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 DetailsFirst-Page Preview
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
AbstractAnti-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 DetailsFirst-Page Preview
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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