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Comparative Study of Paraneoplastic and Nonparaneoplastic Autoimmune Encephalitis With GABABR Antibodies
Florian Lamblin1, Jeroen Kerstens1, Sergio Muñiz-Castrillo1
1From the French Reference Center on Paraneoplastic Neurological Syndrome and Autoimmune Encephalitis (F.L., V.R., G.P., M.V., A.-L.P., M.B., B.J., J.H.), Hospices Civils de Lyon; Institut MeLiS INSERM U1314/CNRS UMR 5284 (F.L., V.R., G.P., M.V., A.-L.P., M.B., B.J., J.H.), Université Claude Bernard Lyon 1; Department of Neurology (F.L.), University Hospital of La Réunion, Saint-Pierre (La Réunion), France; Department of Neurology (J.K., M.H.V.C.-H., M.A.D.B., J.M.V., M.J.T.), Erasmus Medical Center, Rotterdam, The Netherlands; Stanford Center for Sleep Sciences and Medicine (S.M.-C., V.P.S., E.M.), Stanford University, Palo Alto, CA; Clinical Neurology (A.V.), Department of Neurosciences, Azienda Sanitaria Universitaria Friuli Centrale (ASU FC); Department of Medicine (DAME) (A.V.), University of Udine Medical School, Italy; Department of Immunology (D.G.), Hôpital Lyon Sud, Hospices Civils de Lyon, France; Department of Immunology (M.S.), Laboratory Medical Immunology, Erasmus Medical Center, Rotterdam, The Netherlands; Department of Neurology (L.T., L.H.), University Hospital of Nancy; Department of Clinical Neurosciences (J.A., C.M.), University Hospital of Bordeaux, Bordeaux, France; Department of Neuro-Oncology (D.P.), Pitié Salpêtrière Hospital, AP-HP, Paris; Department of Neurology (L.K.), University Hospital of Strasbourg; Department of Neurology (V.B.), Côte d'Azur University, Nice; Department of Neurology (J.-C.G.A.), University Hospital of Saint-Etienne; Stroke Center Neurology Division (A.W.), Hopital Foch, Suresnes; University Grenoble Alpes (P.K.), Inserm, U1216, CHU Grenoble Alpes, Grenoble Institut Neurosciences; Neurological Intensive Care Unit (S.D.), Pitié-Salpêtrière Hospital, AP-HP, Paris; Department of Neurology (G.A.), Hôpitaux Civils de Colmar; Department of Public Health (N.T., M.N.), Hospices Civils de Lyon; and Department of Medicine (A.M.), Centre Leon Berard, UNICANCER, Lyon, France.
Background And Objectives:
While patients with paraneoplastic autoimmune encephalitis (AE) with gamma-aminobutyric-acid B receptor antibodies (GABABR-AE) have poor functional outcomes and high mortality, the prognosis of nonparaneoplastic cases has not been well studied.
Methods:
Patients with GABABR-AE from the French and the Dutch Paraneoplastic Neurologic Syndromes Reference Centers databases were retrospectively included and their data collected; the neurologic outcomes of paraneoplastic and nonparaneoplastic cases were compared. Immunoglobulin G (IgG) isotyping and human leukocyte antigen (HLA) genotyping were performed in patients with available samples.
Results:
A total of 111 patients (44/111 [40%] women) were enrolled, including 84 of 111 (76%) paraneoplastic and 18 of 111 (16%) nonparaneoplastic cases (cancer status was undetermined for 9 patients). Patients presented with seizures (88/111 [79%]), cognitive impairment (54/111 [49%]), and/or behavioral disorders (34/111 [31%]), and 54 of 111 (50%) were admitted in intensive care unit (ICU). Nonparaneoplastic patients were significantly younger (median age 54 years [range 19-88] vs 67 years [range 50-85] for paraneoplastic cases, p < 0.001) and showed a different demographic distribution. Nonparaneoplastic patients more often had CSF pleocytosis (17/17 [100%] vs 58/78 [74%], p = 0.02), were almost never associated with KTCD16-abs (1/16 [6%] vs 61/70 [87%], p < 0.001), and were more frequently treated with second-line immunotherapy (11/18 [61%] vs 18/82 [22%], p = 0.003). However, no difference of IgG subclass or HLA association was observed, although sample size was small (10 and 26 patients, respectively). After treatment, neurologic outcome was favorable (mRS ≤2) for 13 of 16 (81%) nonparaneoplastic and 37 of 84 (48%) paraneoplastic cases (p = 0.03), while 3 of 18 (17%) and 42 of 83 (51%) patients had died at last follow-up (p = 0.008), respectively. Neurologic outcome no longer differed after adjustment for confounding factors but seemed to be negatively associated with increased age and ICU admission. A better survival was associated with nonparaneoplastic cases, a younger age, and the use of immunosuppressive drugs.
Discussion:
Nonparaneoplastic GABABR-AE involved younger patients without associated KCTD16-abs and carried better neurologic and vital prognoses than paraneoplastic GABABR-AE, which might be due to a more intensive treatment strategy. A better understanding of immunologic mechanisms underlying both forms is needed.
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