1. Paraneoplastic Neurological Syndromes as Initial Presentation of Tumors: An Eight-Year Single-Center Experience.
- Author
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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, and Tzartos, John S.
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PARANEOPLASTIC syndromes , *NEUROMUSCULAR diseases , *TUMORS , *NERVE tissue , *MYONEURAL junction , *THYMUS tumors , *PAPILLARY carcinoma - Abstract
Background: Paraneoplastic Neurological Syndromes (PNS) comprise a diverse group of disorders propagated by immune-mediated effects of malignant tumors on neural tissue. Methods: 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. Results: 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 (n = 4), anti-P/Q-VGCC (n = 3), anti-Hu (n = 3), anti-Yo (n = 1), anti-Ma (n = 1), anti-titin (n = 1), anti-IgLON5 (n = 1), and anti-GAD65 (n = 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. Conclusions: This study highlights the clinical heterogeneity of PNS, emphasizing the need for early suspicion and prompt treatment initiation for optimal outcomes. [ABSTRACT FROM AUTHOR]
- Published
- 2024
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