Melanis Konstantinos, Stefanou Maria-Ioanna, Kitsos Dimitrios K, Athanasaki Athanasia, Theodorou Aikaterini, Koropouli Eleftheria, Keramida Anna, Dimitriadou Evangelia Makrina, Tzanetakos Dimitrios, Andreadou Elizabeth, Koutroulou Ioanna, Giannopoulos Sotirios, Paraskevas George P, Tsivgoulis Georgios, Tzartos John S
Second Department of Neurology, "Attikon" University Hospital, School of Medicine, National and Kapodistrian University of Athens, 12462 Athens, Greece.
First Department of Neurology, "Eginition" University Hospital, School of Medicine, National & Kapodistiran University of Athens, 12462 Athens, Greece.
J Clin Med. 2024 Jan 31;13(3):824. doi: 10.3390/jcm13030824.
Paraneoplastic Neurological Syndromes (PNS) comprise a diverse group of disorders propagated by immune-mediated effects of malignant tumors on neural tissue.
A single-center longitudinal study was performed including consecutive adult patients treated at a tertiary academic hospital between 2015 and 2023 and diagnosed with PNS. PNS were ascertained using the 2004 and the revised 2021 PNS-Care diagnostic criteria.
Thirteen patients who fulfilled the 2004 definite PNS criteria were included. PNS comprise diverse neurological syndromes, with neuromuscular junction disorders (54%) and limbic encephalitis (31%) being predominant. PNS-related antibodies were detected in 85% of cases, including anti-AChR ( = 4), anti-P/Q-VGCC ( = 3), anti-Hu ( = 3), anti-Yo ( = 1), anti-Ma ( = 1), anti-titin ( = 1), anti-IgLON5 ( = 1), and anti-GAD65 ( = 1). Thymoma (31%), small-cell lung cancer (23%), and papillary thyroid carcinoma (18%) were the most frequent tumors. Imaging abnormalities were evident in 33% of cases. Early immunotherapy within 4-weeks from symptom onset was associated with favorable outcomes. At a mean follow-up of 2 ± 1 years, two patients with anti-Hu and anti-Yo antibodies died (18%). Four and three patients fulfilled the 2021 PNS-Care diagnostic criteria for definite and probable PNS, respectively.
This study highlights the clinical heterogeneity of PNS, emphasizing the need for early suspicion and prompt treatment initiation for optimal outcomes.
副肿瘤性神经系统综合征（PNS）是一组由恶性肿瘤对神经组织的免疫介导作用引发的多种疾病。
进行了一项单中心纵向研究，纳入2015年至2023年期间在一家三级学术医院接受治疗并被诊断为PNS的连续成年患者。使用2004年和修订后的2021年PNS-Care诊断标准确定PNS。
纳入了13例符合2004年明确PNS标准的患者。PNS包括多种神经系统综合征，以神经肌肉接头疾病（54%）和边缘叶脑炎（31%）为主。85%的病例检测到PNS相关抗体，包括抗AChR（=4）、抗P/Q-VGCC（=3）、抗Hu（=3）、抗Yo（=1）、抗Ma（=1）、抗肌联蛋白（=1）、抗IgLON5（=1）和抗GAD65（=1）。胸腺瘤（31%）、小细胞肺癌（23%）和甲状腺乳头状癌（18%）是最常见的肿瘤。33%的病例存在影像学异常。症状出现后4周内早期免疫治疗与良好预后相关。平均随访2±1年，两名抗Hu和抗Yo抗体阳性患者死亡（18%）。分别有4例和3例患者符合2021年PNS-Care明确和可能PNS的诊断标准。
本研究强调了PNS的临床异质性，强调了早期怀疑和及时开始治疗以获得最佳结果的必要性。
