Department of Pathology, Medstar Georgetown University Hospital, Washington, , , District of Columbia, USA.
Latimer Neurology Center, Washington, , District of Columbia, USA.
Dev Neurosci. 2023;45(6):335-341. doi: 10.1159/000534061. Epub 2023 Sep 12.
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.
儿童自身免疫性神经精神障碍与链球菌感染相关（PANDAS）是在 A 组链球菌感染后急性发作或神经精神症状恶化。它被认为是对链球菌感染的自身免疫反应的结果，但没有足够的证据完全支持这一理论。尽管这种疾病主要被认为是一种儿童疾病，但也有报道称成年人也会发病。PANDAS 是一种明确的临床实体，但这种疾病的神经病理学尚未确定。我们描述了一名 26 岁女性被诊断为 PANDAS 的临床过程。她自杀了，她的大脑被生物银行用于进一步研究。我们检查了储存的组织，并进行了特殊染色、免疫组织化学和免疫荧光分析，以表征这种疾病的神经病理学。颞叶、海马和基底节的组织学显示轻度神经胶质增生和阿尔茨海默病 II 型星形胶质细胞。海马 CA1 和 CA2 区可见急性缺氧缺血性改变。免疫组化显示基底节实质/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。这些发现表明 CD4 和 CD25 阳性 T 细胞可能在理解这种疾病的神经炎症和发病机制方面发挥重要作用。该病例代表了 PANDAS 的首例神经病理学评估报告。
