Abstract Background Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy targeting aquaporin-4 (AQP4) water channels. While optic neuritis and longitudinally extensive transverse myelitis are classical, hypothalamic and brainstem lesions can trigger severe autonomic dysfunction. Cardiac arrhythmias and arrest are exceedingly rare but life-threatening manifestations. Case A 74-year-old woman with a history of coiled posterior cerebral artery aneurysm, cerebrovascular disease, rectal cancer (status post resection/chemoradiation with ileostomy), discoid lupus, chronic bicytopenia, and heart failure with preserved ejection fraction presented with altered mentation, weakness, and recurrent falls after multiple prior hospitalizations for similar episodes. On arrival, she was hypothermic (33 °C), bradycardic (HR 40 bpm), and hypotensive (BP 94/65 mmHg), prompting empiric management for sepsis.MRI brain revealed progressive T2/FLAIR hyperintensities in the bilateral globus pallidus and thalamus with stable pontine changes. Serum AQP4-IgG titer exceeded 10,000, confirming NMOSD; ANA, RNP, and SSA were positive. MRI spine was negative for demyelination. She received IV methylprednisolone (5 days) followed by 7 sessions of plasmapheresis. Despite transient improvement, she developed recurrent bradycardia, hypotension, and gastrointestinal dysmotility consistent with global dysautonomia. Echocardiogram showed EF 60% with mild-to-moderate aortic insufficiency and mid-LV cavity obstruction.Serial EKGs demonstrated progressive PR-interval prolongation (254 → 332 ms), evolving from sinus bradycardia with first-degree AV block to junctional bradycardia and complete heart block. Electrophysiology consultation and pacing were deferred. On hospital day 28, she was found unresponsive with glucose 54 mg/dL. Following dextrose administration, she developed apnea, severe bradycardia, and pulseless arrest. Return of spontaneous circulation was achieved after four CPR cycles, but post-arrest imaging revealed anoxic brain injury. Telemetry showed ectopic atrial and junctional escape rhythms, Mobitz I, and third-degree AV block. She was transitioned to comfort care and expired. Discussion Only three cases of sinus arrest in NMOSD have been reported—all with medullary lesions and recovery after corticosteroids (Ryouhei 2020; Endo 2020; Lin 2023). Our patient uniquely demonstrated fatal autonomic failure without medullary involvement, highlighting systemic rather than focal dysautonomia. Progressive conduction disease despite immunotherapy underscores a possible immune-mediated nodal dysfunction and supports earlier pacing consideration. Conclusion NMOSD can precipitate catastrophic autonomic dysregulation and cardiac arrest even in the absence of medullary lesions. Early recognition of dysautonomia and proactive electrophysiologic evaluation may be lifesaving. Multidisciplinary awareness is essential to prevent this under-recognized “fatal face” of NMOSD. This abstract is funded by: None
Amal et al. (Fri,) studied this question.