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Unilateral cortical autoimmune encephalitis: A case series and comparison to late-onset Rasmussen's encephalitis
Damman, Sophia; Sukpornchairak, Persen; Ahituv, Amit; Chen, Alex; Wang, David; Sawlani, Komal; Steriade, Claude; Abboud, Hesham
OBJECTIVE:To report a novel anatomical pattern of autoimmune encephalitis characterized by strictly unilateral cortical inflammation and a clinical picture overlapping with late-onset Rasmussen's encephalitis. METHODS:We retrospectively gathered data of patients identified at two tertiary referral academic centers who met inclusion criteria. RESULTS:We identified twelve cases (average age 65, +/- 19.8 years, 58% female). All patients had unilateral cortical inflammation manifesting with focal seizures, cognitive decline, hemicortical deficits, and unilateral MRI and/or EEG changes. Six cases were idiopathic, two paraneoplastic, two iatrogenic (in the setting of immune checkpoint inhibitors), and two post-COVID-19. Serologically, ten patients were seronegative, one had high titer anti-GAD65, and one had anti-NMDAR. Five patients met Rasmussen's encephalitis criteria, and six did not fully meet the criteria but had symptoms significantly overlapping with the condition. Most patients had significant improvement with immunotherapy. DISCUSSION/CONCLUSIONS:Unilateral cortical AE seems to be more prevalent in the elderly and more frequently idiopathic and seronegative. Patients with this anatomical variant of autoimmune encephalitis have overlapping features with late-onset Rasmussen's encephalitis but are more responsive to immunotherapy. In cases refractory to immunotherapy, interventions used in refractory Rasmussen's encephalitis may be considered, such as functional hemispherectomy.
PMID: 38728930
ISSN: 1872-8421
CID: 5664792
Comparative analysis of patients with new onset refractory status epilepticus preceded by fever (febrile infection-related epilepsy syndrome) versus without prior fever: An interim analysis
Jimenez, Anthony D; Gopaul, Margaret; Asbell, Hannah; Aydemir, Seyhmus; Basha, Maysaa M; Batra, Ayush; Damien, Charlotte; Day, Gregory S; Eka, Onome; Eschbach, Krista; Fatima, Safoora; Fields, Madeline C; Foreman, Brandon; Gerard, Elizabeth E; Gofton, Teneille E; Haider, Hiba A; Hantus, Stephen T; Hocker, Sara; Jongeling, Amy; Kalkach Aparicio, Mariel; Kandula, Padmaja; Kang, Peter; Kazazian, Karnig; Kellogg, Marissa A; Kim, Minjee; Lee, Jong Woo; Marcuse, Lara V; McGraw, Christopher M; Mohamed, Wazim; Orozco, Janet; Pimentel, Cederic; Punia, Vineet; Ramirez, Alexandra M; Steriade, Claude; Struck, Aaron F; Taraschenko, Olga; Treister, Andrew K; Yoo, Ji Yeoun; Zafar, Sahar; Zhou, Daniel J; Zutshi, Deepti; Gaspard, Nicolas; Hirsch, Lawrence J; Hanin, Aurelie
Febrile infection-related epilepsy syndrome (FIRES) is a subset of new onset refractory status epilepticus (NORSE) that involves a febrile infection prior to the onset of the refractory status epilepticus. It is unclear whether FIRES and non-FIRES NORSE are distinct conditions. Here, we compare 34 patients with FIRES to 30 patients with non-FIRES NORSE for demographics, clinical features, neuroimaging, and outcomes. Because patients with FIRES were younger than patients with non-FIRES NORSE (median = 28 vs. 48 years old, p = .048) and more likely cryptogenic (odds ratio = 6.89), we next ran a regression analysis using age or etiology as a covariate. Respiratory and gastrointestinal prodromes occurred more frequently in FIRES patients, but no difference was found for non-infection-related prodromes. Status epilepticus subtype, cerebrospinal fluid (CSF) and magnetic resonance imaging findings, and outcomes were similar. However, FIRES cases were more frequently cryptogenic; had higher CSF interleukin 6, CSF macrophage inflammatory protein-1 alpha (MIP-1a), and serum chemokine ligand 2 (CCL2) levels; and received more antiseizure medications and immunotherapy. After controlling for age or etiology, no differences were observed in presenting symptoms and signs or inflammatory biomarkers, suggesting that FIRES and non-FIRES NORSE are very similar conditions.
PMID: 38625055
ISSN: 1528-1167
CID: 5668512
One for All-Can We Have and Do We Want a First-Line Monotherapy for Epilepsy? [Comment]
Steriade, Claude
PMID: 39280050
ISSN: 1535-7597
CID: 5719592
MRI Features and Their Association With Outcomes in Children With Anti-NMDA Receptor Encephalitis
Gombolay, Grace; Brenton, J Nicholas; Yang, Jennifer H; Stredny, Coral M; Kammeyer, Ryan; Otten, Catherine E; Vu, NgocHanh; Santoro, Jonathan D; Robles-Lopez, Karla; Christiana, Andrew; Steriade, Claude; Morris, Morgan; Gorman, Mark; Moodley, Manikum; Hardy, Duriel; Kornbluh, Alexandra B; Kahn, Ilana; Sepeta, Leigh N; Yeshokumar, Anusha; ,
OBJECTIVES:How brain MRI lesions associate with outcomes in pediatric anti-NMDA receptor encephalitis (pNMDARE) is unknown. In this study, we correlate T2-hyperintense MRI brain lesions with clinical outcomes in pNMDARE. METHODS:This was a multicenter retrospective cohort study from 11 institutions. Children younger than 18 years with pNMDARE were included. One-year outcomes were assessed by the modified Rankin Score (mRS) with good (mRS ≤2) and poor (mRS ≥3) outcomes. RESULTS:A total of 175 pNMDARE subjects were included, with 1-year mRS available in 142/175 (81%) and 60/175 (34%) had abnormal brain MRIs. The most common T2-hyperintense lesion locations were frontal, temporal, and parietal. MRI features that predicted poor 1-year outcomes included abnormal MRI, particularly T2 lesions in the frontal and occipital lobes. After adjusting for treatment within 4 weeks of onset, improvement within 4 weeks, and intensive care unit admission, MRI features were no longer associated with poor outcomes, but after multiple imputation for missing data, T2 frontal and occipital lesions associated with poor outcomes. DISCUSSION:Abnormal frontal and occipital lesions on MRI may associate with 1-year mRS in pNMDARE. MRI of the brain may be a helpful prognostication tool that should be examined in future studies.
PMCID:10219134
PMID: 37236807
ISSN: 2332-7812
CID: 5757642
Predictors of seizure outcomes of autoimmune encephalitis: A clinical and morphometric quantitative analysis study
Steriade, Claude; Patel, Palak; Haynes, Jennifer; Desai, Ninad; Daoud, Nader; Yuan, Heidi; Borges, Helen; Pardoe, Heath
OBJECTIVE:Autoimmune encephalitis can be followed by treatment-resistant epilepsy. Understanding its predictors and mechanisms are crucial to future studies to improve autoimmune encephalitis outcomes. Our objective was to determine the clinical and imaging predictors of postencephalitic treatment-resistant epilepsy. METHODS:We performed a retrospective cohort study (2012-2017) of adults with autoimmune encephalitis, both antibody positive and seronegative but clinically definite or probable. We examined clinical and imaging (as defined by morphometric analysis) predictors of seizure freedom at long term follow-up. RESULTS:Of 37 subjects with adequate follow-up data (mean 4.3 yrs, SD 2.5), 21 (57 %) achieved seizure freedom after a mean time of 1 year (SD 2.3), and one third (13/37, 35 %) discontinued ASMs. Presence of mesial temporal hyperintensities on the initial MRI was the only independent predictor of ongoing seizures at last follow-up (OR 27.3, 95 %CI 2.48-299.5). Morphometric analysis of follow-up MRI scans (n = 20) did not reveal any statistically significant differences in hippocampal, opercular, and total brain volumes between patients with postencephalitic treatment-resistant epilepsy and those without. SIGNIFICANCE/CONCLUSIONS:Postencephalitic treatment-resistant epilepsy is a common complication of autoimmune encephalitis and is more likely to occur in those with mesial temporal hyperintensities on acute MRI. Volume loss in the hippocampal, opercular, and overall brain on follow-up MRI does not predict postencephalitic treatment-resistant epilepsy, so additional factors beyond structural changes may account for its development.
PMID: 37393702
ISSN: 1872-6968
CID: 5538892
Tackling the Unmet Therapeutic Needs in Nonsurgical Treatments for Epilepsy
Steriade, Claude; French, Jacqueline
PMID: 36066899
ISSN: 2168-6157
CID: 5332412
Aggregation-Seeding Forms of α-Synuclein Are Not Detected in Acute Coronavirus Disease 2019 Cerebrospinal Fluid [Letter]
Russo, Marco J; MacLeod, Karen; Lamoureux, Jennifer; Lebovitz, Russ; Pleshkevich, Maria; Steriade, Claude; Wisniewski, Thomas; Frontera, Jennifer A; Kang, Un Jung
PMID: 36208476
ISSN: 1531-8257
CID: 5351812
Fulminant Idiopathic Intracranial Hypertension in Pregnancy [Case Report]
Tyndel, Felix; Steriade, Claude; Gallo, Antonio; Wennberg, Richard; Radovanovic, Ivan
Fulminant IIH in pregnancy requires multidisciplinary collaboration and immediate CSF diversion.
PMCID:9210042
PMID: 35815103
ISSN: 1662-680x
CID: 5279792
Proposal for an updated seizure classification framework in clinical trials
Steriade, Claude; Sperling, Michael R; DiVentura, Bree; Lozano, Meryl; Shellhaas, Renée A; Kessler, Sudha Kilaru; Dlugos, Dennis; French, Jacqueline
The International League Against Epilepsy (ILAE) seizure classification scheme has been periodically updated to improve its reliability and applicability to clinicians and researchers alike. Here, members of the Epilepsy Study Consortium propose a pragmatic seizure classification, based on the ILAE scheme, designed for use in clinical trials with a focus on outcome measures that have high reliability, broad interpretability across stakeholders, and clinical relevance in the context of the development of novel antiseizure medications. Controversies around the current ILAE classification scheme are discussed in the context of clinical trials, and pragmatic simplifications to the existing scheme are proposed, for intended use by investigators, industry sponsors, and regulatory agencies.
PMID: 34997581
ISSN: 1528-1167
CID: 5136902
Discerning the Role of Autoimmunity and Autoantibodies in Epilepsy: A Review
Steriade, Claude; Gillinder, Lisa; Rickett, Kirsty; Hartel, Gunter; Higdon, Lindsay; Britton, Jeffrey; French, Jacqueline
Importance/UNASSIGNED:The literature on neural autoantibody positivity in epilepsy has expanded over the last decade, with an increased interest among clinicians in identifying potentially treatable causes of otherwise refractory seizures. Observations/UNASSIGNED:Prior studies have reported a wide range of neural autoantibody positivity rates among various epilepsy populations, with the highest frequency reported in individuals with focal epilepsy of unknown cause and new-onset seizures. The antibodies in some cases are of uncertain significance, and their presence can cause conundrums regarding therapy. Conclusions and Relevance/UNASSIGNED:There is likely some role for neural autoantibody assessment in patients with unexplained epilepsy who lack clear evidence of autoimmune encephalitis, but the clinical implications of such testing remain unclear owing to limitations in previous published studies. A framework for study design to bridge the current gaps in knowledge on autoimmune-associated epilepsy is proposed.
PMID: 34515743
ISSN: 2168-6157
CID: 5012212