Clinical features and outcomes in children with seronegative autoimmune encephalitis.

Journal: Developmental medicine and child neurology

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Affiliated Institutions:  Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Neurosciences and Mental Health Program, SickKids Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada. Division of Neurology, Dalhousie University IWK Health Center, Halifax, Nova Scotia, Canada. Division of Neurology, McMaster University, Hamilton, Ontario, Canada. Division of Pediatric Neurology, Department of Pediatrics, Children's Hospital, London Health Science Center, London, Ontario, Canada. Pediatric Neurology, Department of Pediatrics, Saint John Regional Hospital, St. John, New Brunswick, Canada. Division of Neurology, Department of Pediatrics, University of Alberta, Alberta, Canada.

Abstract summary 

To characterize the presenting features and outcomes in children with seronegative autoimmune encephalitis, and to evaluate whether scores at nadir for the Modified Rankin Scale (mRS) and Clinical Assessment Scale for Autoimmune Encephalitis (CASE) or its paediatric-specific modification (ped-CASE) are predictive of outcomes.This observational study included children younger than 18 years of age with seronegative autoimmune encephalitis. Demographics and clinical data were collected. The mRS and CASE/ped-CASE scores were used to evaluate disease severity. Descriptive statistics and logistic regression were used for data analysis and to evaluate associations between scale scores and outcomes.Sixty-three children were included (39 [62%] females, median age 7 years, interquartile range [IQR] 4 years 1 months-11 years 6 months), with follow-up available for 56 out of 63 patients (median follow-up 12.2 months, IQR 13.4-17.8). The most frequent presenting neurological manifestation was encephalopathy (81%). Median CASE/ped-CASE and mRS scores at nadir were 12.0 (IQR 7.0-17.0) and 1.0 (IQR 0-2.0) respectively. Thirty-three patients (59%) had persistent neurological deficits at follow-up. Both scoring systems suggested good functional recovery (mRS score ≤2, 95%; CASE/ped-CASE score <5, 91%). CASE/ped-CASE score was more likely than mRS to distinguish children with worse outcomes.Children with seronegative autoimmune encephalitis are likely to have neurological deficits at follow-up. CASE/ped-CASE is more likely to distinguish children with worse outcomes than MRS.

Authors & Co-authors:  Madani Yea Mahjoub Brna Jones Longoni Nouri Rizk Stewart Wilbur Yeh

Study Outcome 

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Statistics
Citations :  Granerod J, Cousens S, Davies NW, Crowcroft NS, Thomas SL. New estimates of incidence of encephalitis in England. Emerg Infect Dis. 2013;19(9):1455-62.
Authors :  11
Identifiers
Doi : 10.1111/dmcn.15896
SSN : 1469-8749
Study Population
Male,Female
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Publication Country
England