Department of Neurology, University Hospital Schleswig-Holstein, Campus Kiel and Kiel University, Kiel, Germany.
Sleep Unit, Neurology Service, Hospital Clínic Barcelona, Barcelona University, IDIBAPS, CIBERNED, Barcelona, Spain.
J Parkinsons Dis. 2024;14(s2):S323-S331. doi: 10.3233/JPD-230429.
Synucleinopathies are disorders characterized by the aggregation and deposition of pathological α-synuclein conformers. The underlying neurodegenerative processes begin years or decades before the onset of cardinal motor symptoms. This prodromal phase may manifest with various signs or symptoms. However, there are no current standardized laboratory tests to ascertain the progression and conversion of prodromal conditions such as mild cognitive impairment, isolated REM sleep behavior disorder or pure autonomic failure. The aim of this systematic review was to evaluate the diagnostic possibilities using human biofluids as source material to detect pathological α-synuclein in the prodromal phase of synucleinopathies. Our review identified eight eligible studies, that investigated pathological α-synuclein conformers using cerebrospinal fluid from patients with prodromal signs of synulceinopathies to differentiate this patient group from non-synucleinopathies, while only one study investigated this aspect using blood as medium. While previous studies clearly demonstrated a high diagnostic performance of α-synuclein seed amplification assays for differentiating synucleinopathies with Lewy bodies from healthy controls, only few analyses were performed focussing on individuals with prodromal disease. Nevertheless, results for the early detection of α-synuclein seeds using α-synuclein seed amplification assays were promising and may be of particular relevance for future clinical trials and clinical practice.
突触核蛋白病是以病理性α-突触核蛋白聚集和沉积为特征的疾病。潜在的神经退行性过程在主要运动症状出现前数年或数十年开始。这个前驱期可能表现为各种迹象或症状。然而,目前没有标准化的实验室测试来确定前驱期疾病(如轻度认知障碍、孤立性 REM 睡眠行为障碍或单纯自主神经衰竭)的进展和转化。本系统评价的目的是评估使用人体生物液作为检测材料在突触核蛋白病前驱期检测病理性α-突触核蛋白的诊断可能性。我们的综述确定了八项符合条件的研究,这些研究使用脑脊液检测突触核蛋白病前驱期的病理性α-突触核蛋白,以将该患者组与非突触核蛋白病区分开来,而只有一项研究使用血液作为媒介研究了这一方面。虽然先前的研究清楚地表明α-突触核蛋白种子扩增检测在区分具有路易体的突触核蛋白病与健康对照组方面具有较高的诊断性能,但只有少数分析针对前驱期疾病患者进行。然而,使用α-突触核蛋白种子扩增检测早期检测α-突触核蛋白种子的结果是有希望的,可能对未来的临床试验和临床实践具有特别重要的意义。