Purpose Of Review: This article focuses on the seizure manifestations and presentations of autoimmune-associated epilepsy and acute symptomatic seizures in autoimmune encephalitis. It discusses the specificity of the various central nervous system autoantibodies and clarifies when their presence can be considered indicative of an immune etiology. Finally, current recommendations regarding patient selection for autoimmune antibody evaluation are reviewed, and an approach to immunotherapy is provided.
Recent Findings: Although autoimmune seizures are caused by a heterogeneous group of autoantibodies, key features reported in the literature should alert clinicians to the possible diagnosis. In particular, seizure characteristics including frequency, timing, duration, and symptomatology can provide vital clues to help differentiate autoimmune-associated seizures from other causes of epilepsy. Diagnostic certainty also requires an understanding and integration of the spectrum of clinical and paraclinical presentations, and several scoring systems have been developed that may be useful to aid the identification of autoimmune seizures.
Summary: Seizures due to autoimmune etiology are increasingly encountered in clinical practice. It is critical that clinicians recognize immune seizure etiologies early in their course given they are often responsive to immunotherapy but are usually resistant to antiseizure medications. Currently, however, it is unfortunately not uncommon for autoimmune-associated seizure disorders to remain undiagnosed, resulting in missed opportunities to administer effective therapies. Efforts to better understand autoimmune seizure manifestations and treatment strategies are ongoing.
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http://dx.doi.org/10.1212/CON.0000000000001079 | DOI Listing |
Epilepsy Behav
December 2024
Royal Brisbane and Women's Hospital, Brisbane, Australia.
Objective: Anti-GAD65 antibodies have been identified in people with epilepsy for many years, but their pathophysiological relevance is still debated. Susceptibility to autoimmune disease has been associated with Human Leukocyte Antigen (HLA) subtypes. Therefore, this study aimed to determine if there are common HLA types in anti-GAD65 antibody associated epilepsy cases compared to epilepsy controls.
View Article and Find Full Text PDFEpilepsy Curr
April 2024
Neurology, Cleveland Clinic Children's Hospital, Cleveland, OH, USA.
Epilepsy Curr
April 2024
Department of Pharmacology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.
Roughly 80% of the global burden of epilepsy resides in low- and middle-income countries (LMICs; WHO, 2022). Despite numerous new therapies for the treatment of epilepsy, the number of patients who remain resistant to available medications is unchanged. Additionally, no therapy has yet been clinically proven to prevent or attenuate the development of epilepsy in at-risk individuals.
View Article and Find Full Text PDFJ Transl Med
September 2024
Department of Neurology, Affiliated Hospital of Xuzhou Medical University, 99 West Huaihai Road, Xuzhou, Jiangsu, 221002, China.
medRxiv
September 2024
The University of Texas at Austin, Department of Neurology.
Background And Objectives: Autoimmune-associated epilepsy (AAE), a condition which responds favorably to immune therapies but not traditional anti-seizure interventions, is emerging as a significant contributor to cases of drug-resistant epilepsy. Current standards for the diagnosis of AAE rely on screening for known neuronal autoantibodies in patient serum or cerebrospinal fluid. However, this diagnostic method fails to capture a subset of drug-resistant epilepsy patients with suspected AAE who respond to immunotherapy yet remain seronegative (snAAE) for known autoantibodies.
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