Table 1.
Recommended diagnostic criteria for paraneoplastic neurological syndromes (J Neurol Neurosurg Psychiatry 2004) in correspondence with the updated PNS-CARE diagnostic criteria for paraneoplastic neurological syndromes (Neurol Neuroimmunol Neuroinflamm 2021) [3,5].
| 2021 PNS-Care Criteria | 2004 Graus-Criteria | ||
|---|---|---|---|
| Criteria | Score | Criteria | |
|
Clinical Phenotype Risk Level High-risk phenotype (syndrome often triggered by cancer) Intermediate-risk phenotype (can occur with or without cancer) Low-risk phenotype (weaker association with cancer) Laboratory level High-risk antibody (>70% cancer association) Intermediate-risk antibody (30–70% cancer association) Low-risk antibody (<30% cancer association) Tumor Identified, consistent with phenotype and antibody Not identified or not consistent with phenotype, with follow-up <2 years Not found, and follow up >2 years |
3 2 1 3 2 0 4 1 0 |
Classical Syndrome | Non-classical syndrome |
| Tumor present or tumor absent | |||
| PNS Abs present or absent | Improvement after therapy or PNS Abs present | ||
| or well characterized PNS Abs present | |||
| Score | Diagnosis | Diagnosis | |
| ≥8 | Definite PNS | Definite | |
| 6–7 | Probable PNS | ||
| 4–5 | Possible PNS | ||
| <4 | Not PNS | ||
Definite 2004 Graus criteria: (1) classical syndromes characterized by the development of cancer, within 5 years of their initial diagnosis; or (2) non-classical syndromes diagnosed within 5 years of a cancer diagnosis, with the presence of PNS-Abs; or (3) non-classical syndromes showing substantial neurological improvement following cancer treatment; or (4) either classical or non-classical syndromes in conjunction with the identification of well-characterized PNS-Abs. 2021 PNS-Care criteria: The scoring system integrates clinical phenotypes, antibodies, and tumor identification. Phenotypes are stratified as high or intermediate risk, based on distinct features. The scoring system classifies PNS-Abs as high, medium, or low risk. Cancer presence, aligned with the identified antibody within 2 years, is considered. A definitive PNS diagnosis (score ≥ 8) requires a high- or intermediate-risk phenotype, a corresponding antibody, and cancer presence. Abbreviations: PNS: paraneoplastic neurological syndromes; Abs: antibodies.