AI Article Synopsis

  • - The study examined pediatric autoimmune limbic encephalitis (LE) in children under 18, analyzing clinical presentations, investigations, and management over a period from 2008 to 2021 across six UK centers.
  • - Out of 25 identified cases, all children experienced seizures, with significant hospital admissions and various treatments, including steroids and rituximab, though over half faced long-term issues like refractory seizures and memory impairment.
  • - The findings highlight the serious health impacts of autoimmune LE in children, indicating high morbidity and no significant treatment differences in long-term outcomes between those who received rituximab versus those who did not.

Article 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.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8791799PMC
http://dx.doi.org/10.1002/acn3.51494DOI Listing

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