Department of Neuroscience, Mayo Clinic, Jacksonville, Florida 32224, USA.
Mov Disord. 2010;25 Suppl 1:S78-82. doi: 10.1002/mds.22637.
Recently, Braak and coworkers proposed a pathologic staging scheme for Parkinson disease (PD). In this staging, scheme substantia nigra pathology occurs at midstage disease, while involvement of anterior olfactory nucleus, medulla, and pontine tegmentum occur earlier. In the last stages, Lewy bodies (LBs) involve cortical areas. The general principles of the proposed staging system have been confirmed in several studies of PD, but it does not appear to fit with all LB disorders. We studied the density and distribution of LBs with alpha-synuclein immunohistochemistry in normal elderly with incidental LBs (N = 12); progressive supranuclear palsy (PSP) with incidental LBs (N = 18); Lewy body disease (LBD) with minimal or no Alzheimer type pathology (N = 52); LBD with concomitant Alzheimer disease (AD) (N = 84); and cases of AD with amygdala predominant LBs (N = 64). The proportion of cases that fit the PD staging scheme was 67% for incidental LBs; 86% for PSP with LBs; 86% for pure LBD; and 84% for LBD with AD; but only 6% for AD with amygdala predominant LBs. The PD staging scheme is valid, except in the setting of advanced AD. In this situation, LBs may be unrelated to PD and more likely related to factors inherent to AD and the selective vulnerability of the amygdala to both Alzheimer and alpha-synuclein pathologies.
最近，Braak 和同事们提出了一个帕金森病（PD）的病理分期方案。在这个分期方案中，黑质病理学发生在疾病的中期，而前嗅核、延髓和脑桥被盖的受累发生得更早。在最后阶段，路易体（LB）累及皮质区域。该分期系统的一般原则已在几项 PD 研究中得到证实，但它似乎并不适用于所有 LB 疾病。我们用 α-突触核蛋白免疫组化研究了正常老年人偶然 LB（N=12）、偶然 LB 的进行性核上性麻痹（PSP，N=18）、LB 病（LBD）伴或不伴阿尔茨海默病（AD）型病理（N=52）、AD 合并 LBD（N=84）和杏仁核优势 LB 的 AD 病例（N=64）的 LB 密度和分布。符合 PD 分期方案的病例比例为：偶然 LB 为 67%；偶然 LB 的 PSP 为 86%；纯 LBD 为 86%；AD 合并 LBD 为 84%；但仅有 6%的 AD 伴杏仁核优势 LB。PD 分期方案是有效的，除了在 AD 晚期。在这种情况下，LB 可能与 PD 无关，更可能与 AD 固有的因素以及杏仁核对阿尔茨海默病和 α-突触核蛋白病理学的选择性易感性有关。
