当前位置：
X-MOL 学术
›
Ann. Indian Acad. Neurol.
›
论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
Clinical spectrum, therapeutic outcomes and prognostic predictors in paraneoplastic neurological syndromes – Experiences from a tertiary care center in India
Annals of Indian Academy of Neurology ( IF 1.7 ) Pub Date : 2021-01-01 , DOI: 10.4103/aian.aian_975_20 Asish Vijayaraghavan 1 , Pullumpallil Thomas Alexander 1 , Aditya Vijayakrishnan Nair 1 , Ajith Sivadasan 1 , Arun Mathai Mani 1 , Donna Mathew 1 , Atif Shaikh 1 , Rohit Ninan Benjamin 1 , A T Prabhakar 1 , John Jude 2 , Sunithi Mani 2 , Sanjith Aaron 1 , Vivek Mathew 1 , Mathew Alexander 1
Background: Paraneoplastic Neurological Syndromes (PNSs) are a heterogeneous group of immune-mediated disorders that often precede tumor diagnosis. There are few systematic studies on the spectrum and follow-up of PNSs. Objective: To analyze the clinical spectrum, associated tumors, antibody profile, outcomes, and prognostic predictors in a cohort of PNSs admitted in a tertiary care center. Methods: This retrospective study included 97 patients (2008-2019). PNSs were further classified as “classical,” “nonclassical,” “definite,” and “possible.” Clinical profile, diagnostic strategies, therapeutic options, and predictors of outcomes were identified. Results: The median age was 54 years (range 17–81). Thirty-nine (40.2%) had classical PNS, and 58 (59.8%) had nonclassical PNS, 74 (76.3%) had “Definite” PNS while 23 (23.7%) had “Possible” PNS. Cerebellar degeneration, peripheral neuropathy, and encephalopathy were the three most common neurological syndromes. Tumors were diagnosed in 66 (68%) patients; Lung cancer was the most common primary tumor. Antibodies were positive in 52 (53.6%). Anti-Yo antibody and anti-Ma2 antibody were the most common antibodies. The majority (57.7%) received immunotherapy in addition to definitive treatment for the tumor. A good outcome was seen in 53 (54.6%). Factors associated with good outcome were: early diagnosis, mRS <3 at presentation, absence of metastatic disease, and adjuvant immunotherapy. Conclusion: A high index of clinical suspicion is essential for early diagnosis and prompt management of PNS, especially the nonclassical syndromes. Multimodality diagnostic imaging techniques and antibody profiling play a crucial role in the diagnosis. A favorable prognosis can be expected with the judicious use of immunotherapy and definitive treatment of malignancy.
中文翻译：
副肿瘤性神经系统综合征的临床谱、治疗结果和预后预测因素——印度三级护理中心的经验
背景：副肿瘤性神经综合征 (PNS) 是一组异质的免疫介导疾病，通常先于肿瘤诊断。关于 PNS 的谱系和随访的系统研究较少。目的：分析三级医疗中心收治的一组 PNS 的临床谱、相关肿瘤、抗体谱、结果和预后预测因子。方法：这项回顾性研究包括 97 名患者（2008-2019 年）。PNS 进一步分为“经典”、“非经典”、“确定”和“可能”。确定了临床概况、诊断策略、治疗选择和结果预测因素。结果：中位年龄为 54 岁（范围 17-81）。39 人 (40.2%) 患有经典 PNS，58 人 (59.8%) 患有非经典 PNS，74 人 (76.3%) 患有“确定”PNS，而 23 人 (23.7%) 患有“可能”PNS。小脑变性、周围神经病变和脑病是三种最常见的神经系统综合征。66 名 (68%) 患者被诊断出肿瘤；肺癌是最常见的原发性肿瘤。52 例（53.6%）抗体呈阳性。抗Yo抗体和抗Ma2抗体是最常见的抗体。大多数人（57.7%）除了对肿瘤进行根治性治疗外，还接受了免疫治疗。53 人（54.6%）取得了良好的结果。与良好结果相关的因素是：早期诊断、就诊时 mRS <3、无转移性疾病和辅助免疫治疗。结论：临床怀疑的高指数对于 PNS 的早期诊断和及时管理至关重要，尤其是非经典综合征。多模态诊断成像技术和抗体分析在诊断中起着至关重要的作用。通过明智地使用免疫疗法和对恶性肿瘤的明确治疗，可以预期良好的预后。
更新日期：2021-02-16
Annals of Indian Academy of Neurology ( IF 1.7 ) Pub Date : 2021-01-01 , DOI: 10.4103/aian.aian_975_20 Asish Vijayaraghavan 1 , Pullumpallil Thomas Alexander 1 , Aditya Vijayakrishnan Nair 1 , Ajith Sivadasan 1 , Arun Mathai Mani 1 , Donna Mathew 1 , Atif Shaikh 1 , Rohit Ninan Benjamin 1 , A T Prabhakar 1 , John Jude 2 , Sunithi Mani 2 , Sanjith Aaron 1 , Vivek Mathew 1 , Mathew Alexander 1
Affiliation
Background: Paraneoplastic Neurological Syndromes (PNSs) are a heterogeneous group of immune-mediated disorders that often precede tumor diagnosis. There are few systematic studies on the spectrum and follow-up of PNSs. Objective: To analyze the clinical spectrum, associated tumors, antibody profile, outcomes, and prognostic predictors in a cohort of PNSs admitted in a tertiary care center. Methods: This retrospective study included 97 patients (2008-2019). PNSs were further classified as “classical,” “nonclassical,” “definite,” and “possible.” Clinical profile, diagnostic strategies, therapeutic options, and predictors of outcomes were identified. Results: The median age was 54 years (range 17–81). Thirty-nine (40.2%) had classical PNS, and 58 (59.8%) had nonclassical PNS, 74 (76.3%) had “Definite” PNS while 23 (23.7%) had “Possible” PNS. Cerebellar degeneration, peripheral neuropathy, and encephalopathy were the three most common neurological syndromes. Tumors were diagnosed in 66 (68%) patients; Lung cancer was the most common primary tumor. Antibodies were positive in 52 (53.6%). Anti-Yo antibody and anti-Ma2 antibody were the most common antibodies. The majority (57.7%) received immunotherapy in addition to definitive treatment for the tumor. A good outcome was seen in 53 (54.6%). Factors associated with good outcome were: early diagnosis, mRS <3 at presentation, absence of metastatic disease, and adjuvant immunotherapy. Conclusion: A high index of clinical suspicion is essential for early diagnosis and prompt management of PNS, especially the nonclassical syndromes. Multimodality diagnostic imaging techniques and antibody profiling play a crucial role in the diagnosis. A favorable prognosis can be expected with the judicious use of immunotherapy and definitive treatment of malignancy.
中文翻译：
副肿瘤性神经系统综合征的临床谱、治疗结果和预后预测因素——印度三级护理中心的经验
背景：副肿瘤性神经综合征 (PNS) 是一组异质的免疫介导疾病，通常先于肿瘤诊断。关于 PNS 的谱系和随访的系统研究较少。目的：分析三级医疗中心收治的一组 PNS 的临床谱、相关肿瘤、抗体谱、结果和预后预测因子。方法：这项回顾性研究包括 97 名患者（2008-2019 年）。PNS 进一步分为“经典”、“非经典”、“确定”和“可能”。确定了临床概况、诊断策略、治疗选择和结果预测因素。结果：中位年龄为 54 岁（范围 17-81）。39 人 (40.2%) 患有经典 PNS，58 人 (59.8%) 患有非经典 PNS，74 人 (76.3%) 患有“确定”PNS，而 23 人 (23.7%) 患有“可能”PNS。小脑变性、周围神经病变和脑病是三种最常见的神经系统综合征。66 名 (68%) 患者被诊断出肿瘤；肺癌是最常见的原发性肿瘤。52 例（53.6%）抗体呈阳性。抗Yo抗体和抗Ma2抗体是最常见的抗体。大多数人（57.7%）除了对肿瘤进行根治性治疗外，还接受了免疫治疗。53 人（54.6%）取得了良好的结果。与良好结果相关的因素是：早期诊断、就诊时 mRS <3、无转移性疾病和辅助免疫治疗。结论：临床怀疑的高指数对于 PNS 的早期诊断和及时管理至关重要，尤其是非经典综合征。多模态诊断成像技术和抗体分析在诊断中起着至关重要的作用。通过明智地使用免疫疗法和对恶性肿瘤的明确治疗，可以预期良好的预后。
京公网安备 11010802027423号