Brain Inflammation in an Infant With Hemimegalencephaly, Escalating Seizures, and Epileptic Encephalopathy

• Type of Material: Article
• Authors: Se Hee Kim, Northwestern University; John J. Millichap, Northwestern University; Sookyong Koh, Emory University
• Language: English
• Date: 2016-04-04
• Publisher: SAGE Publications (UK and US)
• Publication Version: Final Published Version
• Copyright Statement: © 2016 The Author(s)
• License: Creative Commons Attribution-Noncommercial 3.0 Unported
• Final Published Version (URL): http://dx.doi.org/10.1177/2329048X16633629
• Title of Journal or Parent Work: Child Neurology Open
• ISSN: 2329-048X
• Volume: 3
• Issue: 0
• Grant/Funding Information: The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This study was supported by NIH/NINDS R01 NS073768 (SK).
• Keywords: Malformations of cortical development; Epilepsy; Neuroinflammation; Hemispherectomy; Early-onset seizures
• Research Categories: Health Sciences, Immunology; Health Sciences, Medicine and Surgery

Abstract

Hemimegalencephaly, a congenital brain malformation typically characterized by enlargement of one hemisphere, is frequently associated with intractable epilepsy. The authors report a case of a 12-month-old girl with hemimegalencephaly who underwent semiurgent hemispherectomy because of rapidly escalating seizures, arrested development, and associated encephalopathy. The brain tissue was examined and evaluated for neuroinflammation. Immunohistochemical analysis of the brain tissue revealed the presence of abundant activated CD68-positive microglia and reactive astrogliosis. Detection of active inflammatory changes in the brain of a patient with hemimegalencephaly complicated by intractable epilepsy suggests a potential role of ongoing brain inflammation in seizure exacerbation and epileptic encephalopathy.