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

被引：40

作者：

Gastaldi, M. ^{[1
]}

Mariotto, S. ^{[2
]}

Giannoccaro, M. P. ^{[3
,4
]}

Iorio, R. ^{[5
,6
]}

Zoccarato, M. ^{[7
,8
]}

Nosadini, M. ^{[8
,9
]}

Benedetti, L. ^{[10
]}

Casagrande, S. ^{[11
,12
]}

Di Filippo, M. ^{[13
]}

Valeriani, M. ^{[14
]}

Ricci, S. ^{[15
]}

Bova, S. ^{[16
]}

Arbasino, C. ^{[17
]}

Mauri, M. ^{[18
]}

Versino, M. ^{[18
]}

Vigevano, F. ^{[14
]}

Papetti, L. ^{[14
]}

Romoli, M. ^{[13
,19
]}

Lapucci, C. ^{[10
]}

Massa, F. ^{[10
]}

Sartori, S. ^{[8
,9
]}

Zuliani, L. ^{[8
,20
]}

Barilaro, A. ^{[12
]}

De Gaspari, P. ^{[8
]}

Spagni, G. ^{[6
]}

Evoli, A. ^{[5
,6
]}

Liguori, R. ^{[3
,4
]}

Ferrari, S. ^{[2
]}

Marchioni, E. ^{[21
]}

Giometto, B. ^{[22
]}

Massacesi, L. ^{[11
,12
]}

Franciotta, D. ^{[1
]}

机构：

[1] IRCCS Mondino Fdn, Neuroimmunol Lab, Pavia, Italy

[2] Univ Verona, Dept Neurosci Biomed & Movement Sci, Neurol Unit, Verona, Italy

[3] Univ Bologna, Dept Biomed & Neuromotor Sci, Bologna, Italy

[4] IRCCS Ist Sci Neurol Bologna, UOC Clin Neurol, Bologna, Italy

[5] Fdn Policlin Univ Agostino Gemelli IRCCS, Ist Neurol, Rome, Italy

[6] Univ Cattolica Sacro Cuore, Rome, Italy

[7] AULSS Euganea, Osped S Antonio, Padua, Italy

[8] Paediat Res Inst Citta Speranza, Neuroimmunol Grp, Padua, Italy

[9] Univ Hosp Padua, Dept Womens & Childrens Hlth, Paediat Neurol & Neurophysiol Unit, Padua, Italy

[10] IRCCS Osped Policlin S Martino, Genoa, Italy

[11] Florence Univ, Neurosci Dept, Florence, Italy

[12] Careggi Univ Hosp, Florence, Italy

[13] Perugia Univ, S Maria della Misericordia Hosp, Neurol Clin, Perugia, Italy

[14] Bambino Gesu Children Hosp, Neurol Unit, Rome, Italy

[15] Osped Citta di Castello & Branca, Citta Di Castello, Italy

[16] Children Hosp Vittore Buzzi, Pediat Neurol Unit, ASST Fatebenefratelli Sacco, Milan, Italy

[17] Osped Civile, Voghera, Italy

[18] Insubria Univ, Neurol & Stroke Unit, Varese, Italy

[19] Rimini Infermi Hosp AUSL Romagna, Neurol Unit, Rimini, Italy

[20] Osped S Bortolo, Dept Neurol, Vicenza, Italy

[21] IRCCS Mondino Fdn, Neuroncol Unit, Pavia, Italy

[22] APSS Osped S Chiara, Trento, Italy

来源：

EUROPEAN JOURNAL OF NEUROLOGY | 2020年 / 27卷 / 04期

关键词：

diagnostic criteria; immunotherapy; neuronal antibodies; NMDA RECEPTOR ENCEPHALITIS; DIAGNOSIS; AUTOANTIBODIES;

D O I：

10.1111/ene.14139

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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 < 0.001) and received second-line therapies more often (33.3% vs. 10.8%, P = 0.01). Delays in first-line therapy initiation were associated with poor response (P = 0.022; odds ratio 1.02; confidence interval 1.00-1.04). Conclusions In-house diagnostics improved Ab detection allowing better patient management but was available in a patient subgroup only, implying possible Ab-positive-AE underestimation. Notwithstanding this limitation, our findings suggest that Ab-negative-AE and Ab-positive-AE patients share similar oncological profiles, warranting appropriate tumor screening. Ab-negative-AE patients risk worse responses due to delayed and less aggressive treatments.

引用

页码：633 / 643

页数：11

共 20 条

[1] Does early treatment improve outcomes in N-methyl-D-aspartate receptor encephalitis? [J].