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Objective <p>The diagnosis of paraneoplastic neurologic syndromes (PNS) requires exclusion of alternative etiologies. However, the spectrum of alternative diagnoses encountered during PNS workup (“better explanations”) has not been systematically examined. We aimed to characterize this spectrum.</p> Methods <p>Retrospective population-based study in Friuli-Venezia-Giulia (Italy). Among 878 consecutive patients tested for PNS antibodies at a centralized tertiary center serving three hospitals (2009–2017), alternative diagnoses were identified. Results of a logistic regression model were used to develop the <i>PNS DDx Score</i>.</p> Results <p>Among 661 patients with alternative diagnoses, median age was 66&#xa0;years, and 51% were male. CNS involvement predominated in 397 patients (60%), peripheral in 215 (33%), and extra-nervous system in 49 (7%). Etiologic diagnosis was reached in 269 with central involvement, including degenerative (97, 36%), autoimmune (47, 17%), and vascular (37, 14%). In peripheral presentations, a diagnosis was reached in 112 and included autoimmune (51, 45%), toxic-metabolic (40, 36%), and vasculitis (11, 10%). PNS antibody testing increased by 96% from 2009 to 2017; diagnostic yield remained low (7% vs. 8%). Intermediate-risk or other syndromes (OR 7.97, CI95% 3.07–23.80; OR 192.82 CI95%, 66.11–739.59) and low-risk/absence of tumor (OR 6.90, CI95% 2.75–18.92) were associated with alternative diagnoses, whereas increasing age (OR 0.95, CI95% 0.92–0.97) was inversely associated. These variables (age, syndrome, and tumor type) were incorporated in the <i>PNS DDx Score</i>.</p> Conclusion <p>Alternative diagnoses during PNS workup are common in patients &lt; 55&#xa0;years and those without high-risk syndromes. The <i>PNS DDx Score</i> can be used to identify patients at risk of misdiagnosis.</p>

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Alternative diagnoses of suspected paraneoplastic neurological syndromes: a population-based study

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年3月11日

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  • Alberto Vogrig,
  • Francesco Bax,
  • Gian Luigi Gigli,
  • Andrea Bernardini,
  • Alessandro Marini,
  • Michele Rana,
  • Paolo Passadore,
  • Daniele Bagatto,
  • Francesco Curcio,
  • Sergio Muñiz-Castrillo,
  • Jérôme Honnorat,
  • Martina Fabris,
  • Mariarosaria Valente

摘要

Objective

The diagnosis of paraneoplastic neurologic syndromes (PNS) requires exclusion of alternative etiologies. However, the spectrum of alternative diagnoses encountered during PNS workup (“better explanations”) has not been systematically examined. We aimed to characterize this spectrum.

Methods

Retrospective population-based study in Friuli-Venezia-Giulia (Italy). Among 878 consecutive patients tested for PNS antibodies at a centralized tertiary center serving three hospitals (2009–2017), alternative diagnoses were identified. Results of a logistic regression model were used to develop the PNS DDx Score.

Results

Among 661 patients with alternative diagnoses, median age was 66 years, and 51% were male. CNS involvement predominated in 397 patients (60%), peripheral in 215 (33%), and extra-nervous system in 49 (7%). Etiologic diagnosis was reached in 269 with central involvement, including degenerative (97, 36%), autoimmune (47, 17%), and vascular (37, 14%). In peripheral presentations, a diagnosis was reached in 112 and included autoimmune (51, 45%), toxic-metabolic (40, 36%), and vasculitis (11, 10%). PNS antibody testing increased by 96% from 2009 to 2017; diagnostic yield remained low (7% vs. 8%). Intermediate-risk or other syndromes (OR 7.97, CI95% 3.07–23.80; OR 192.82 CI95%, 66.11–739.59) and low-risk/absence of tumor (OR 6.90, CI95% 2.75–18.92) were associated with alternative diagnoses, whereas increasing age (OR 0.95, CI95% 0.92–0.97) was inversely associated. These variables (age, syndrome, and tumor type) were incorporated in the PNS DDx Score.

Conclusion

Alternative diagnoses during PNS workup are common in patients < 55 years and those without high-risk syndromes. The PNS DDx Score can be used to identify patients at risk of misdiagnosis.