Seronegative autoimmune limbic encephalitis presenting as super- refractory status epilepticus in Budd–Chiari syndrome: A Multi-hit perspective

New-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.

Authors

Institutions

Publication Details

Journal
Journal of Neurosciences in Rural Practice
Published
2026-10-28
DOI
https://doi.org/10.25259/jnrp_101_2026
Primary Topic
Autoimmune Neurological Disorders and Treatments
Type
article
Field-Weighted Citation Impact
0.00
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Seronegative autoimmune limbic encephalitis presenting as super- refractory status epilepticus in Budd–Chiari syndrome: A Multi-hit perspective

Venkatesh Venkatappa Srikrishna, Avinash Hannabe Rajanna
Journal of Neurosciences in Rural Practice
Autoimmune Neurological Disorders and Treatments
article

Seronegative autoimmune limbic encephalitis presenting as super- refractory status epilepticus in Budd–Chiari syndrome: A Multi-hit perspective

Venkatesh Venkatappa Srikrishna, Avinash Hannabe Rajanna
article en

Abstract

New-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.

Journal of Neurosciences in Rural PracticeVol. 0
Bangalore Medical College and Research Institute (IN)
Openalex Percentile: Top 13%
Autoimmune Neurological Disorders and Treatments
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.

Laika access coming soon