基于尸检病例的自身免疫性脑炎回顾。

IF 1.8 4区 医学 Q3 MEDICINE, RESEARCH & EXPERIMENTAL Experimental Neurobiology Pub Date : 2024-02-29 DOI:10.5607/en23036
Yu-Mi Shim, Seong-Ik Kim, So Dug Lim, Kwanghoon Lee, Eric Eunshik Kim, Jae Kyung Won, Sung-Hye Park
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引用次数: 0

摘要

自身免疫性脑炎(AIE)是一种免疫反应性脑炎,是公认的最常见的非感染性脑炎。然而,通过活检或尸检明确诊断 AIE 的罕见性是了解和治疗该疾病的一大障碍。在本文中,我们介绍了 AIE 的病理结果,并根据一例表现为 CD8+ T 淋巴细胞占优势脑炎的 AIE 病例回顾了相关文献。我们描述了一名 80 岁男性死者的临床进展、影像诊断、实验室数据和尸检结果。患者在去世前 6 个月被诊断为肺结核,并接受了适当的药物治疗。入院前一周,患者出现了嗜睡、食欲下降和意识模糊等症状。虽然通过药物治疗(包括纠正低钠血症),患者的病情暂时有所好转,但病情发展迅速,6 周后死亡。脑组织显示灰质和白质、脑膜和血管周围有淋巴细胞浸润,其中以CD8+ T淋巴细胞为主,这表明这是一个AIE病例。没有检测到病毒感染或潜在肿瘤的证据。尸检显示,该患者还患有阿尔茨海默病、动脉粥样硬化、动脉硬化和与衰老相关的tau星形胶质细胞病变。这份报告强调了病理检查在诊断 AIE 中的关键作用,尤其是在无法进行血清学自身抗体检测或怀疑患者患有多种疾病时。
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An Autopsy-proven Case-based Review of Autoimmune Encephalitis.

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.

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来源期刊
Experimental Neurobiology
Experimental Neurobiology Neuroscience-Cellular and Molecular Neuroscience
CiteScore
4.30
自引率
4.20%
发文量
29
期刊介绍: Experimental Neurobiology is an international forum for interdisciplinary investigations of the nervous system. The journal aims to publish papers that present novel observations in all fields of neuroscience, encompassing cellular & molecular neuroscience, development/differentiation/plasticity, neurobiology of disease, systems/cognitive/behavioral neuroscience, drug development & industrial application, brain-machine interface, methodologies/tools, and clinical neuroscience. It should be of interest to a broad scientific audience working on the biochemical, molecular biological, cell biological, pharmacological, physiological, psychophysical, clinical, anatomical, cognitive, and biotechnological aspects of neuroscience. The journal publishes both original research articles and review articles. Experimental Neurobiology is an open access, peer-reviewed online journal. The journal is published jointly by The Korean Society for Brain and Neural Sciences & The Korean Society for Neurodegenerative Disease.
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