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

Saraswathy Sabanathan, Omar Abdel‐Mannan, Kshitij Mankad, Ata Siddiqui, Krishna Das, Lucinda Carr, Christin Eltze, Michael Eyre, Jon Gadian, Cheryl Hemingway, Marios Kaliakatsos, Rachel Kneen, Deepa Krishnakumar, Bryan Lynch, Amitav Parida, Thomas Rossor, Micheal Taylor, Evangeline Wassmer, Sukhvir Wright, Ming Lim*Yael Hacohen*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

7 Citations (Scopus)
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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.

Original languageEnglish
Pages (from-to)67-78
Number of pages12
JournalAnnals of Clinical and Translational Neurology
Volume9
Issue number1
Early online date11 Jan 2022
DOIs
Publication statusPublished - Jan 2022

Keywords

  • Research Article
  • Research Articles

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