Kobayashi Atsushi, Arima Kunimasa, Ogawa Masafumi, Murata Miho, Fukuda Takahiro, Kitamoto Tetsuyuki
Division of CJD Science and Technology, Department of Prion Research, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, 980-8575, Japan.
Acta Neuropathol. 2008 Nov;116(5):561-6. doi: 10.1007/s00401-008-0425-8. Epub 2008 Aug 28.
Plaque-type deposition of prion protein (PrP) in the brain has been extremely rare in sporadic Creutzfeldt-Jakob disease patients with methionine homozygosity at polymorphic codon 129 of the PrP gene and type 1 abnormal isoform of PrP (sCJD-MM1). Here we report three sCJD-MM1 patients who showed prominent PrP-positive amyloid plaques in the cerebral and cerebellar white matter. All three patients showed clinical courses of long duration (2 years < or =), particularly at the end-stage. The white matter of these patients was severely damaged because of the prolonged disease duration. Furthermore, Alzheimer's amyloid precursor protein, which accumulates within the axonal swellings under pathological conditions, co-accumulated with the PrP-amyloid plaques. These findings suggest that the axonal damage reflecting the prolonged disease duration causes the deposition of PrP-amyloid plaques in the white matter. The present study shows that PrP-amyloid plaques can occur in the white matter of sCJD-MM1 cases.
在朊蛋白（PrP）基因多态密码子129处为甲硫氨酸纯合子且PrP为1型异常异构体的散发性克雅氏病（sCJD-MM1）患者中，脑内出现斑块型朊蛋白沉积极为罕见。在此，我们报告了3例sCJD-MM1患者，其大脑和小脑白质中出现了显著的PrP阳性淀粉样斑块。所有3例患者病程均较长（2年及以上），尤其是在疾病末期。由于病程延长，这些患者的白质严重受损。此外，在病理条件下积聚于轴突肿胀内的阿尔茨海默病淀粉样前体蛋白与PrP淀粉样斑块共同积聚。这些发现提示，反映病程延长的轴突损伤导致了PrP淀粉样斑块在白质中的沉积。本研究表明，PrP淀粉样斑块可出现在sCJD-MM1病例的白质中。
