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Abstract Details

Seropositive Pediatric Autoimmune Encephalitis: A Single Center Experience
Autoimmune Neurology
Autoimmune Neurology Posters (7:00 AM-5:00 PM)
023
To describe clinical characteristics and identify unique features associated with antibody-positive autoimmune encephalitis (AE). 
Over the last decade, pediatric AE has emerged as a new cause of encephalopathy, seizures, psychiatric symptoms and other neurological conditions. Identification of auto-antibodies allows for proper recognition and treatment of these conditions. 
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.

Among 53 AE cases, 19 had serum or CSF antibodies. Among antibody-positive AE patients, there were 9 (47%) males and 10 (53%) females; 10 (52.6%) anti-NMDAR-antibodies, 5 (26%) anti-VGKC-antibodies and 4 (21%) 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%). One patient with anti-LGI1 VGKC-antibodies had faciobrachial dystonic seizures. 2 (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. 7 (36.8%) had focal EEG findings. 9(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. 6 (31.5%) attained complete remission; 1 underwent excision of ovarian teratoma. Long term sequalae included neurocognitive disturbances (52.6%), epilepsy (31.5%) and movement disorders (5%).

The most frequent presentation of AE in our cohort was neuropsychiatric disturbances, followed by altered mental status. Given diverse presentations and findings on MRI, EEG and CSF, one should maintain broad differential diagnosis when evaluating pediatric patients with subacute onset neurological symptoms. 
Authors/Disclosures
Praveen Hariharan, MD (University of Minnesota)
PRESENTER
Dr. Hariharan has nothing to disclose.
Daniel A. Crespo, MD (Bryan) Dr. Crespo has nothing to disclose.
Geetanjali S. Rathore, MD, FAAN (Childrens NEBRASKA) Dr. Rathore has nothing to disclose.