Clinical and electrographic features of persistent seizures and status epilepticus associated with anti‐NMDA receptor encephalitis (anti‐NMDARE)

• Published in: Epileptic disorders 2020-12, Vol.22 (6), p.739-751
• Main Authors: Gofshteyn, Jacqueline S., Yeshokumar, Anusha K., Jette, Nathalie, Thakur, Kiran T., Luche, Nicole, Yozawitz, Elissa, Varnado, Shelley, Klenofsky, Britany, Tuohy, Mary Claire, Ankam, Jyoti, Torres, Sarah, Hesdorffer, Dale, Nelson, Aaron, Wolf, Steven, McGoldrick, Patricia, Yan, Helena, Basma, Natasha, Grinspan, Zachary
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Age Factors; Anti-N-Methyl-D-Aspartate Receptor Encephalitis - complications; Anti-N-Methyl-D-Aspartate Receptor Encephalitis - physiopathology; Anticonvulsants - administration & dosage; anti‐NMDA receptor encephalitis; autoimmune encephalitis; Child; Child, Preschool; Convulsions & seizures; Databases, Factual; Drug Resistant Epilepsy - drug therapy; Drug Resistant Epilepsy - etiology; Drug Resistant Epilepsy - physiopathology; EEG; Electroencephalography; Encephalitis; Epilepsies, Partial - drug therapy; Epilepsies, Partial - etiology; Epilepsies, Partial - physiopathology; Epilepsy; Epilepsy - drug therapy; Epilepsy - etiology; Epilepsy - physiopathology; Frontal lobe; Frontal Lobe - physiopathology; Glutamate receptors; Glutamic acid receptors (ionotropic); Humans; N-Methyl-D-aspartic acid receptors; Phenotypes; Retrospective Studies; seizure risk factors; Seizures; Sleep; Status Epilepticus - drug therapy; Status Epilepticus - etiology; Status Epilepticus - physiopathology; Young Adult
• ISSN: 1294-9361; 1950-6945; 1950-6945
• DOI: 10.1684/epd.2020.1218

Aims. Based on a multicenter cohort of people with anti‐NMDA receptor encephalitis (anti‐NMDARE), we describe seizure phenotypes, electroencephalographic (EEG) findings, and anti‐seizure treatment strategies. We also investigated whether specific electrographic features are associated with persistent seizures or status epilepticus after acute presentation. Methods. In this retrospective cohort study, we reviewed records of children and adults with anti‐NMDARE between 2010 and 2014 who were included in the Rare Epilepsy of New York City database, which included the text of physician notes from five academic medical centers. Clinical history (e.g., seizure semiology) and EEG features (e.g., background organization, slowing, epileptiform activity, seizures, sleep architecture, extreme delta brush) were ed. We compared clinical features associated with persistent seizures (ongoing seizures after one month from presentation) and status epilepticus, using bivariate and multivariable analyses. Results. Among the 38 individuals with definite anti‐NMDARE, 32 (84%) had seizures and 29 (76%) had seizures captured on EEG. Electrographic‐only seizures were identified in five (13%) individuals. Seizures started at a median of four days after initial symptoms (IQR: 3–6 days). Frontal lobe‐onset focal seizures were most common (n=12; 32%). Most individuals (31/38; 82%) were refractory to anti‐seizure medications. Status epilepticus was associated with younger age (15 years [9–20] vs. 23 years [18–27]; p=0.04) and Hispanic ethnicity (30 [80%] vs. 8 [36%]; p=0.04). Persistent seizures (ongoing seizures after one month from presentation) were associated with younger age (nine years [3–14] vs. 22 years [15–28]; p