Relapse risk factors in anti-N-methyl-D-aspartate receptor encephalitis

Italian Working Group on Paediatric Anti-N-methyl-D-aspartate Receptor Encephalitis

Research output: Contribution to journalArticle

Abstract

AIM: To identify factors that may predict and affect the risk of relapse in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.

METHOD: This was a retrospective study of an Italian cohort of patients with paediatric (≤18y) onset anti-NMDAR encephalitis.

RESULTS: Of the 62 children included (39 females; median age at onset 9y 10mo, range 1y 2mo-18y; onset between 2005 and 2018), 21 per cent relapsed (median two total events per relapsing patient, range 2-4). Time to first relapse was median 31.5 months (range 7-89mo). Severity at first relapse was lower than onset (median modified Rankin Scale [mRS] 3, range 2-4, vs median mRS 5, range 3-5; admission to intensive care unit: 0/10 vs 3/10). At the survival analysis, the risk of relapsing was significantly lower in patients who received three or more different immune therapies at first disease event (hazard ratio 0.208, 95% confidence interval 0.046-0.941; p=0.042). Neurological outcome at follow-up did not differ significantly between patients with relapsing and monophasic disease (mRS 0-1 in 39/49 vs 12/13; p=0.431), although follow-up duration was significantly longer in relapsing (median 84mo, range 14-137mo) than in monophasic patients (median 32mo, range 4-108mo; p=0.002).

INTERPRETATION: Relapses may occur in about one-fifth of children with anti-NMDAR encephalitis, are generally milder than at onset, and may span over a long period, although they do not seem to be associated with severity in the acute phase or with outcome at follow-up. Aggressive immune therapy at onset may reduce risk of relapse.

WHAT THIS PAPER ADDS: Relapses of anti-N-methyl-D-aspartate receptor encephalitis may span over a long period. Relapses were not associated with severity in the acute phase or outcome at follow-up. Aggressive immune therapy at onset appears to decrease risk of relapse.

Original languageEnglish
JournalDevelopmental Medicine and Child Neurology
DOIs
Publication statusE-pub ahead of print - Jun 7 2019

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Anti-N-Methyl-D-Aspartate Receptor Encephalitis
Recurrence
Survival Analysis
Age of Onset
Intensive Care Units
Therapeutics
Retrospective Studies
Confidence Intervals
Pediatrics

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Relapse risk factors in anti-N-methyl-D-aspartate receptor encephalitis. / Italian Working Group on Paediatric Anti-N-methyl-D-aspartate Receptor Encephalitis.

In: Developmental Medicine and Child Neurology, 07.06.2019.

Research output: Contribution to journalArticle

Italian Working Group on Paediatric Anti-N-methyl-D-aspartate Receptor Encephalitis. Relapse risk factors in anti-N-methyl-D-aspartate receptor encephalitis. Developmental Medicine and Child Neurology. 2019 Jun 7. https://doi.org/10.1111/dmcn.14267
Italian Working Group on Paediatric Anti-N-methyl-D-aspartate Receptor Encephalitis. / Relapse risk factors in anti-N-methyl-D-aspartate receptor encephalitis. In: Developmental Medicine and Child Neurology. 2019.
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author = "{Italian Working Group on Paediatric Anti-N-methyl-D-aspartate Receptor Encephalitis} and Margherita Nosadini and Tiziana Granata and Sara Matricardi and Elena Freri and Francesca Ragona and Laura Papetti and Agnese Suppiej and Massimiliano Valeriani and Stefano Sartori",
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AU - Italian Working Group on Paediatric Anti-N-methyl-D-aspartate Receptor Encephalitis

AU - Nosadini, Margherita

AU - Granata, Tiziana

AU - Matricardi, Sara

AU - Freri, Elena

AU - Ragona, Francesca

AU - Papetti, Laura

AU - Suppiej, Agnese

AU - Valeriani, Massimiliano

AU - Sartori, Stefano

N1 - © 2019 Mac Keith Press.

PY - 2019/6/7

Y1 - 2019/6/7

N2 - AIM: To identify factors that may predict and affect the risk of relapse in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.METHOD: This was a retrospective study of an Italian cohort of patients with paediatric (≤18y) onset anti-NMDAR encephalitis.RESULTS: Of the 62 children included (39 females; median age at onset 9y 10mo, range 1y 2mo-18y; onset between 2005 and 2018), 21 per cent relapsed (median two total events per relapsing patient, range 2-4). Time to first relapse was median 31.5 months (range 7-89mo). Severity at first relapse was lower than onset (median modified Rankin Scale [mRS] 3, range 2-4, vs median mRS 5, range 3-5; admission to intensive care unit: 0/10 vs 3/10). At the survival analysis, the risk of relapsing was significantly lower in patients who received three or more different immune therapies at first disease event (hazard ratio 0.208, 95% confidence interval 0.046-0.941; p=0.042). Neurological outcome at follow-up did not differ significantly between patients with relapsing and monophasic disease (mRS 0-1 in 39/49 vs 12/13; p=0.431), although follow-up duration was significantly longer in relapsing (median 84mo, range 14-137mo) than in monophasic patients (median 32mo, range 4-108mo; p=0.002).INTERPRETATION: Relapses may occur in about one-fifth of children with anti-NMDAR encephalitis, are generally milder than at onset, and may span over a long period, although they do not seem to be associated with severity in the acute phase or with outcome at follow-up. Aggressive immune therapy at onset may reduce risk of relapse.WHAT THIS PAPER ADDS: Relapses of anti-N-methyl-D-aspartate receptor encephalitis may span over a long period. Relapses were not associated with severity in the acute phase or outcome at follow-up. Aggressive immune therapy at onset appears to decrease risk of relapse.

AB - AIM: To identify factors that may predict and affect the risk of relapse in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.METHOD: This was a retrospective study of an Italian cohort of patients with paediatric (≤18y) onset anti-NMDAR encephalitis.RESULTS: Of the 62 children included (39 females; median age at onset 9y 10mo, range 1y 2mo-18y; onset between 2005 and 2018), 21 per cent relapsed (median two total events per relapsing patient, range 2-4). Time to first relapse was median 31.5 months (range 7-89mo). Severity at first relapse was lower than onset (median modified Rankin Scale [mRS] 3, range 2-4, vs median mRS 5, range 3-5; admission to intensive care unit: 0/10 vs 3/10). At the survival analysis, the risk of relapsing was significantly lower in patients who received three or more different immune therapies at first disease event (hazard ratio 0.208, 95% confidence interval 0.046-0.941; p=0.042). Neurological outcome at follow-up did not differ significantly between patients with relapsing and monophasic disease (mRS 0-1 in 39/49 vs 12/13; p=0.431), although follow-up duration was significantly longer in relapsing (median 84mo, range 14-137mo) than in monophasic patients (median 32mo, range 4-108mo; p=0.002).INTERPRETATION: Relapses may occur in about one-fifth of children with anti-NMDAR encephalitis, are generally milder than at onset, and may span over a long period, although they do not seem to be associated with severity in the acute phase or with outcome at follow-up. Aggressive immune therapy at onset may reduce risk of relapse.WHAT THIS PAPER ADDS: Relapses of anti-N-methyl-D-aspartate receptor encephalitis may span over a long period. Relapses were not associated with severity in the acute phase or outcome at follow-up. Aggressive immune therapy at onset appears to decrease risk of relapse.

U2 - 10.1111/dmcn.14267

DO - 10.1111/dmcn.14267

M3 - Article

C2 - 31175679

JO - Developmental Medicine and Child Neurology

JF - Developmental Medicine and Child Neurology

SN - 0012-1622

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