eprintid: 10142386
rev_number: 12
eprint_status: archive
userid: 608
dir: disk0/10/14/23/86
datestamp: 2022-01-25 11:53:27
lastmod: 2022-01-25 11:53:27
status_changed: 2022-01-25 11:53:27
type: article
metadata_visibility: show
creators_name: Sabanathan, S
creators_name: Abdel-Mannan, O
creators_name: Mankad, K
creators_name: Siddiqui, A
creators_name: Das, K
creators_name: Carr, L
creators_name: Eltze, C
creators_name: Eyre, M
creators_name: Gadian, J
creators_name: Hemingway, C
creators_name: Kaliakatsos, M
creators_name: Kneen, R
creators_name: Krishnakumar, D
creators_name: Lynch, B
creators_name: Parida, A
creators_name: Rossor, T
creators_name: Taylor, M
creators_name: Wassmer, E
creators_name: Wright, S
creators_name: Lim, M
creators_name: Hacohen, Y
title: Clinical features, investigations, and outcomes of pediatric limbic encephalitis: A multicenter study
ispublished: inpress
divisions: UCL
divisions: B02
divisions: C07
divisions: D07
divisions: F87
note: © 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 (http://creativecommons.org/licenses/by/4.0/).
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.
date: 2022-01-11
date_type: published
official_url: https://doi.org/10.1002/acn3.51494
oa_status: green
full_text_type: pub
language: eng
primo: open
primo_central: open_green
verified: verified_manual
elements_id: 1915671
doi: 10.1002/acn3.51494
language_elements: English
lyricists_name: Abdel-Mannan, Omar
lyricists_name: Hacohen, Yael
lyricists_id: OABDE15
lyricists_id: YHACO24
actors_name: Flynn, Bernadette
actors_id: BFFLY94
actors_role: owner
full_text_status: public
publication: Annals of Clinical and Translational Neurology
event_location: United States
citation:        Sabanathan, S;    Abdel-Mannan, O;    Mankad, K;    Siddiqui, A;    Das, K;    Carr, L;    Eltze, C;                                                         ... Hacohen, Y; + view all <#>        Sabanathan, S;  Abdel-Mannan, O;  Mankad, K;  Siddiqui, A;  Das, K;  Carr, L;  Eltze, C;  Eyre, M;  Gadian, J;  Hemingway, C;  Kaliakatsos, M;  Kneen, R;  Krishnakumar, D;  Lynch, B;  Parida, A;  Rossor, T;  Taylor, M;  Wassmer, E;  Wright, S;  Lim, M;  Hacohen, Y;   - view fewer <#>    (2022)    Clinical features, investigations, and outcomes of pediatric limbic encephalitis: A multicenter study.                   Annals of Clinical and Translational Neurology        10.1002/acn3.51494 <https://doi.org/10.1002/acn3.51494>.    (In press).    Green open access   
 
document_url: https://discovery-pp.ucl.ac.uk/id/eprint/10142386/1/Ann%20Clin%20Transl%20Neurol%20-%202022%20-%20Sabanathan%20-%20Clinical%20features%20%20investigations%20%20and%20outcomes%20of%20pediatric%20limbic.pdf