Anti-IgLON5 Encephalopathy with Concomitant Herpes Virus Encephalitis

Wang Y.a· Li R.a· Sun X.a· Liao J.a· Li J.b· Xia H.c· Peng L.a· Qiu W.a· Shu Y.a

Author affiliations

aDepartment of Neurology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
bDepartment of Nuclear Medicine, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
cDepartment of Research and Development, Hugobiotech Co., Ltd., Beijing, China

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Article / Publication Details

First-Page Preview

Abstract of Case Report

Received: August 19, 2021
Accepted: November 08, 2021
Published online: March 30, 2022

Number of Print Pages: 5
Number of Figures: 3
Number of Tables: 0

ISSN: 1021-7401 (Print)
eISSN: 1423-0216 (Online)

For additional information: https://www.karger.com/NIM

Abstract

Anti-IgLON5 encephalopathy is a new and rare autoimmune encephalitis with unclear pathophysiology. In this study, we reported an unusual case of anti-IgLON5 encephalopathy with concomitant herpes virus encephalitis. A 51-year-old man with HLA-DQB1*05:01 and HLA-DRB1*10:01, who suffered from an episode of acute encephalitis, mental disorders, and memory impairment was admitted to our hospital. Human alpha herpes virus 1, human gamma herpes virus 4 (Epstein-Barr virus), and IgLON5-IgG were detected in the cerebrospinal fluid, indicating anti-IgLON5 encephalopathy with concomitant herpes virus encephalitis of this patient. Brain magnetic resonance imaging revealed T2 hyperintensities in the left temporal lobe and enhancement in the hippocampus. A mild sleep disorder was also found by video polysomnography. The patient was then treated with antiviral drugs, intravenous immunoglobulins, methylprednisolone, and protein A immunoadsorption. After treatment, the patient’s clinical symptoms were partially improved. This is the first reported case of anti-IgLON5 encephalopathy with concomitant herpes virus encephalitis.

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References Sabater L, Gaig C, Gelpi E, Bataller L, Lewerenz J, Torres-Vega E, et al. A novel non-rapid-eye movement and rapid-eye-movement parasomnia with sleep breathing disorder associated with antibodies to IgLON5: a case series, characterisation of the antigen, and post-mortem study. Lancet Neurol. 2014;13:575–86. Gaig C, Graus F, Compta Y, Högl B, Bataller L, Brüggemann N, et al. Clinical manifestations of the anti-IgLON5 disease. Neurology. 2017;88:1736–43. Landa J, Gaig C, Plaguma J, Saiz A, Antonell A, Sanchez-Valle R, et al. Effects of IgLON5 antibodies on neuronal cytoskeleton: a link between autoimmunity and neurodegeneration. Ann Neurol. 2020;88:1023–7. Chen N, Wang W, Wang F, Dong L, Zhao S, Zhang W, et al. The distributions of HLA-A, HLA-B, HLA-C, HLA-DRB1 and HLA-DQB1 allele and haplotype at high-resolution level in Zhejiang Han population of China. Int J Immunogenet. 2019;46:7–16. Armangue T, Leypoldt F, Malaga I, Raspall-Chaure M, Marti I, Nichter C, et al. Herpes simplex virus encephalitis is a trigger of brain autoimmunity. Ann Neurol. 2014;75:317–23. Armangue T, Moris G, Cantarin-Extremera V, Conde CE, Rostasy K, Erro ME, et al. Autoimmune post-herpes simplex encephalitis of adults and teenagers. Neurology. 2015;85:1736–43. Article / Publication Details

First-Page Preview

Abstract of Case Report

Received: August 19, 2021
Accepted: November 08, 2021
Published online: March 30, 2022

Number of Print Pages: 5
Number of Figures: 3
Number of Tables: 0

ISSN: 1021-7401 (Print)
eISSN: 1423-0216 (Online)

For additional information: https://www.karger.com/NIM

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