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Semin Neurol 2025; 45(02): 275-286
DOI: 10.1055/a-2525-3511
Review Article

Seizures Associated with Autoimmune Disorders — Current Treatment Approaches

Lisa Gillinder 
1   Princess Alexandra Hospital, Woolloongabba Qld, Australia
2   School of Medicine, the University of Queensland, Brisbane, Australia
,
Jeffrey W. Britton
3   Epilepsy Division, Department of Neurology, Mayo Clinic, Rochester, Minnesota
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Abstract

Autoimmune-associated seizures and epilepsy are increasingly recognized in clinical practice and can arise in the setting of acute encephalitis but in some cases may present with chronic focal epilepsy. These conditions are usually resistant to antiseizure therapy but may respond definitively to timely immunotherapy. Early diagnosis and treatment are critical to minimize neural injury and optimize outcomes. Treatment is guided by consensus opinion because definitive trials are currently lacking. The initial management approach usually involves first-line agents such as corticosteroids, intravenous immunoglobulin (IVIg), or plasma exchange, with second-line agents like rituximab or cyclophosphamide. Maintenance therapy is considered to prevent relapses, which occur in up to 35% of patients. Relapse management requires careful differentiation from postencephalitic epilepsy, which in the absence of active inflammation does not respond to immunotherapy. This review discusses treatment strategies for autoimmune-associated seizure disorders, including acute symptomatic seizures and epilepsy. We discuss expected outcomes on the basis of the underlying pathogenesis including cases mediated by autoantibodies targeting specific neuronal surface/synaptic antigens, and intracellular epitopes, and for cases lacking defined biomarkers. Specific approaches are outlined for disorders such as anti-LGI1, anti-NMDAR, anti-GABA-BR, and anti-GAD65 encephalitides, emphasizing tailored immunotherapy based on pathophysiology and clinical context.

Keywords

acute symptomatic seizures - autoimmune-associated epilepsy - autoimmune encephalitis - treatment - anti-glutamic acid decarboxylase 65 (GAD65) - anti-LGI1 - anti-NMDAR - anti-GABA-BR - seronegative autoimmune limbic encephalitis - paraneoplastic

Note

J.W.B.: Inventor agreement—Seer Medical—no royalties received to date.




Publication History

Article published online:
12 March 2025

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