Department of Neurology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, China.
Department of Neurology, Suzhou Ninth People's Hospital, Suzhou, 215200, China.
Neurosci Lett. 2021 Jun 21;756:135964. doi: 10.1016/j.neulet.2021.135964. Epub 2021 May 19.
Differential diagnosis of Parkinson's disease (PD), multiple system atrophy (MSA) and progressive supranuclear paralysis (PSP) is challenging. This study aimed to investigate the expression of phosphorylated α-synuclein (p-α-syn) and phosphorylated tau-protein (p-tau) in sural nerves from patients with PD, MSA and PSP to find biomarkers for differential diagnosis. Clinical evaluations and sural nerve biopsies were performed on 8 PD patients, 8 MSA patients, 6 PSP patients and 8 controls (CTRs). Toluidine blue staining was used to observe morphological changes in sural nerves. The deposition of p-α-syn and p-tau was detected by immunohistochemistry with semiquantitative evaluation. Locations of p-α-syn and p-tau were identified by double immunofluorescent staining. In case groups, the density of nerve fibres decreased with swollen or fragmented Schwann cells (SCs). All cases (22/22) but no CTRs (0/8) presented p-α-syn immunoreactivity with gradually decreasing semiquantitative levels among the PD (6.00 ± 2.07), MSA (5.00 ± 2.33) and PSP (3.50 ± 1.52) groups. p-tau aggregates were found in 7/8 MSA (1.88 ± 1.46) and 6/6 PSP (1.67 ± 0.52) patients but not in PD patients or CTRs. There were different expression patterns of p-α-syn and p-tau in PD, MSA and PSP patients. These findings suggest that peripheral sensory nerve injury exists in PD, MSA and PSP patients. With a different expression pattern and level, p-α-syn and p-tau in sural nerves may serve as novel biomarkers for differential diagnosis of PD, MSA and PSP.
帕金森病(PD)、多系统萎缩(MSA)和进行性核上性麻痹(PSP)的鉴别诊断具有挑战性。本研究旨在探讨磷酸化α-突触核蛋白(p-α-syn)和磷酸化tau 蛋白(p-tau)在 PD、MSA 和 PSP 患者的腓肠神经中的表达,寻找用于鉴别诊断的生物标志物。对 8 例 PD 患者、8 例 MSA 患者、6 例 PSP 患者和 8 例对照者(CTR)进行临床评估和腓肠神经活检。甲苯胺蓝染色观察腓肠神经的形态学变化。免疫组织化学检测 p-α-syn 和 p-tau 的沉积,并进行半定量评价。通过双重免疫荧光染色确定 p-α-syn 和 p-tau 的定位。在病例组中,神经纤维密度降低,施万细胞(SCs)肿胀或断裂。所有病例(22/22)而非对照者(0/8)均存在 p-α-syn 免疫反应性,PD(6.00±2.07)、MSA(5.00±2.33)和 PSP(3.50±1.52)组的半定量水平逐渐降低。7/8 例 MSA(1.88±1.46)和 6/6 例 PSP(1.67±0.52)患者存在 p-tau 聚集,但 PD 患者和对照者均未发现。PD、MSA 和 PSP 患者存在 p-α-syn 和 p-tau 的不同表达模式。这些发现提示 PD、MSA 和 PSP 患者存在周围感觉神经损伤。腓肠神经中的 p-α-syn 和 p-tau 表达模式和水平不同,可能作为 PD、MSA 和 PSP 鉴别诊断的新生物标志物。