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

来  源:   DOI:10.5607/en23036   PDF(Pubmed)

Abstract:
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.
摘要:
自身免疫性脑炎(AIE)是一种免疫反应性脑炎,被认为是最常见的非感染性脑炎。然而,通过活检或尸检进行明确的AIE诊断非常罕见,这是对了解和管理疾病的重大障碍.在这篇文章中,我们介绍了AIE的病理学发现,并根据一例以CD8+T淋巴细胞为主的脑炎表现的AIE病例对文献进行了综述.我们描述了临床进展,诊断成像,实验室数据,以及一名80岁男性患者的尸检结果。患者在死亡前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,患者表现出睡眠倾向等症状,食欲下降,和困惑。尽管患者通过药物治疗包括纠正低钠血症在时间上有所改善,患者进展迅速,在6周内死亡。脑组织显示灰质和白质有淋巴细胞浸润,软脑膜,和以CD8+T淋巴细胞为主的血管周围浸润,暗示了一个AIE的案例。没有检测到病毒感染或潜在肿瘤的证据。尸检显示这个病人也有老年痴呆症,动脉粥样硬化,动脉硬化,和衰老相关的tau星形胶质细胞病。本报告强调病理检查在AIE诊断中的关键作用。特别是当血清学自身抗体检测不可用或当患者被怀疑患有多种疾病时。
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