RT Journal Article
SR Electronic
T1 Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e1014
DO 10.1212/NXI.0000000000001014
VO 8
IS 4
A1 Graus, Francesc
A1 Vogrig, Alberto
A1 Muñiz-Castrillo, Sergio
A1 Antoine, Jean-Christophe G.
A1 Desestret, Virginie
A1 Dubey, Divyanshu
A1 Giometto, Bruno
A1 Irani, Sarosh R.
A1 Joubert, Bastien
A1 Leypoldt, Frank
A1 McKeon, Andrew
A1 Prüss, Harald
A1 Psimaras, Dimitri
A1 Thomas, Laure
A1 Titulaer, Maarten J.
A1 Vedeler, Christian A.
A1 Verschuuren, Jan J.
A1 Dalmau, Josep
A1 Honnorat, Jerome
YR 2021
UL http://nn.neurology.org/content/8/4/e1014.abstract
AB Objective The contemporary diagnosis of paraneoplastic neurologic syndromes (PNSs) requires an increasing understanding of their clinical, immunologic, and oncologic heterogeneity. The 2004 PNS criteria are partially outdated due to advances in PNS research in the last 16 years leading to the identification of new phenotypes and antibodies that have transformed the diagnostic approach to PNS. Here, we propose updated diagnostic criteria for PNS.Methods A panel of experts developed by consensus a modified set of diagnostic PNS criteria for clinical decision making and research purposes. The panel reappraised the 2004 criteria alongside new knowledge on PNS obtained from published and unpublished data generated by the different laboratories involved in the project.Results The panel proposed to substitute “classical syndromes” with the term “high-risk phenotypes” for cancer and introduce the concept of “intermediate-risk phenotypes.” The term “onconeural antibody” was replaced by “high risk” (>70% associated with cancer) and “intermediate risk” (30%–70% associated with cancer) antibodies. The panel classified 3 levels of evidence for PNS: definite, probable, and possible. Each level can be reached by using the PNS-Care Score, which combines clinical phenotype, antibody type, the presence or absence of cancer, and time of follow-up. With the exception of opsoclonus-myoclonus, the diagnosis of definite PNS requires the presence of high- or intermediate-risk antibodies. Specific recommendations for similar syndromes triggered by immune checkpoint inhibitors are also provided.Conclusions The proposed criteria and recommendations should be used to enhance the clinical care of patients with PNS and to encourage standardization of research initiatives addressing PNS.AMPAR=α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; ANNA=antineuronal nuclear antibody; CASPR2=contactin-associated protein-like 2; CBA=cell-based assay; CRMP5=collapsin response-mediator protein 5; DNER=delta/notch-like epidermal growth factor-related receptor; EM=encephalomyelitis; GABABR=gamma-aminobutyric-acid B receptor; GAD65=glutamic acid decarboxylase 65; ICI=immune checkpoint inhibitor; IHC/IF=immunohistochemistry/immunofluorescence; irAE=immune-related adverse event; LE=limbic encephalitis; LEMS=Lambert-Eaton myasthenic syndrome; LGI1=leucine-rich glioma-inactivated 1; mGluR=metabotropic glutamate receptor; NSCLC=non-SCLC; OMS=opsoclonus-myoclonus syndrome; PCA=Purkinje cell antibody; PNS=paraneoplastic neurologic syndrome; SCLC=small-cell lung cancer; SNN=sensory neuronopathy; SPS=stiff-person syndrome; VGCC=voltage-gated calcium channel