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Reader Response: Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes

  • Yin-Xi Zhang, Neurologist, Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University
  • Meng-Ting Cai, Neurologist, Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University
  • Yang Zheng, Neurologist, Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University
Submitted June 22, 2021

Yin-Xi Zhang, Neurologist, Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University; Meng-Ting Cai, Neurologist, Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University; Yang Zheng, Neurologist, Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University

We read with interest the updated diagnostic criteria for paraneoplastic neurologic syndromes (PNS) by Graus et al.1 A novel clinical scoring system--PNS-Care Score—was developed to enhance the diagnostic certainty with three levels of evidence: definite, probable, and possible. However, we found some difficulties in its clinical application. Firstly, the item “cancer” scored 4 only in conditions of being detected and consistent with phenotype and antibody (if present), or not consistent but antigen- expression demonstrated. What is the definition and scope of “consistent?” Does it include the commonly seen cancers in the three tables?1

Additionally, for those where “not consistent” cancers were detected— without distinct result of antigen expression at the 2-year or longer follow-up duration— the score is unclear. When a newly found tumor is unusual and no antigen expression is performed—perhaps untested due to limited resources— is it inconsistent by default? Meanwhile, what are the tumor scores in this condition at different follow-up phases? For instance, a patient who developed polyradiculoneuropathy with anti-Ma 2 antibody was found to have non-Hodgkin lymphoma at the 3-year follow-up.

References

1. Graus F, Vogrig A, Muñiz-Castrillo S, et al. Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. Neurol Neuroimmunol Neuroinflamm. 2021;8(4):e1014. Published 2021 May 18. doi:10.1212/NXI.0000000000001014.

Disclosure: The authors report no relevant disclosures. Contact journal@neurology.org for full disclosures. 

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Neurology: Neuroimmunology & Neuroinflammation
Online ISSN: 2332-7812

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