Seronegative autoimmune limbic encephalitis presenting as super- refractory status epilepticus in Budd–Chiari syndrome: A Multi-hit perspective
Avinash Hannabe Rajanna, Venkatesh Venkatappa SrikrishnaNew-onset refractory status epilepticus (NORSE) and super-refractory status epilepticus (SRSE) are severe neurological emergencies. Their occurrence in patients with systemic thromboinflammatory conditions such as Budd–Chiari syndrome may suggest overlapping vascular, inflammatory, and autoimmune mechanisms. A 25-year-old female with Budd–Chiari syndrome (status post hepatic vein angioplasty and stenting) presented with new- onset generalized tonic–colonic seizures progressing to SRSE, with a Glasgow coma scale (GCS) of E2V1M4 (7/15) at presentation. Neuroimaging demonstrated bilateral mesial temporal lobe T2/FLAIR hyperintensities suggestive of limbic encephalitis, while extensive infectious, metabolic, and autoimmune evaluations were negative. High-dose intravenous methylprednisolone was initiated on day 12 of illness. Despite aggressive management, seizure control remained inadequate, and the patient ultimately developed hemodynamic instability leading to a fatal outcome. This case highlights the diagnostic challenges of NORSE/SRSE in patients with systemic prothrombotic disorders. After exclusion of alternative etiologies, a diagnosis compatible with probable seronegative autoimmune limbic encephalitis was considered. A speculative multi-hit thromboinflammatory hypothesis is proposed.