Yokota Osamu, Tsuchiya Kuniaki, Uchihara Toshiki, Ujike Hiroshi, Terada Seishi, Takahashi Mafuyu, Kimura Yuji, Ishizu Hideki, Akiyama Haruhiko, Kuroda Shigetoshi
Department of Neuropsychiatry, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Okayama 700-8558, Japan.
Neuropathology. 2007 Feb;27(1):21-35. doi: 10.1111/j.1440-1789.2006.00736.x.
Lewy bodies (LB) usually extend from the brainstem to the cerebrum in patients with Parkinson's disease. However, whether the patterns of progression of LB and neuronal loss in Parkinson's disease are identical to those in other Lewy body diseases (LBD) remains unclear. In addition, pathological data on the autonomic nervous system involvement in LBD are limited. We present here the clinicopathological characteristics of two autopsy cases with both Alzheimer's disease and dementia with Lewy bodies (DLB), possibly diagnosed as having Lewy body variant of Alzheimer's disease (LBV/AD). Our patients presented clinically with dementia without parkinsonism. Histopathologically, phosphorylated alpha-synuclein-positive LB and Lewy neurites were abundant in the limbic system, especially in the amygdala, and to a lesser degree, in the neocortex, including the primary motor cortex. The amygdala was also most severely affected by neuronal loss, and the other limbic areas and neocortex were affected to a lesser degree. Despite the existence of a small number of LB and many Lewy neurites, neurons in the brainstem nuclei were relatively well preserved. The Braak stages of concurrent neurofibrillary changes and senile plaques were stage V and C, respectively, in both cases. Tyrosine hydroxylase-positive nerve fibers were relatively well spared in one case examined compared with Parkinson's disease cases. Furthermore, many Lewy neurites immunopositive for phosphorylated a-synuclein were found in the nerve fascicles of the epicardium in one case examined and in Parkinson's disease cases to a lesser degree. These findings suggest that: (i) in at least some LBV/AD cases, the amygdala develops neuronal loss and Lewy-related pathology prior to the brainstem nuclei; and (ii) the depletion of nerves in the heart tissue of LBV/AD is not necessarily complete despite the development of Lewy-related pathology.
路易小体（LB）在帕金森病患者中通常从脑干延伸至大脑。然而，帕金森病中路易小体和神经元丢失的进展模式是否与其他路易体病（LBD）相同仍不清楚。此外，关于自主神经系统受累于路易体病的病理数据有限。我们在此呈现两例尸检病例的临床病理特征，这两例患者同时患有阿尔茨海默病和路易体痴呆（DLB），可能被诊断为阿尔茨海默病路易体变异型（LBV/AD）。我们的患者临床上表现为无帕金森综合征的痴呆。组织病理学上，磷酸化α-突触核蛋白阳性的路易小体和路易神经突在边缘系统丰富，尤其是在杏仁核，在新皮层包括初级运动皮层程度较轻。杏仁核也受神经元丢失影响最严重，其他边缘区域和新皮层受影响程度较轻。尽管存在少量路易小体和许多路易神经突，但脑干核中的神经元相对保存良好。两例患者中并发神经原纤维缠结和老年斑的Braak分期分别为V期和C期。与帕金森病病例相比，在一例检查病例中酪氨酸羟化酶阳性神经纤维相对保存良好。此外，在一例检查病例的心外膜神经束中发现许多对磷酸化α-突触核蛋白免疫阳性的路易神经突，在帕金森病病例中程度较轻。这些发现表明：（i）在至少一些LBV/AD病例中，杏仁核在脑干核之前发生神经元丢失和路易体相关病理改变；（ii）尽管发生了路易体相关病理改变，但LBV/AD心脏组织中的神经消耗不一定完全。
Zotero 插件