■虽然多系统萎缩(MSA)表现出高度异质性的运动和非运动症状,临床表型与预后之间的关联尚不清楚.
■我们旨在使用数据驱动的方法评估MSA的临床表型,并测量表型对生存和卧床状态的影响。
■从首都医科大学宣武医院招募了193名MSA患者,谁的历史,运动和非运动症状采用聚类分析进行检查.95名参与者在平均31.87个月后通过电话进行了随访。我们采用Kaplan-Meier分析来检查生存率,并进行Cox和logistic回归分析来确定与生存率和卧床状态相关的因素。
■我们确定了MSA的四种临床特征:以小脑症状为主,睡眠和情绪障碍占主导地位,刚性运动占优势,和恶性弥漫性。总中位生存期为7.75年(95%CI7.19-8.31)。在从症状发作到诊断多年后,年龄和性别,恶性弥漫性和刚性运动障碍占优势的集群患者的死亡风险高于睡眠和心境障碍占优势的集群.此外,恶性弥漫性和僵硬的运动障碍占优势的簇患者卧床的风险高于小脑症状占优势的簇.
■除了两个经典亚型外,还确定了恶性弥漫性和睡眠和情绪障碍为主,帕金森病,和小脑症状变异。通常,具有刚性运动障碍的患者的预后要比小脑症状为主的患者差。弥漫性症状,尤其是姿势不稳定,和诊断时的认知改变,表明快速的功能丧失和疾病进展。不同的概况和预后可能表明各种潜在的病理机制。
多系统萎缩(MSA)是一种复杂的疾病,可以影响患者的运动和非运动功能。然而,我们不太了解这些不同的症状与患者的健康状况如何随着时间的推移而改变。在这项研究中,我们对193例MSA患者进行了研究,以进一步了解这些患者在诊断时是否可以分为不同的亚组,以及这些亚组是否可能与他们的生存率和未来的移动能力相关.我们发现了四个主要的亚组患者:以小脑(大脑的一部分)功能障碍为特征的第1组,第二组以睡眠和情绪问题为特征,第3组的特点是僵硬和缓慢的运动,第4组有上述弥漫性症状。在追踪95名患者近32个月后,我们发现那些以僵硬和缓慢运动为特征的,与那些以睡眠和情绪问题为特征的人相比,那些有弥漫性症状的人死亡的可能性更高。第3组和第4组也有更高的机会变得无法下床。这表明,患有严重僵硬症状和诊断缓慢的患者的前景往往比没有的患者差。如果在诊断患者时发现多种MSA症状,尤其是思考的麻烦,也是疾病正在迅速恶化的迹象。通过了解这些疾病模式,我们可以更好地定制治疗方法,并为MSA患者提供更好的支持。
UNASSIGNED: While multiple system atrophy (MSA) presents with high heterogeneous motor and nonmotor symptoms, the associations between clinical phenotypes and prognosis are unclear.
UNASSIGNED: We aimed to evaluate clinical phenotypes of MSA using data-driven approach and measure the impact of phenotypes on survival and bedbound status.
UNASSIGNED: 193 MSA patients were recruited from Xuanwu Hospital Capital Medical University, whose history, motor and non-motor symptoms were examined using cluster analysis. Ninety-five participants were followed-up via telephone after a mean of 31.87 months. We employed Kaplan- Meier analysis to examine survival and performed Cox and logistic regression analyses to identify factors associated with survival and bedbound status.
UNASSIGNED: We identified four clinical profiles of MSA: cerebellar symptom-dominant, sleep and mood disorder-dominant, rigid akinetic-dominant, and malignant diffuse. The overall median survival was 7.75 years (95% CI 7.19-8.31). After adjusting for years from symptom onset to diagnosis, age and sex, patients in the malignant diffuse and rigid akinetic-dominant clusters had greater risk of death than sleep and mood disorder-dominant cluster. Furthermore, patients in the malignant diffuse and rigid akinetic-dominant clusters had higher risk of being bedbound than cerebellar symptom-dominant cluster.
UNASSIGNED: The malignant diffuse and sleep and mood disorder-dominant were identified besides the two classical subtypes, parkinsonism, and cerebellar symptom-variant. Patients with rigid-akinetic motor profiles have a worse prognosis than cerebellar symptom-dominant profiles in general. Diffuse symptoms, especially postural instability, and cognitive alterations at diagnosis, indicate rapid functional loss and disease progression. The different profiles and prognoses might indicate varied underlying pathological mechanisms.
Multiple system atrophy (MSA) is a complex disease that can affect both movement and non-movement functions of patients. However, we do not know much about how these different symptoms relate to how the patient’s health might change over time. In this study, we looked at 193 MSA patients to learn more about if the patients can be distinguished into different subgroups at diagnosis and if the subgroups might be associated with their survival and ability to move in the future. We found four main subgroups of patients: group 1 characterized by the dysfunction of cerebellum (a part of the brain), group 2 characterized by sleep and mood problems, group 3 characterized by rigidity and slow movements, and group 4 with diffuse symptoms mentioned above. After tracking 95 patients for nearly 32 months, we found that those characterized by rigidity and slow movements, and those with diffuse symptoms had a higher chance of dying compared to those characterized by sleep and mood problems. Group 3 and 4 also had a higher chance of becoming unable to move out of bed. This suggests that patients with severe symptoms of rigidity and slowness at diagnosis tend to have a worse outlook than those without. And if multiple MSA symptoms are found when the patient is diagnosed, especially trouble with thinking, are also signs that the disease is getting worse quickly. By understanding these disease patterns, we can better tailor treatments and provide better support for people with MSA.