• Brain & development · Aug 2013

    Review Case Reports

    VGKC complex antibodies in pediatric severe acute encephalitis: a study and literature review.

    • Jainn-Jim Lin, Kuang-Lin Lin, Shao-Hsuan Hsia, Huei-Shyong Wang, Cheng-Hsun Chiu, and CHEESE Study Group.
    • Division of Pediatric Neurology, College of Medicine, Chang Gung University, Taoyuan, Taiwan.
    • Brain Dev. 2013 Aug 1; 35 (7): 630-5.

    BackgroundAntibodies to surface proteins like voltage-gated potassium channel (VGKC) complexes are increasingly found in different neurologic diseases and encephalitis in adults and recently, in children. Detecting such antibodies can help identify forms of encephalitis that may respond to immuno-therapies. However, there are few reports on VGKC complex antibodies in pediatric severe acute encephalitis.MethodsThis study retrospectively reviewed antibodies to VGKC, leucine-rich glioma-inactivated 1 (Lgi1), and contactin-associated protein-like 2 (Caspr2) in 46 children with severe acute encephalitis. Published cases of VGKC complex antibodies in pediatric encephalitis in the period of 2000-2012 were also reviewed.ResultsElevated VGKC complex antibodies (>100pM) were detected in one of the 46 children with severe acute encephalitis. The 4-year and 6-month-old girl presented with seizure and disturbed consciousness. Viral PCR/culture and serologic evidence of influenza A infection was noted. She also had complications of epilepsy, impaired cognition, and altered behavior and psychology. Antibodies to Lgi1 and Caspr2 were not detected. Ten previously published reports revealed that VGKC complex antibodies can occur in children with limbic encephalitis and acute or sub-acute encephalitis.ConclusionThe incidence of VGKC complex antibodies in pediatric severe acute encephalitis is not high with only one (2.2%) of 46 children in this study. And, this is the first report on the association of VGKC complex antibodies and patients with influenza A-related severe acute encephalitis. The mechanism of VGKC complex antibodies in pediatric severe acute encephalitis warrants further study.Copyright © 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

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