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

Area of Science:

• Pediatric Neurology
• Neuroimmunology
• Autoimmune Encephalitis

Background:

• Autoimmune limbic encephalitis (LE) is a rare but severe neurological condition affecting children.
• Understanding its clinical spectrum, management, and outcomes is crucial for improving patient care.

Purpose of the Study:

• To delineate the clinical presentation, diagnostic investigations, treatment strategies, and disease trajectory of pediatric autoimmune LE.
• To identify factors influencing outcomes in children diagnosed with autoimmune LE.

Main Methods:

• A retrospective observational study was conducted using data from the UK Childhood Neuroinflammatory Disease network.
• Twenty-five children under 18 diagnosed with LE between 2008 and 2021 were identified from six tertiary centers.
• Clinical and paraclinical data were systematically collected from medical records.

Main Results:

• The cohort of 25 children presented with seizures, intensive care unit admissions, and varied neuroimaging findings.
• Antibody testing revealed limited positivity for common antibodies, with two cases positive for anti-NMDAR and two for anti-GAD antibodies.
• Initial treatments included steroids, IVIg, and plasma exchange, with rituximab as a common second-line therapy. Despite treatment, 52% had refractory seizures and 64% had memory impairment at follow-up.

Conclusions:

• Autoimmune LE in children is associated with substantial morbidity, including refractory epilepsy and cognitive deficits.
• Current treatment approaches, including rituximab, did not show a significant difference in modified Rankin Scale scores or long-term outcomes in this cohort.
• Further research is needed to optimize therapeutic strategies for pediatric autoimmune LE.