Clinical features of paediatric and adult autoimmune encephalitis: A multicenter sample. (January 2021)

• Record Type: Journal Article
• Authors: Roliz, Annie; Shah, Yash; Morse, Anne; Troester, Matthew; Lynch, Rebecca; Pickle, Jacob; Karkare, Shefali; Fernandez-Carbonell, Cristina; Kothare, Sanjeev
• Is Part Of: European journal of paediatric neurology. Volume 30(2021)
• Page Start: 82
• Page End: 87
• Publication Date: 2021-01
• Subjects: Autoimmune encephalitis -- Sleep -- Epilepsy -- Seizures -- Adults -- Children. character count for title: 86; Pediatric neurology -- Periodicals; Nervous System Diseases -- Periodicals; Child -- Periodicals; Infant -- Periodicals; Neurologie pédiatrique -- Périodiques; Pediatric neurology; Electronic journals; Periodicals; Electronic journals; 618.928
• DOI: 10.1016/j.ejpn.2021.01.001
• Languages: English
• ISSNs: 1090-3798

Abstract

Abstract: Background: Autoimmune encephalitis (AE) is a heterogeneous class of inflammatory diseases of the brain that can present with a wide spectrum of neuropsychiatric symptoms. Patients may be negative for CSF anti-neuronal antibodies, which can make the diagnosis of AE challenging. Distinguishing features between paediatric and adult patients with AE are not well characterized. Objective: Describe the clinical presentation, seizure type, EEG and sleep patterns in paediatric and adult patients with AE. Methods/Design: Retrospective review of clinical data from paediatric and adult patients diagnosed with AE from three medical centers between 1/2008–12/2019. Results: We included 100 patients with AE, including 65 children. Median age at presentation was 14 years (1–88years). Fifty-five patients had positive CSF autoantibody results (NMDAR 36%, VGKC Ab 10%, anti-GAD65 4%, miscellaneous 3%), and 47 patients were autoantibody-negative. Paediatric patients were more likely to present with psychiatric symptoms, focal seizures and/or status epilepticus, and sleep disturbances compared to adult patients (p < 0.05). There was a higher incidence of NMDA-R encephalitis in children compared to adults. Conclusion: Paediatric patients with AE were more likely to present with psychiatric symptoms, sleep disturbances, focal seizures, and/or status epilepticus compared to adults (p < 0.05). Insomnia and hypersomnia are common sleep problems associated with AE that should be screened early in the diagnostic evaluation. Further studies can be performed to explore the relationship between sleep disturbances and long-term cognitive effects and the incidence of chronic epilepsy in this subset of patients. Highlights: Differences in clinical manifestations of AE between children and adults is useful in establishing an earlier diagnosis. Prevalence of psychiatric symptoms, focal seizures, status epilepticus, and sleep disturbances are more common in children. Sleep disturbances are an important early symptom of AE that should be screened for in the initial diagnosis of AE.