Vinters H V, Hudson A J, Kaufmann J C
Ann Neurol. 1986 Oct;20(4):540-3. doi: 10.1002/ana.410200418.
Postmortem neuropathological findings in a patient with biopsy-proved familial Gerstmann-Sträussler-Scheinker disease of eight years' duration included severe spongy change in the neocortex, extensive and often large amyloid deposits throughout the cerebral hemispheres and cerebellum, and severe astrocytic gliosis throughout all areas of gray and white matter within the brain. The degree of cortical spongy change was much greater than that in relatives who died with a similar clinical history, indicating the phenotypic heterogeneity in this familial disorder.
一名经活检证实患有病程八年的家族性格斯特曼-施特劳斯勒-谢inker病患者的尸检神经病理学发现包括新皮质严重海绵状改变、大脑半球和小脑广泛且常为大量的淀粉样蛋白沉积，以及脑内所有灰质和白质区域严重的星形细胞胶质增生。皮质海绵状改变的程度远大于有类似临床病史而死亡的亲属，表明这种家族性疾病存在表型异质性。
