Unmasking the Connection: Cardiac Arrest in Stiff Person Syndrome

Background : Cardiac arrest, characterized by the sudden loss of heart function, accounts for over 400,000 deaths annually in the United States, often resulting from unwitnessed ventricular arrhythmias. Stiff Person Syndrome (SPS), a rare neurological disorder, is marked by progressive muscle rigidity, painful spasms, which can occasionally lead to autonomic dysfunction and fulminant myocarditis. Case Presentation: We present a rare case of a male patient in his 30s with SPS who developed whole-body spasms, syncope, and respiratory distress. During extubation, he experienced cardiac arrest, requiring advanced cardiac life support (ACLS) and reintubation. Comprehensive evaluations including ECG, cardiac MRI, and laboratory testing suggested autonomic dysfunction and myocarditis as potential contributors to ventricular arrhythmias. Management involved sedation, diazepam, gabapentin, corticosteroids, and implantation of an implantable cardioverter-defibrillator (ICD) for non-sustained ventricular tachycardia. Conclusion : This case highlights the critical need for recognizing cardiac involvement in SPS and emphasizes early diagnosis and intervention to prevent fatal arrhythmias and improve patient outcomes.