Division of Pathology, Department of Clinical Sciences Lund, Lund University, Lund, Sweden.
J Neuropathol Exp Neurol. 2023 Feb 21;82(3):242-249. doi: 10.1093/jnen/nlad001.
The purpose of this study was to investigate the cause of death in subjects with α-synucleinopathies (ASs) and the confirmed presence of cardiac α-synuclein (α-syn), compared to non-AS disorders in a neuropathologically confirmed cohort. In total, 78 neuropathologically confirmed AS cases positive for cardiac α-syn were included in the study. Individuals with other neurocognitive diseases, having no α-syn in the brainstem or above, nor in cardiac nerves, served as controls (n = 53). Data regarding the cause of death, cardiac α-syn, pathological cardiac findings, and cardio- and cerebrovascular disease were assembled from autopsy reports and medical records. In the AS group, there was a significantly higher prevalence of sudden cardiac death ([SCD]; n = 40, 51.3%) compared to the control group (n = 12, 22.6%, p < 0.001). No statistically significant differences between the groups were reported regarding other cardiac conditions on autopsy or regarding cardio- and cerebrovascular disease from the medical records. The most prevalent cause of death in the AS group was SCD, which differed significantly from the control group. This suggests that α-syn deposits in cardiac nerves may cause lethal alterations in cardiac function, warranting further research.
本研究旨在调查在神经病理学确认的队列中,与非α-突触核蛋白病(AS)相比,α-突触核蛋白(α-syn)阳性的 AS 患者的死亡原因。本研究共纳入 78 例经病理学证实存在心脏α-syn 的 AS 病例。以无脑干或以上脑内 α-syn 且心脏神经中无 α-syn 的其他神经认知疾病患者作为对照组(n=53)。从尸检报告和病历中收集了有关死亡原因、心脏α-syn、病理性心脏发现以及心脑血管疾病的数据。在 AS 组中,突然心脏死亡(SCD;n=40,51.3%)的发生率明显高于对照组(n=12,22.6%,p<0.001)。尸检时两组其他心脏情况或病历中的心脑血管疾病无统计学差异。AS 组最常见的死亡原因是 SCD,与对照组有显著差异。这表明心脏神经中的α-syn 沉积可能导致心脏功能的致命改变,需要进一步研究。