Life-Threatening Bradycardia in Anti-NMDA-Receptor Encephalitis and a Novel Use for Permanent Pacing

Pediatric anti-NMDA receptor encephalitis (pNMDARE) is an autoantibody-mediated disorder that can cause severe autonomic dysfunction, including symptomatic bradycardia and asystole. Dysautonomia can last for years, making it very challenging to manage.

To describe outcomes of 5 pNMDARE patients with life-threatening bradycardic and/or asystolic events who were managed with permanent or semi-permanent pacemaker implantation.

We performed a retrospective chart review of 5 patients from multiple institutions. We included patients with a diagnosis of pNMDARE (confirmed by positive cerebrospinal fluid and/or serum anti-NMDAR antibodies) who had a permanent or semi-permanent pacemaker placed due to symptomatic bradycardia, sinus pauses, and/or asystole. Assessed outcomes included mortality, the presence of additional bradycardic/asystolic events after pacemaker implantation, pacemaker complications (lead/device infection, device malfunction), and the ongoing need for ventricular pacing.

Four patients had permanent pacing systems placed, and one patient had a semi-permanent pacemaker placed. Three patients required continued intermittent ventricular pacing months to years after disease onset. None of the patients had further episodes of symptomatic bradycardia or asystole after pacemaker implantation. One patient, who had a severe, intractable form of pNMDARE, died after discontinuing immunotherapies; she had multiple pacemaker interrogations that demonstrated no sign of pacemaker dysfunction.

Permanent and semi-permanent pacemakers are a safe, effective management strategy for cases of pNMDARE with prolonged courses of severe bradycardia and/or asystole.