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Patient characteristics and outcome in patients with anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis

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Abstract

Objective

Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis is a rare subtype of autoimmune encephalitis. We report patients diagnosed with anti-AMPAR encephalitis in western China, focusing on their clinical presentations, imaging results, treatment strategies, and prognosis.

Methods

Data from patients diagnosed with anti-AMPAR encephalitis in the neurology center of West China Hospital from August 2018 to July 2021 were retrospectively collected and analyzed. Based on the diagnostic criteria of autoimmune encephalitis, nine cases were included.

Results

Four patients (44%) were males, and the median age at presentation was 54 years (range, 25–85). Short-term memory loss was the most common initial symptom. Additional types of autoantibodies were identified in three patients. After presentation, four patients were found to have tumors: two with small cell lung cancer, one with ovarian teratoma, and one with thymoma. All patients accepted first-line immune therapy, and follow-up was available from 8 patients (median 20 weeks, range 4–78). At the last follow-up, three patients showed good outcomes (modified Rankin scale [mRS] 0–2; 37.5%). Five patients showed poor outcomes (mRS 3–6; 62.5%): two had minimal changes and remained hospitalized, two had residual severe cognitive impairments, and one patient died during follow-up. Outcomes were worse among patients with tumors. Finally, only one patient experienced relapse during follow-up.

Conclusion

Anti-AMPAR encephalitis should be considered in the differential diagnosis for middle- and senior-aged patients who present with predominantly acute or subacute short-term memory impairment. The long-term prognosis is correlated with the presence of a tumor.

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Data availability

All data contained in the article are available from the corresponding author upon reasonable request.

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Acknowledgements

The authors are indebted to our patient and her family for their support and permission.

Funding

Supported by the National Natural Science Foundation of China (grants 82071459) and the Institute of Brain Science and Brain-Inspired Technology of West China Hospital, Sichuan University (grants ZYJC21001).

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Author notes

  1. Jingfang Lin and Jierui Wang equally contributed to this study.

Authors and Affiliations

  1. Department of Neurology, West China Hospital, Sichuan University, No. 37 Guoxue Road, Chengdu, 610041, Sichuan, China

    Jingfang Lin, Jierui Wang & Jinmei Li

Authors
  1. Jingfang Lin
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  2. Jierui Wang
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  3. Jinmei Li
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Contributions

LJF and WJR collected the data and wrote the manuscript, and LJM conceptualized and revised the manuscript. All authors reviewed the manuscript.

Corresponding author

Correspondence to Jinmei Li.

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Ethical approval

This study was approved by the Research Ethics Committee of the West China Hospital of Sichuan University, and written informed consent was obtained from all participants.

Consent to participate

Written informed consent was obtained from the participants included in the study.

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The authors declare no competing interests.

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Lin, J., Wang, J. & Li, J. Patient characteristics and outcome in patients with anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis. Neurol Sci 44, 3253–3259 (2023). https://doi.org/10.1007/s10072-023-06769-x

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  • DOI: https://doi.org/10.1007/s10072-023-06769-x

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