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

Ann Clin Transl Neurol. 2022 Jan;9(1):67-78. doi: 10.1002/acn3.51494. Epub 2022 Jan 11.

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 types

  • Multicenter Study
  • Observational Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Autoantibodies / blood
  • Autoantibodies / cerebrospinal fluid
  • Autoantibodies / immunology*
  • Autoimmune Diseases* / immunology
  • Autoimmune Diseases* / pathology
  • Autoimmune Diseases* / physiopathology
  • Autoimmune Diseases* / therapy
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Humans
  • Immunologic Factors / administration & dosage*
  • Intensive Care Units, Pediatric
  • Limbic Encephalitis* / immunology
  • Limbic Encephalitis* / pathology
  • Limbic Encephalitis* / physiopathology
  • Limbic Encephalitis* / therapy
  • Male
  • Outcome Assessment, Health Care
  • Plasma Exchange*
  • Retrospective Studies
  • Rituximab / administration & dosage
  • Seizures

Substances

  • Autoantibodies
  • Immunologic Factors
  • Rituximab

Supplementary concepts

  • Autoimmune limbic encephalitis