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A 70-year-old woman presented with aphasia caused by acute infarction in the left middle cerebral artery. Cardiac investigation revealed progressively increasing mobile mass in the left atrial appendage over 2 months (from 9 to 15 mm). Decision was made to proceed with mass resection, and pathological evaluation confirmed Masson tumor. (Level of Difficulty: Advanced.), Graphical abstract

Background: We report a young woman who presented with acute psychosis and autonomic dysfunction who was diagnosed with Anti-N-Methyl-D-Aspartate Receptor Encephalitis (anti-NMDARE). Her life-threatening autonomic instability was controlled only after the placement of a permanent cardiac pacemaker. Case Presentation: A 29-year-old woman presented with abnormal movements and depersonalization. Vital signs showed BP 132/94 mmHg, HR 110 beats/min, RR 26 cycles/min, temperature of 101 F and spO2 of 80 % on RA. She was emergently intubated. Laboratory evaluation showed elevated CRP 6.98 mg/dL and CK 3329 U/L. Brain MRI revealed an abnormality in the right amygdala and hippocampus. Infectious workup was negative; however, a high titer of CSF NMDAR antibody was present. CT abdomen and pelvis revealed a 1.1 cm cyst within the left ovary. With a diagnosis of anti-NMDARE, she was treated with plasmapheresis, methylprednisolone, and immunoglobulin G therapy and underwent bilateral salpingo-oophorectomy with pathology confirming a teratoma. Despite these treatments, she continued to display autonomic dysfunction. Telemetry showed tachycardia-bradycardia with asystole that remained refractory to treatment and required placement of a permanent pacemaker (PPM). She showed clinical improvement after PPM and treatment ( Figure ). Conclusion: This is a rare case of severe bradyarrhythmia in anti NMDARE that necessitated placement of a permanent cardiac pacemaker. Neural-mapping studies show that the amygdala and hippocampus strongly modulate autonomic control. Disruption of cortical circuitry related to autonomic tone likely lead to arrhythmias in our case. Class I indications for pacemakers in the ICU include asystole, symptomatic bradycardia with hypotension and/or unresponsive to atropine. Early recognition, cardiorespiratory support, multistep pharmacological care and sometimes early electrophysiological support in anti-NMDARE are paramount.