Seropositive Pediatric Autoimmune Encephalitis: A Single Center Experience

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Mentor: Geetanjali Rathore Program: Neurology Type: Original research Background: The objective of the study is to describe the clinical characteristics and identify unique features associated with antibody-positive autoimmune encephalitis (AE). Methods: Charts of children aged below 18-years diagnosed with AE between 2005 and 2020 were reviewed. Demographics, clinical characteristics, laboratory, imaging, electrophysiological findings and long-term-neurological-sequalae were collected. Results: Among 53 AE cases, 19 had serum or CSF antibodies. Among antibody-positive AE patients, there were 9 (47%) male and 10 (53%) female patients of whom; 10 (52.6%) had anti-NMDAR-antibodies, 5 (26%) anti-VGKC-antibodies and 4 (21%) demonstrated GAD65-antibodies. Clinical presentation included neuropsychiatric symptoms (68.4%), altered mental status (63%), movement disorders (57.9%), new-onset seizures (52.6%), speech difficulties (15.7%), status epilepticus (10.5%) and sleep dysfunction (5%). Two patients (10.5%) required ICU admission and 9 (47.3%) experienced relapses needing readmissions. MRI Brain was abnormal with T2 hyperintensities in 13 patients (31.5%) - 3 in parietal lobe, 2 in temporal lobe and 2 in frontal lobe. Seven patients (36.8%) had focal EEG findings. Nine patients (47.3%) had CSF pleocytosis and protein was normal in all patients. Acute management consisted of several different combination regimens including intravenous steroids (89%), intravenous immunoglobulin (IVIG) (84%), rituximab (15.8%) and plasma exchange (PLEX) (10.5%). Maintenance regimen included IVIG (42%), Rituximab (36.8%) and 1 PLEX. Six patients (31.5%) attained complete remission and one patient underwent excision of ovarian teratoma. Long term sequalae included neurocognitive disturbances (52.6%), epilepsy (31.5%) and movement disorders (5%). Conclusion: The most frequent presentation of AE in our cohort was neuropsychiatric disturbances, followed by altered mental status. Given diverse clinical presentations, one should maintain broad differential diagnosis when evaluating pediatric patients with subacute onset neurological symptoms.

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