Departments of Neuropathology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo, Japan.
Neuropathology. 2013 Feb;33(1):47-58. doi: 10.1111/j.1440-1789.2012.01329.x. Epub 2012 Jun 4.
We analyzed the incidence and extent of Lewy-related α-synucleinopathy (LBAS) in the olfactory mucosa, as well as the central and peripheral nervous systems of consecutive autopsy cases from a general geriatric hospital. The brain and olfactory mucosa were immunohistochemically examined using antibodies raised against phosphorylated α-synuclein. Thirty-nine out of 105 patients (37.1%) showed LBAS in the central or peripheral nervous systems. Seven patients presented LBAS (Lewy neurites) in the olfactory lamina propria mucosa. One out of the seven cases also showed a Lewy neurite in a bundle of axons in the cribriform plate, but α-synuclein deposits were not detected in the olfactory receptor neurons. In particular, high incidence of α-synuclein immunopositive LBAS in the olfactory mucosa was present in the individuals with clinically as well as neuropathologically confirmed Parkinson's disease and dementia with Lewy bodies (6/8 cases, 75%). However, this pathologic alteration was rare in the cases with incidental or subclinical Lewy body diseases (LBD) (one out of 31 cases, 3.2%). In the olfactory bulb, the LBAS was usually present in the glomeruli and granular cells of most symptomatic and asymptomatic cases with LBD. Our studies further confirmed importance of the olfactory entry zone in propagation of LBAS in the human aging nervous system.
我们分析了连续尸检病例的嗅黏膜、中枢神经系统和周围神经系统中与 Lewy 相关的α-突触核蛋白病(LBAS)的发生率和程度。使用针对磷酸化α-突触核蛋白的抗体对大脑和嗅黏膜进行免疫组织化学检查。105 例患者中有 39 例(37.1%)在中枢或周围神经系统中出现 LBAS。7 例患者在嗅黏膜固有层中出现 LBAS(Lewy 神经突)。这 7 例中的 1 例在筛板的轴突束中也出现 Lewy 神经突,但嗅受体神经元中未检测到α-突触核蛋白沉积。特别是在临床和神经病理学上均确诊为帕金森病和路易体痴呆的患者中,嗅黏膜中α-突触核蛋白阳性 LBAS 的发生率较高(6/8 例,75%)。然而,在偶发或亚临床Lewy 体病(LBD)病例中,这种病理改变很少见(31 例中有 1 例,3.2%)。在嗅球中,LBAS 通常存在于大多数有症状和无症状的 LBD 患者的肾小球和颗粒细胞中。我们的研究进一步证实了嗅入区在人类衰老神经系统中 LBAS 传播中的重要性。