Clinical features, investigations, and outcomes of pediatric limbic encephalitis:A multicenter study

Abstract

Objectives: To describe the clinical presentation, investigations, management, and disease course in pediatric autoimmune limbic encephalitis (LE). Methods: In this retrospective observational study, from the UK Childhood Neuroinflammatory Disease network, we identified children from six tertiary centers with LE <18 years old between 2008 and 2021. Clinical and paraclinical data were retrieved from medical records. Results: Twenty-five children fulfilling LE criteria were identified, with median age of 11 years (IQR 8, 14) and median follow-up of 24 months (IQR 18, 48). All children presented with seizures; 15/25 (60%) were admitted to intensive care. Neuroimaging demonstrated asymmetric mesial temporal changes in 8/25 (32%), and extra-limbic changes with claustrum involvement in 9/25 (38%). None were positive for LGI1/CASPR2 antibodies (Abs), 2/25 were positive for serum anti-NMDAR Abs, and 2/15 positive for anti-Hu Abs; one died from relapsing neuroblastoma. Two children had serum and CSF anti-GAD antibodies. Initial immune therapy included steroids in 23/25 (92%), intravenous immunoglobulin (IVIg) in 14/25 (56%), and plasma exchange in 7/25 (28%). The commonest second-line treatment was rituximab in 15/25 (60%). Median duration of hospital admission was 21 days (IQR 11, 30). At last follow-up, 13/25 (52%) had refractory seizures and 16/25 (64%) had memory impairment. Six children (24%) had modified Rankin Scale (mRS) scores ≥3. There was no significant difference in mRS, or long-term cognitive and epilepsy outcomes in those who received rituximab versus those who did not. Interpretation: A diagnosis of autoimmune LE was associated with significant morbidity and adverse outcomes in this pediatric cohort.

Publication DOI: https://doi.org/10.1002/acn3.51494
Divisions: College of Health & Life Sciences > School of Biosciences
College of Health & Life Sciences > Aston Pharmacy School
College of Health & Life Sciences > Aston Institute of Health & Neurodevelopment (AIHN)
College of Health & Life Sciences
Additional Information: © 2022 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. Funding: MRC Clinical Academic Research Partnership. Grant Number: MR/T024437/1; British Paediatric Neurology Association. Grant Number: GN2835; Action Medical Research; Berkeley Foundation's A. W. Pidgley Memorial Clinical Research Training Fellowship. Grant Number: ABN/182533; MS Society; Association of British Neurologists
Uncontrolled Keywords: Neuroscience(all),Clinical Neurology
Publication ISSN: 2328-9503
Full Text Link:
Related URLs: http://www.scop ... tnerID=8YFLogxK (Scopus URL)
https://onlinel ... 1002/acn3.51494 (Publisher URL)
PURE Output Type: Article
Published Date: 2022-01
Published Online Date: 2022-01-11
Accepted Date: 2021-12-13
Authors: Sabanathan, Saraswathy
Abdel-Mannan, Omar
Mankad, Kshitij
Siddiqui, Ata
Das, Krishna
Carr, Lucinda
Eltze, Christin
Eyre, Michael
Gadian, Jon
Hemingway, Cheryl
Kaliakatsos, Marios
Kneen, Rachel
Krishnakumar, Deepa
Lynch, Bryan
Parida, Amitav
Rossor, Thomas
Taylor, Micheal
Wassmer, Evangeline
Wright, Sukhvir (ORCID Profile 0000-0002-5464-3779)
Lim, Ming
Hacohen, Yael

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