Department of Physiology, Genetics and Microbiology, University of Alicante, Spain.
Banner Sun Health Research Institute, Sun City, Arizona, USA.
Mov Disord. 2018 Aug;33(8):1315-1324. doi: 10.1002/mds.27392. Epub 2018 May 8.
PD patients often have visual alterations, for example, loss of visual acuity, contrast sensitivity or motion perception, and diminished electroretinogram responses. PD pathology is mainly characterized by the accumulation of pathological α-synuclein deposits in the brain, but little is known about how synucleinopathy affects the retina.
To study the correlation between α-synuclein deposits in the retina and brain of autopsied subjects with PD and incidental Lewy body disease.
We evaluated the presence of phosphorylated α-synuclein in the retina of autopsied subjects with PD (9 subjects), incidental Lewy body disease (4 subjects), and controls (6 subjects) by immunohistochemistry and compared the retinal synucleinopathy with brain disease severity indicators.
Whereas controls did not show any phosphorylated α-synuclein immunoreactivity in their retina, all PD subjects and 3 of 4 incidental Lewy body disease subjects had phosphorylated α-synuclein deposits in ganglion cell perikarya, dendrites, and axons, some of them resembling brain Lewy bodies and Lewy neurites. The Lewy-type synucleinopathy density in the retina significantly correlated with Lewy-type synucleinopathy density in the brain, with the Unified Parkinson's disease pathology stage and with the motor UPDRS.
These data suggest that phosphorylated α-synuclein accumulates in the retina in parallel with that in the brain, including in early stages preceding development of clinical signs of parkinsonism or dementia. Therefore, the retina may provide an in vivo indicator of brain pathology severity, and its detection could help in the diagnosis and monitoring of disease progression. © 2018 International Parkinson and Movement Disorder Society.
PD 患者常出现视觉改变，例如视力下降、对比敏感度或运动知觉下降，以及视网膜电图反应减弱。PD 病理学主要表现为脑内病理性 α-突触核蛋白沉积，但对于突触核蛋白病如何影响视网膜知之甚少。
研究尸检 PD 患者和偶发路易体病患者脑内 α-突触核蛋白沉积与视网膜之间的相关性。
我们通过免疫组织化学方法评估了尸检 PD 患者（9 例）、偶发路易体病患者（4 例）和对照组（6 例）视网膜中磷酸化 α-突触核蛋白的存在，并将视网膜突触核蛋白病与脑疾病严重程度指标进行了比较。
对照组视网膜中未显示任何磷酸化 α-突触核蛋白免疫反应性，而所有 PD 患者和 4 例偶发路易体病患者中有 3 例在神经节细胞胞体、树突和轴突中出现磷酸化 α-突触核蛋白沉积，其中一些类似于脑路易体和路易神经突。视网膜中的 Lewy 型突触核蛋白病密度与脑内 Lewy 型突触核蛋白病密度、帕金森病统一病理分期和运动 UPDRS 显著相关。
这些数据表明，磷酸化 α-突触核蛋白在视网膜中的积累与脑内的积累平行，包括在出现帕金森病或痴呆的临床症状之前的早期阶段。因此，视网膜可能提供脑病理学严重程度的体内指标，其检测有助于诊断和监测疾病进展。
