Prognosis of patients with parkinsonism varies greatly between the various parkinsonian syndromes. However, it is often difficult to distinguish the different forms, particularly in early stages. We examined predictors of mortality and functional outcome in patients with recent-onset parkinsonism with an initially uncertain diagnosis (n = 156). Patients were recruited between 2003 and 2006, comprehensively investigated, and followed prospectively (up to 15 years, mean 7 years). A final clinical diagnosis was established after follow-up. Independent predictors of mortality were investigated with multivariable Cox regression and combined into a simple prediction model. Model performance to predict 5- and 10-year mortality and functional outcome after 3 years was evaluated and externally validated in a second cohort of 62 patients with parkinsonism with an initially uncertain diagnosis. Ninety-one patients died (58%). Orthostatic hypotension, impaired cognition, abnormal tandem gait, and elevated neurofilament light chain concentration in serum or CSF were associated with mortality. A simple model that combined these factors showed excellent performance for prediction of functional outcome after 3 years and mortality after 5 and 10 years (c-statistic ~0.90 for all models). Model performance was confirmed after external validation: prediction of functional outcome after 3 years (c-statistic 0.89, 95% CI 0.80–0.98) and mortality after 5 years (c-statistic 0.91, 95% CI 0.84–0.99) were comparable to the results in the discovery cohort. These findings help clinicians to estimate a patient’s prognosis, irrespective of the specific diagnosis.

帕金森综合征患者的预后在各种帕金森综合征之间差异很大。然而，通常很难区分不同的形式，特别是在早期阶段。我们检查了新发帕金森病患者的死亡率和功能结果的预测因素，这些患者最初的诊断不确定（n = 156)。患者在 2003 年至 2006 年间被招募，进行了全面调查，并进行了前瞻性随访（最长 15 年，平均 7 年）。随访后确定最终临床诊断。使用多变量 Cox 回归研究了死亡率的独立预测因素，并将其组合成一个简单的预测模型。在第二组 62 名最初诊断不明确的帕金森病患者中评估和外部验证了预测 5 年和 10 年死亡率和 3 年后功能结果的模型性能。91 名患者死亡（58%）。体位性低血压、认知障碍、串联步态异常以及血清或脑脊液中神经丝轻链浓度升高与死亡率相关。所有模型的c -statistic ~0.90）。模型性能在外部验证后得到证实：对 3 年后功能结果的预测（c -statistic 0.89, 95% CI 0.80–0.98）和 5 年后死亡率（c -statistic 0.91, 95% CI 0.84–0.99）与结果发现队列。无论具体诊断如何，这些发现都有助于临床医生估计患者的预后。