Lakshmi Shree Kulumani Mahadevan, Melissa Murphy, Marina Selenica, Elizabeth Latimer, Brent T Harris
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We examined the banked tissue and performed special stains, immunohistochemical, and immunofluorescence analyses to characterize the neuropathology of this condition. Histology of the temporal lobes, hippocampus, and basal ganglia shows mild gliosis and Alzheimer's type II astrocytes. Acute hypoxic ischemic changes were noted in hippocampus CA1 and CA2 areas. Immunostaining shows increased parenchymal/perivascular GFAP staining and many vessels with mild increases in CD3-, CD4-, and CD25-stained lymphocytes in the basal ganglia. The findings suggest that CD4- and CD25-positive T cells might have an important role in understanding the neuroinflammation and pathogenesis of this condition. 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引用次数: 0
摘要
与链球菌感染相关的小儿自身免疫性神经精神障碍(PANDAS)是指 A 组链球菌感染后神经精神症状的急性发作或加重。有人认为这是链球菌感染引起自身免疫反应的结果,但目前还没有足够的证据完全支持这一理论。虽然这种疾病主要被认为是一种儿童疾病,但有报道称它也会发生在成年人身上。PANDAS 是一种定义明确的临床实体,但其神经病理学尚未确定。我们描述了一名被诊断为 PANDAS 的 26 岁女性的临床病程。她自杀身亡,其大脑被制成生物样本用于进一步研究。我们检查了库中的组织,并进行了特殊染色、免疫组化和免疫荧光分析,以确定这种疾病的神经病理学特征。颞叶、海马和基底节的组织学表现为轻度胶质增生和阿尔茨海默氏症 II 型星形胶质细胞。海马 CA1 和 CA2 区出现急性缺氧缺血性改变。免疫染色显示,基底节实质/血管周围 GFAP 染色增加,许多血管中 CD3-、CD4- 和 CD25 染色的淋巴细胞轻度增加。研究结果表明,CD4 和 CD25 阳性的 T 细胞可能在了解神经炎症和这种疾病的发病机制方面起着重要作用。该病例是首例 PANDAS 神经病理学评估报告。
Clinicopathologic Characteristics of PANDAS in a Young Adult: A Case Report.
Pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS) is an acute onset or exacerbation of neuropsychiatric symptoms following a group A streptococcus infection. It is believed to be a result of autoimmune response to streptococcal infection, but there is insufficient evidence to fully support this theory. Although this disease is primarily thought to be a disease of childhood, it is reported to occur also in adults. PANDAS is a well-defined clinical entity, but the neuropathology of this condition has not been established yet. We describe the clinical course of a 26-year-old female diagnosed with PANDAS. She committed suicide and her brain was biobanked for further studies. We examined the banked tissue and performed special stains, immunohistochemical, and immunofluorescence analyses to characterize the neuropathology of this condition. Histology of the temporal lobes, hippocampus, and basal ganglia shows mild gliosis and Alzheimer's type II astrocytes. Acute hypoxic ischemic changes were noted in hippocampus CA1 and CA2 areas. Immunostaining shows increased parenchymal/perivascular GFAP staining and many vessels with mild increases in CD3-, CD4-, and CD25-stained lymphocytes in the basal ganglia. The findings suggest that CD4- and CD25-positive T cells might have an important role in understanding the neuroinflammation and pathogenesis of this condition. The case represents the first neuropathological evaluation report for PANDAS.
期刊介绍:
''Developmental Neuroscience'' is a multidisciplinary journal publishing papers covering all stages of invertebrate, vertebrate and human brain development. Emphasis is placed on publishing fundamental as well as translational studies that contribute to our understanding of mechanisms of normal development as well as genetic and environmental causes of abnormal brain development. The journal thus provides valuable information for both physicians and biologists. To meet the rapidly expanding information needs of its readers, the journal combines original papers that report on progress and advances in developmental neuroscience with concise mini-reviews that provide a timely overview of key topics, new insights and ongoing controversies. The editorial standards of ''Developmental Neuroscience'' are high. We are committed to publishing only high quality, complete papers that make significant contributions to the field.