BACKGROUND: 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. We studied AE-DC impact on patients' management, focusing on the subgroup of Ab-negative-AE. METHODS: 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 define final categories. Patients were classified as Ab-positive-AE [NMDAR-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%). One-hundred-eighteen patients fulfilled AE-DC, 81(68.6%) with Ab-positive-AE (Ab-positive-LE, 40; NMDAR-E, 32; definite-AE, 9), 37(31.4%) with Ab-negative-AE (Ab-negative-LE, 17; probable/possible-AE, 20). Clinical phenotypes were similar in Ab-positive- vs 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 associated with poor response (p=0.022; OR,1.02; CI,1.00-1.04). CONCLUSIONS: In-house diagnostics improved Ab detection, allowing better patients' 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 share similar oncologic profiles, warranting appropriate tumor screening. Ab-negative-AE patients risk worse responses due to delayed and less aggressive treatments.

Subgroup Comparison According to Clinical Phenotype and Serostatus in Autoimmune Encephalitis: A Multicenter Retrospective Study / Gastaldi M, Mariotto S, Giannoccaro MP, 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, Massa F, Sartori S,7, Zuliani L, Barilaro A, De Gaspari P, Spagni G, Evoli A, Liguori R, Ferrari S, Marchioni E, Giometto B, Massacesi L, Franciotta D.. - In: EUROPEAN JOURNAL OF NEUROLOGY. - ISSN 1468-1331. - ELETTRONICO. - 27:(2020), pp. 633-643. [10.1111/ene.14139]

Subgroup Comparison According to Clinical Phenotype and Serostatus in Autoimmune Encephalitis: A Multicenter Retrospective Study

Gastaldi M;Iorio R;Casagrande S;Bova S;Lapucci C;Sartori S;Barilaro A;Spagni G;Ferrari S;Massacesi L;Franciotta D.
2020

Abstract

BACKGROUND: 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. We studied AE-DC impact on patients' management, focusing on the subgroup of Ab-negative-AE. METHODS: 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 define final categories. Patients were classified as Ab-positive-AE [NMDAR-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%). One-hundred-eighteen patients fulfilled AE-DC, 81(68.6%) with Ab-positive-AE (Ab-positive-LE, 40; NMDAR-E, 32; definite-AE, 9), 37(31.4%) with Ab-negative-AE (Ab-negative-LE, 17; probable/possible-AE, 20). Clinical phenotypes were similar in Ab-positive- vs 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 associated with poor response (p=0.022; OR,1.02; CI,1.00-1.04). CONCLUSIONS: In-house diagnostics improved Ab detection, allowing better patients' 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 share similar oncologic profiles, warranting appropriate tumor screening. Ab-negative-AE patients risk worse responses due to delayed and less aggressive treatments.
2020
27
633
643
Goal 3: Good health and well-being for people
Gastaldi M, Mariotto S, Giannoccaro MP, Iorio R, Zoccarato M, Nosadini M, Benedetti L, Casagrande S, Di Filippo M, Valeriani M, Ricci S, Bova S, Arbas...espandi
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1180703
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