Internal Medicine, Rutgers University, New Jersey Medical School, Newark, NJ. Electronic address: johnkerndo@gmail.com.
authors: John
authors: J
authors: Kern
authors: Department of Internal Medicine, Rutgers University, New Jersey Medical School, Newark, NJ.
authors: Daniel
authors: D
authors: Bodek
authors: Department of Internal Medicine, Rutgers University, New Jersey Medical School, Newark, NJ.
authors: Osama Tariq
authors: OT
authors: Niazi
authors: Department of Internal Medicine, Rutgers University, New Jersey Medical School, Newark, NJ.
authors: James
authors: J
authors: Maher
mesh_terms: D058647:Adrenergic alpha-2 Receptor Agonists / Q000008:administration & dosage; D001341:Autonomic Nervous System / Q000503:physiopathology*; D001342:Autonomic Nervous System Diseases / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D006348:Cardiac Surgical Procedures / Q000009:adverse effects*; D020927:Dexmedetomidine / Q000008:administration & dosage; D004421:Dystonia / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D004562:Electrocardiography; D006801:Humans; D000860:Hypoxia / Q000150:complications* / Q000503:physiopathology; D007262:Infusions, Intravenous; D008297:Male; D011183:Postoperative Complications; D013577:Syndrome; D055815:Young Adult
chemical_list: D058647:Adrenergic alpha-2 Receptor Agonists; D020927:Dexmedetomidine
keywords: autonomic function; cardiac arrest; neurology; trauma