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CSF IgA NMDAR antibodies are potential biomarkers for teratomas in anti-NMDAR encephalitis
Virginie Desestret1, Aude Chefdeville1, Aurélien Viaccoz1
1Lyon Neuroscience Research Center (V.D., A.C., C. Bost, F.D., V.R., C. Blanc, O.P., J.-C.A., J.H.), INSERM U1028/CNRS UMR 5292; Université de Lyon-Université Claude Bernard Lyon 1 (V.D., A.C., C. Bost, F.D., V.R., C. Bardel, O.P., J.H.); the Department of Neurology D (V.D.) and French Reference Center on Paraneoplastic Neurological Syndrome (A.V., C. Bost, F.D., G.P., V.R., M.-O.C., C. Blanc, J.-C.A., J.-Y.D., J.H.), Hospices Civils de Lyon, Hôpital Neurologique, Bron; Service de Biostatistique (C. Bardel), Hospices Civils de Lyon, CNRS UMR 5558; Laboratoire de Biométrie et Biologie Evolutive (C. Bardel), Equipe Biostatistique-Santé, Villeurbanne; the Department of Biopathology (I.T.), Centre de Recherche en Cancérologie de Lyon; Service de Neurologie (J.-C.A.), CHU de Saint-Etienne et Université de Lyon, Saint-Etienne; and Service de Neurologie Mazarin (J.-Y.D.), Groupe Hospitalier Pitié-Salpêtrière, APHP, Université Pierre et Marie Curie-Paris 6, Centre de Recherche de l'Institut du Cerveau et de la Moelle Épinière, UMR S975, CNRS, UMR 7225, Paris, France.
Objective:
To evaluate the presence of immunoglobulin A (IgA) subtype of anti-NMDA receptor (NMDAR) antibodies (IgA-NMDAR-Abs) in the CSF of patients with immunoglobulin G (IgG)-NMDAR-Ab encephalitis and to describe the potential association with a specific clinical pattern.
Methods:
The retrospective analysis for the presence of IgA-NMDAR-Abs in 94 CSF samples from patients with anti-NMDAR encephalitis diagnosed between October 2007 and February 2014 was conducted at the French Reference Centre on Paraneoplastic Neurological Syndrome. This observational study compared 39 patients with both IgA- and IgG-NMDAR-Abs to 55 patients with only IgG-NMDAR-Abs.
Results:
In the retrospective cohort, 41% of the patients with NMDAR-Ab encephalitis had both CSF IgG- and IgA-NMDAR-Abs. Approximately half of the IgA-NMDAR-Ab-positive patients (18/38, 49%) definitively possessed associated tumors, primarily ovarian teratomas (17/18, 94%), compared with only 5% (3/55) of the patients in the IgA-NMDAR-Ab-negative group (p < 0.001). In the adult female population at risk for ovarian teratoma, the detection of CSF IgA-NMDAR-Ab positivity showed 85% sensitivity, 70% specificity, a 57% positive predictive value, and a 90% negative predictive value for the diagnosis of ovarian teratoma. No other specific clinical features or clinical outcome were associated with CSF IgA-NMDAR-Ab positivity.
Conclusion:
These results suggest that in patients with IgG-NMDAR-Ab encephalitis, CSF IgA-NMDAR-Abs could be used as a biological marker for the presence of an ovarian teratoma.
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