Abstract
ObjectivesTo describe the clinical presentation, investigations, management, and disease course in pediatric autoimmune limbic encephalitis (LE).MethodsIn 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.ResultsTwenty‐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.InterpretationA diagnosis of autoimmune LE was associated with significant morbidity and adverse outcomes in this pediatric cohort.
Highlights
Autoimmune limbic encephalitis (LE) is a rare but wellrecognized neuroinflammatory condition in which patients typically present with memory deficits, seizures, and psychiatric disturbances alongside radiologic changes in the medial temporal lobe.[1,2] The mean incidence rates of antibodymediated autoimmune encephalitis in the Netherlands between 2015 and 2018 was 1.54 children/million.[3]
LE was first described as a paraneoplastic syndrome associated with small cell lung cancer[4] in adult patients; with the discovery of antineuronal antibodies, we know that it most frequently occurs in association with antibodies against the neuronal secreted protein leucine-rich glioma-inactivated (LGI1) and contactin-associated protein-like 2 (CASPR2).[5,6]
The majority of studies in the pediatric literature have focused on studying autoimmune encephalitis with known antibodies against cell surface (e.g., GABA(B)R-ab, AMPA, and glycine receptors) or synaptic proteins (e.g., GAD, LGI1 and CASPR2); an example is anti-N-methylD-aspartate receptor encephalitis, the most frequently described pediatric autoimmune encephalitis (AE), in which the antibodies target the NR1 subunit of the receptor.[9]
Summary
Autoimmune limbic encephalitis (LE) is a rare but wellrecognized neuroinflammatory condition in which patients typically present with memory deficits, seizures, and psychiatric disturbances alongside radiologic changes in the medial temporal lobe.[1,2] The mean incidence rates of antibodymediated autoimmune encephalitis in the Netherlands between 2015 and 2018 was 1.54 children/million (95% CI 0.95–2.35).[3]. The majority of studies in the pediatric literature have focused on studying autoimmune encephalitis with known antibodies against cell surface (e.g., GABA(B)R-ab, AMPA, and glycine receptors) or synaptic proteins (e.g., GAD, LGI1 and CASPR2); an example is anti-N-methylD-aspartate receptor (anti-NMDAR) encephalitis, the most frequently described pediatric autoimmune encephalitis (AE), in which the antibodies target the NR1 subunit of the receptor.[9]
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