Antibody-Negative but Not Immune-Negative: Seronegative Autoimmune Encephalitis Presenting as NORSE
Authors
Shamisha Khade, Vibhor Pardasani, Sunetra Phulsunge, Rakesh SinghFiles
Abstract
Abstract
Background: New-onset refractory status epilepticus (NORSE) is a rare neurological emergency characterized by prolonged seizures without prior epilepsy or clear acute structural cause. Autoimmune encephalitis is increasingly recognized as an important etiology.
Case Presentation: We report a 28-year-old previously healthy male who presented with acute febrile illness followed by focal seizures rapidly progressing to refractory status epilepticus. Neuroimaging revealed bilateral basal ganglia and mesial temporal involvement. Extensive infectious and autoimmune workup was negative. Despite escalation of antiseizure medications and anaesthetic agents, seizures remained refractory, necessitating immunotherapy with intravenous immunoglobulin and rituximab, following which gradual neurological recovery was observed.
Conclusion: This case highlights the importance of early recognition of NORSE and prompt initiation of immunomodulatory therapy in suspected autoimmune encephalitis, even in seronegative cases.
References
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