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. 2020 Oct;10(10):e01793.
doi: 10.1002/brb3.1793. Epub 2020 Aug 12.

Anti-LGI1, anti-GABABR, and Anti-CASPR2 encephalitides in Asia: A systematic review

Prinska Ghimire  1 , Ujjwal Prakash Khanal  1 , Bikram Prasad Gajurel  2 , Ragesh Karn  2 , Reema Rajbhandari  2 , Sunanda Paudel  2 , Niraj Gautam  2 , Rajeev Ojha  2
Affiliations

Anti-LGI1, anti-GABABR, and Anti-CASPR2 encephalitides in Asia: A systematic review

Prinska Ghimire et al. Brain Behav. 2020 Oct.

Abstract

Aim: We aim to review the literature to collate and describe features of encephalitides arising from autoantibodies against leucine-rich glioma-inactivated 1 (LGI1), gamma aminobutyric acid receptor (GABABR), and contactin-associated protein-like 2 (CASPR2) in Asian populations and compare them with findings of Western studies.

Methods: Peer-reviewed articles published till 24 May 2020 were searched, and original, full-text studies from Asia with serum/CSF antibody-based diagnosis and at least 2 patients were selected. Twenty-four studies with 263 patients (139 anti-LGI1, 114 anti-GAGABR, and 10 anti-CASPR2) were included. Data were pooled to produce descriptive information on demographics, clinical characteristics, diagnostics, treatments, and outcome.

Results: The mean age was 54.2 (anti-LGI1), 55.2 (anti-GABABR), and 47.7 years (anti-CASPR2), with an overall male predominance of 62.0%. Commonest clinical features across all types were seizures (87.5%), memory deficits (80.7%), psychiatric disturbances (75.9%), and altered consciousness (52.9%). Four anti-LGI1, 40 anti-GABABR, and 1 anti-CASPR2 patients had tumors. CSF, MRI, and EEG were abnormal in 33.3%, 54.1%, and 75% patients in anti-LGI1; 60.0%, 49.6%, and 85.7% in anti-GABABR; and 50%, 44.4%, and 100% in anti-CASPR2 patients, respectively. 95.6% patients received first-line therapy alone (steroids/IVIG/Plasma therapy), and 4.4% received second-line therapy (rituximab/cyclophosphamide). 91.7%, 63.6%, and 70% of patients had favorable outcomes (modified Rankin Score 0-2) with mortality rates at 2.5%, 23.2%, and 0% in the three types, respectively.

Conclusion: Our findings suggest that these disorders present in Asian patients at a relatively young age often with features of seizures, memory deficits, and psychiatric disturbances and usually demonstrate a favorable clinical outcome.

Keywords: anti-CASPR2 encephalitis; anti-GABABR encephalitis; anti-LGI1 encephalitis; autoimmune encephalitis.

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Conflict of interest statement

None of the authors have any conflicts of interest to disclose.

Figures

FIGURE 1
FIGURE 1
PRISMA flowchart showing the selection process for included studies
See this image and copyright information in PMC

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