Mori Hideo, Oda Masaya, Komori Takashi, Arai Nobutaka, Takanashi Masashi, Mizutani Toshio, Hirai Shunnichi, Mizuno Yosikuni
Department of Neurology, Juntendo University Scvhool of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113, Japan.
Acta Neuropathol. 2002 Sep;104(3):273-8. doi: 10.1007/s00401-002-0555-3. Epub 2002 May 4.
Lewy bodies (LBs), whose major component is alpha-synuclein, are a pathological hallmark of Parkinson's disease (PD) but have rarely been reported in progressive supranuclear palsy (PSP). Whether LBs in PSP represent the aging process or the coexistence of PD remains unclear. We found LBs in 5 of 16 patients with PSP. In 4 patients LBs were distributed widely throughout the brain stem and cerebrum in a pattern similar to that in PD. In the remaining patient one LB was found in the pontine reticular formation. Semiquantitative analysis showed that neuronal loss in the locus coeruleus and the dorsal vagal nucleus was more severe in patients with LBs than in patients without LBs. Double-labeling immunohistochemical studies showed co-localization of alpha-synuclein and tau in some neurons. Our study suggests that patients who have PSP with LBs constitute a subset of patients with PSP in whom Lewy body disease is also present.
路易小体（LBs）的主要成分是α-突触核蛋白，是帕金森病（PD）的病理标志，但在进行性核上性麻痹（PSP）中鲜有报道。PSP中的路易小体是代表衰老过程还是与PD共存仍不清楚。我们在16例PSP患者中的5例发现了路易小体。在4例患者中，路易小体广泛分布于脑干和大脑，其分布模式与PD相似。在其余1例患者中，在脑桥网状结构中发现了1个路易小体。半定量分析显示，存在路易小体的患者中蓝斑和迷走神经背核的神经元丢失比没有路易小体的患者更严重。双标免疫组化研究显示，α-突触核蛋白和tau在一些神经元中共定位。我们的研究表明，伴有路易小体的PSP患者构成了同时存在路易体病的PSP患者亚组。
