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  • KSBNS 2024

Article

Review Article

Exp Neurobiol 2024; 33(1): 1-17

Published online February 29, 2024

https://doi.org/10.5607/en23036

© The Korean Society for Brain and Neural Sciences

An Autopsy-proven Case-based Review of Autoimmune Encephalitis

Yu-Mi Shim1†, Seong-Ik Kim1†, So Dug Lim2, Kwanghoon Lee1, Eric Eunshik Kim1, Jae Kyung Won1 and Sung-Hye Park1,3*

1Department of Pathology, Seoul National University College of Medicine, Seoul 03080,
2Department of Pathology, KonKuk University School of Medicine, Seoul 05029,
3Institute of Neuroscience, Seoul National University College of Medicine, Seoul 03080, Korea

Correspondence to: *To whom correspondence should be addressed.
TEL: 82-2-2072-3090, FAX: 82-2-743-5530
e-mail: shparknp@snu.ac.kr
These authors contributed equally to this article.

Received: October 16, 2023; Revised: February 19, 2024; Accepted: February 20, 2024

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Autoimmune encephalitis (AIE) is a type of immunoreactive encephalitic disorder and is recognized as the most prevalent noninfectious encephalitis. Nevertheless, the rarity of definitive AIE diagnosis through biopsy or autopsy represents a significant hurdle to understanding and managing the disease. In this article, we present the pathological findings of AIE and review the literature based on a distinct case of AIE presenting as CD8+ T-lymphocyte predominant encephalitis. We describe the clinical progression, diagnostic imaging, laboratory data, and autopsy findings of an 80-year-old deceased male patient. The patient was diagnosed with pulmonary tuberculosis 6 months before death and received appropriate medications. A week before admission to the hospital, the patient manifested symptoms such as a tendency to sleep, decreased appetite, and confusion. Although the patient temporally improved with medication including correction of hyponatremia, the patient progressed rapidly and died in 6 weeks. The brain tissue revealed lymphocytic infiltration in the gray and white matter, leptomeninges, and perivascular infiltration with a predominance of CD8+ T lymphocytes, suggesting a case of AIE. There was no detectable evidence of viral infection or underlying neoplasm. The autopsy revealed that this patient also had Alzheimer’s disease, atherosclerosis, arteriolosclerosis, and aging-related tau astrogliopathy. This report emphasizes the pivotal role of pathological examination in the diagnosis of AIE, especially when serological autoantibody testing is not available or when a patient is suspected of having multiple diseases.

Graphical Abstract


Keywords: Autoimmune diseases of the nervous system, Encephalitis, Autopsy, CD8-positive T-lymphocytes, Noninfectious disease