Although colocalization of amyloid β (Aβ) with prion protein (PrP) in the kuru plaque has previously been observed in the brain of prion diseases patients, the participating Aβ species has not been identified. Here, we present an immunohistochemical assessment of the brain and spinal cord of a 69-year-old Japanese female patient with Gerstmann-Sträussler-Scheinker disease with a P105L mutation on the PRNP gene (GSS-P105L). Immunohistochemical assessment of serial brain sections was performed using anti-PrP and -Aβ antibodies in the hippocampus, frontal and occipital lobes. She died 69 years after a 21-year clinical course. Immunohistochemistorical examination revealed that ~50% of the kuru plaques in the cerebrum were colocalized with Aβ, and Aβ42 was predominantly observed to be colocalized with PrP-plaques. The Aβ deposition patterns were unique, and distinct from diffuse plaques observed in the normal aging brain or Alzheimer’s disease brain. The spinal cord exhibited degeneration in the lateral corticospinal tract, posterior horn, and fasciculus gracilis. We have demonstrated for the first time that Aβ42, rather than Aβ40, is the main Aβ component associated with PrP-plaques, and also the degeneration of the fasciculus gracilis in the spinal cord in GSS-P105L, which could be associated with specific clinical features of GSS-P105L.

尽管先前已经在pr病毒病患者的大脑中观察到了淀粉样蛋白β（Aβ）与病毒蛋白（PrP）在库鲁斑中的共定位，但尚未鉴定出参与的Aβ物种。在这里，我们介绍了一名69岁的Gerstmann-Sträussler-Scheinker病，PRNP突变为P105L的日本女性患者的大脑和脊髓的免疫组织化学评估基因（GSS-P105L）。在海马，额叶和枕叶中使用抗PrP和-Aβ抗体进行了连续脑切片的免疫组织化学评估。经过21年的临床治疗，她去世了69年。免疫组织化学检查显示，大脑中约50％的库鲁斑块与Aβ共定位，并且主要观察到Aβ42与PrP斑共定位。Aβ沉积模式是独特的，并且与在正常衰老的大脑或阿尔茨海默氏病大脑中观察到的弥散性斑块不同。脊髓在外侧皮质脊髓束，后角和束状筋膜中表现出变性。我们首次证明，Aβ42而不是Aβ40是与PrP斑块相关的主要Aβ成分，