Subgroup comparison according to clinical phenotype and serostatus in autoimmune encephalitis: a multicenter retrospective study

M. Gastaldi, S. Mariotto, M.P. Giannoccaro, R. Iorio, M. Zoccarato, M. Nosadini, L. Benedetti, S. Casagrande, M. Di Filippo, M. Valeriani, S. Ricci, S. Bova, C. Arbasino, M. Mauri, M. Versino, F. Vigevano, L. Papetti, M. Romoli, C. Lapucci, F. MassaS. Sartori, L. Zuliani, A. Barilaro, P. De Gaspari, G. Spagni, A. Evoli, R. Liguori, S. Ferrari, E. Marchioni, B. Giometto, L. Massacesi, D. Franciotta

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Abstract

Background and purpose : Autoimmune encephalitides (AE) include a spectrum of neurological disorders whose diagnosis revolves around the detection of neuronal antibodies (Abs). Consensus-based diagnostic criteria (AE-DC) allow clinic-serological subgrouping of AE, with unclear prognostic implications. The impact of AE-DC on patients’ management was studied, focusing on the subgroup of Ab-negative-AE. Methods: This was a retrospective multicenter study on patients fulfilling AE-DC. All patients underwent Ab testing with commercial cell-based assays (CBAs) and, when available, in-house assays (immunohistochemistry, live/fixed CBAs, neuronal cultures) that contributed to defining final categories. Patients were classified as Ab-positive-AE [N-methyl-d-aspartate-receptor encephalitis (NMDAR-E), Ab-positive limbic encephalitis (LE), definite-AE] or Ab-negative-AE (Ab-negative-LE, probable-AE, possible-AE). Results: Commercial CBAs detected neuronal Abs in 70/118 (59.3%) patients. Testing 37/48 Ab-negative cases, in-house assays identified Abs in 11 patients (29.7%). A hundred and eighteen patients fulfilled the AE-DC, 81 (68.6%) with Ab-positive-AE (Ab-positive-LE, 40; NMDAR-E, 32; definite-AE, nine) and 37 (31.4%) with Ab-negative-AE (Ab-negative-LE, 17; probable/possible-AE, 20). Clinical phenotypes were similar in Ab-positive-LE versus Ab-negative-LE. Twenty-four/118 (20.3%) patients had tumors, and 19/118 (16.1%) relapsed, regardless of being Ab-positive or Ab-negative. Ab-positive-AE patients were treated earlier than Ab-negative-AE patients (P = 0.045), responded more frequently to treatments (92.3% vs. 65.6%, P 
Original languageEnglish
Pages (from-to)633-643
Number of pages11
JournalEuropean Journal of Neurology
Volume27
Issue number4
DOIs
Publication statusPublished - 2020

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Gastaldi, M., Mariotto, S., Giannoccaro, M. P., Iorio, R., Zoccarato, M., Nosadini, M., Benedetti, L., Casagrande, S., Di Filippo, M., Valeriani, M., Ricci, S., Bova, S., Arbasino, C., Mauri, M., Versino, M., Vigevano, F., Papetti, L., Romoli, M., Lapucci, C., ... Franciotta, D. (2020). Subgroup comparison according to clinical phenotype and serostatus in autoimmune encephalitis: a multicenter retrospective study. European Journal of Neurology, 27(4), 633-643. https://doi.org/10.1111/ene.14139