(632) Hot and Bothered: A Rare Case of Dyskinesia Hyperpyrexia Syndrome

Introduction: Dyskinesia hyperpyrexia syndrome (DHS) is a rare syndrome in patients with longstanding Parkinson's disease (PD) that presents a challenging diagnosis. There is symptomatic overlap with parkinsonian hyperpyrexia syndrome, neuroleptic malignant syndrome, and infection in PD patients, but pathology and management are distinct. DHS is a syndrome of excessive dopaminergic stimulation, characterized by continuous dyskinesia, rhabdomyolysis, hyperthermia, and associated end-organ dysfunction. Reported triggers include high ambient temperature, infection, trauma, and dopaminergic drug dosage increase. Treatment involves dopaminergic drug reduction and supportive care.

Description: A 53-year-old female with PD was initially admitted with hypercalcemia. After undergoing parathyroidectomy for parathyroid adenoma she gradually developed acute kidney injury (AKI) as well as agitation and hallucinations. One week after surgery she abruptly deteriorated, developing diffuse persistent dyskinetic movements, tachycardia, and fever. Labs showed hyperkalemia, hypocalcemia, worsening AKI, acidosis, creatinine kinase at 5559.
She was intubated and sedated for airway protection, but tremors and fevers persisted. She developed multi-organ failure and required dialysis briefly. Selegiline, Amantadine, and long-acting Carbidopa-levodopa were held for concern for DHS secondary to dopaminergic drug buildup in setting of renal failure. Dyskinesia and hyperthermia improved within days, and medications were titrated back on. Tracheostomy and PEG tube were placed. Her mental status has since improved.

Discussion: DHS is rare (13 cases reported as of 2022). Hallmarks of abnormal movements, rhabdomyolysis, and hyperthermia are seen in a variety of pathologies. Diagnosis relies on history (medications and inciting factors), and clinical nuance in presentation: dyskinesia/tremors vs tremor/rigidity. In this case, identification of continuous dyskinesia without rigidity in the setting of consistent dopaminergic drug administration and AKI (decreased drug clearance – particularly Amantadine) led to diagnosis and appropriate management of DHS. This case underscores the importance of accurate diagnosis and also signals the importance of monitoring kidney function and being mindful of medications in acutely ill PD patients.