副肿瘤神经综合征 (PNS) 诊断标准于 2004 年首次提出，并于 2021 年更新。PNS-CARE 评分源自更新的标准，是一个综合模型，用于为疑似 PNS 患者分配可能性。在这项研究中，我们评估了 2021 年 PNS-CARE 评分的实用性和适用性，并介绍了我们的 PNS 队列。这是一项回顾性研究。我们确定了 Mayo Clinic 疑似患有 PNS 的患者 (1/2005-12/2020)，并收集了相关信息，包括人口统计、PNS 表现和临床结果。纳入标准如下：(1) 患有与 PNS 一致的综合征的患者；(2) 图表中有足够信息的患者。排除标准如下：(1) 仅在 2005 年之前进行评估，(2) 未经神经科评估的患者，(3) 在免疫检查点抑制剂后出现症状，(4) 未纳入 2021 年标准的综合征。所有患者均根据 2021 年和 2004 年 PNS 标准进行评估。我们在初次就诊时确定了 484 名疑似患有 PNS 的患者，其中 212 名 (44%) 在完成评估后被认为患有 PNS。在这 212 名患者中，最常见的自身抗体是 PCA1 (Yo)-IgG (17%)、KLHL11-IgG (16%) 和 CRMP5-IgG (14%)，最常见的表型是快速进展小脑综合征 (29%) ）、脑干脑炎（14%）和边缘脑炎（8%）。 2021 年 PNS 标准明确/可能分类（PNS-CARE 评分≥ 6）的敏感性和特异性分别为 93% 和 100%，而 2004 年 PNS 标准明确分类的敏感性和特异性分别为 67% 和 99% 。我们发现 15 名 PNS-CARE 评分≤5 的患者在我们的审查中可能患有 PNS。这些患者中最常见的表现是 KLHL11-IgG 脑干脑炎（7/15，47%），可能伴有睾丸肿瘤烧毁。我们的研究验证了 PNS-CARE 评分。更清楚地了解典型的三七总皂甙表现和常见的潜在恶性肿瘤和自身抗体有助于更早、更准确的诊断，这对于下游临床决策至关重要。尽管存在高风险抗体和/或潜在的恶性肿瘤，但一些具有中风险表型的患者不符合可能/明确的标准。

Paraneoplastic neurologic syndrome (PNS) diagnostic criteria were first proposed in 2004 and updated in 2021. The PNS-CARE score, derived from the updated criteria, is a composite model for assigning likelihood for patients with suspected PNS. In this study, we evaluated the utility and applicability of the 2021 PNS-CARE score and present our PNS cohort.This is a retrospective study. We identified Mayo Clinic patients suspected to have PNS (1/2005-12/2020) and collected relevant information including demographics, PNS presentation, and clinical outcomes. Inclusion criteria were the following: (1) patients with a syndrome consistent with PNS and (2) patients with sufficient information available in charts. Exclusion criteria were the following: (1) evaluation only before 2005, (2) patients not evaluated by neurology, (3) presentation after immune checkpoint inhibitors, and (4) syndromes not included in 2021 criteria. All patients were evaluated for the 2021 and 2004 PNS criteria.We identified 484 patients suspected to have PNS at initial presentation, of whom 212 (44%) were considered to have PNS after completion of evaluation. Among these 212 patients, the most common autoantibodies were PCA1 (Yo)-IgG (17%), KLHL11-IgG (16%), and CRMP5-IgG (14%) and the most common phenotypes were rapidly progressive cerebellar syndrome (29%), brainstem encephalitis (14%), and limbic encephalitis (8%). The 2021 PNS criteria definite/probable categorization (PNS-CARE score ≥ 6) had a sensitivity and specificity of 93% and 100%, respectively, while the 2004 PNS criteria definite categorization had a sensitivity and specificity of 67% and 99%, respectively. We found 15 patients with a PNS-CARE score ≤5 who likely had PNS on our review. The most common presentation among these patients was KLHL11-IgG brainstem encephalitis (7/15, 47%) with likely burned-out testicular tumor.Our study validates the PNS-CARE score. A clearer understanding of typical PNS presentation and common underlying malignancies and autoantibodies can aid in earlier and more accurate diagnosis, which is crucial for downstream clinical decisions. Some patients with an intermediate-risk phenotype do not meet probable/definite criteria despite the presence of high-risk antibodies and/or underlying malignancy.