TY - JOUR
T1 - Clinical features, investigations, and outcomes of pediatric limbic encephalitis: A multicenter study
AU - Sabanathan, Saraswathy
AU - Abdel‐Mannan, Omar
AU - Mankad, Kshitij
AU - Siddiqui, Ata
AU - Das, Krishna
AU - Carr, Lucinda
AU - Eltze, Christin
AU - Eyre, Michael
AU - Gadian, Jon
AU - Hemingway, Cheryl
AU - Kaliakatsos, Marios
AU - Kneen, Rachel
AU - Krishnakumar, Deepa
AU - Lynch, Bryan
AU - Parida, Amitav
AU - Rossor, Thomas
AU - Taylor, Micheal
AU - Wassmer, Evangeline
AU - Wright, Sukhvir
AU - Lim, Ming
AU - Hacohen, Yael
N1 - Funding Information:
Cheryl Hemingway has received educational and travel grants from Merck Serono and Bayer and Biogen.
Funding Information:
Omar Abdel‐mannan is funded by an Association of British Neurologists, MS Society, and The Berkeley Foundation's A. W. Pidgley Memorial Clinical Research Training Fellowship (ABN/182533). Michael Eyre is supported by Action Medical Research and the British Paediatric Neurology Association (GN2835). Cheryl Hemingway is funded by an MRC Clinical Academic Research Partnership grant (MR/T024437/1) Yael Hacohen is funded by the UK MS Society (Award number 92).
Funding Information:
Ming Lim receives research grants from Action Medical Research, the DES society, GOSH charity, National Institute for Health Research, MS Society, and SPARKS charity; receives research support grants from the London Clinical Research Network and Evelina Appeal; has received consultation fees from CSL Behring, Novartis and Octapharma; received travel grants from Merck Serono.
Funding Information:
We thank all the numerous primary physicians and allied health professionals caring for these children during their complex course of disease. The authors thank the following physicians; Prab Prabhakar, Sanjay Bhate, Catherine Devile, Suresh Pujar (Great Ormond Street Hospital), Elaine Hughes, Karine Lascelles, Shan-Shan Tang, Jean-Pierre Lin, Ruth Williams (Evelina London Children's Hospital), Manali Chitre (Addenbrooke's Hospital), Nicholas M. Allen and Eilish Twomey (Children's University Hospital, Dublin), and the allied health professionals who cared for children during their disease course.
Publisher Copyright:
© 2022 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.
PY - 2022/1
Y1 - 2022/1
N2 - 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.
AB - 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.
KW - Research Article
KW - Research Articles
UR - http://www.scopus.com/inward/record.url?scp=85122684539&partnerID=8YFLogxK
U2 - 10.1002/acn3.51494
DO - 10.1002/acn3.51494
M3 - Article
SN - 2328-9503
VL - 9
SP - 67
EP - 78
JO - Annals of Clinical and Translational Neurology
JF - Annals of Clinical and Translational Neurology
IS - 1
ER -