Abstract Details

Title IVIg Treatment Ameliorates Inflammation in Patients with Sleep-related Electrical Status Epilepticus (ESES)

Topic Autoimmune Neurology

Presentation(s) P5 - Poster Session 5 (5:30 PM-6:30 PM)

Poster/Presentation
Number 14-003

Objective This study aims to determine the levels of inflammatory parameters in Sleep-Related Electrical Status Epilepticus (ESES) patients treated with intravenous Immunoglobulin (IVIg) and to identify potential biomarkers associated with immunotherapy-responsiveness.

Background ESES is an electroclinical syndrome, particularly specific to childhood. While some patients have a structural brain disorder identified in the etiology, those with an unknown cause are classified as idiopathic. Previous studies have shown that steroid and IVIg are more effective for ESES compared to antiepileptic drugs and result in significant improvement in EEG findings.

Design/Methods The study included 34 idiopathic ESES patients (20 boys; 8.6±3.2-year old) and 20 healthy children (9 boys; 12.3±2.5-year old). Cerebrospinal fluid (CSF) and serum samples were collected before and one year after IVIg treatment. Levels of CXCL13, YKL-40/chitinase-3-like protein 1, glial fibrillary acidic protein (GFAP), high mobility group box 1 (HMGB1) and IL-2 receptor were measured by ELISA. A broad panel of cognitive, linguistic and psychiatric tests were administered before and one-year after IVIg treatment.

Results Levels of all inflammatory parameters were significantly reduced in both CSF and serum samples of ESES patients after IVIg treatment and showed trends toward approaching to levels of healthy individuals. Epileptic seizures and EEG findings of 18 patients regressed after IVIg treatment. Pre-treatment serum GFAP levels were significantly higher (p=0.020) and post-treatment CXCL13 levels were significantly lower (p=0.023) in treatment resistant ESES patients (n=16). A weak negative correlation was found between language test scores and serum/CSF YKL-40 and CXCL13 levels.

Conclusions Our findings indicate that IVIg treatment suppresses factors of neuroinflammation. Patients with enhanced astrocytic activity are more likely to be immunotherapy-resistant. The use of a multiplex test kit measuring a panel of neuroinflammation parameters may contribute to the monitorization of treatment status of ESES patients.

Authors/Disclosures
Zuhal Yapici, MD (Gen Pharmaceuticals) PRESENTER	Dr. Yapici has nothing to disclose.
Hande Yuceer Korkmaz	No disclosure on file
Gizem Koral (Istanbul University)	No disclosure on file
Pinar Topaloglu	No disclosure on file
Ahmed Serkan Emekli (Department of Neurology, Istanbul Faculty of Medicine, Istanbul University)	Ahmed Serkan Emekli has nothing to disclose.
Elif Is (Istanbul University)	No disclosure on file
Zerrin Karaaslan, MD, PhD	Dr. Karaaslan has nothing to disclose.
Merve Savas (Istanbul Atlas University)	No disclosure on file
FIRAT OZ	No disclosure on file
Cem Kucukali	No disclosure on file
Vuslat Yilmaz	No disclosure on file
Erdem Tuzun	Erdem Tuzun has nothing to disclose.

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