Paediatric anti-N-methyl-d-aspartate receptor encephalitis: The first Italian multicenter case series

Stefano Sartori, Margherita Nosadini, Elisabetta Cesaroni, Raffaele Falsaperla, Giuseppe Capovilla, Francesca Beccaria, Maria Margherita Mancardi, Giuseppe Santangelo, Leandra Giunta, Clementina Boniver, Gaetano Cantalupo, Alberto Cappellari, Paola Costa, Bernardo Dalla Bernardina, Robertino Dilena, Maria Grazia Natali Sora, Maria Federica Pelizza, Dario Pruna, Domenico Serino, Francesca VanadiaFederico Vigevano, Nelia Zamponi, Caterina Zanus, Irene Toldo, Agnese Suppiej

Research output: Contribution to journalArticle

32 Citations (Scopus)

Abstract

Background Given the rarity of this condition, especially in children, there is a paucity of large reported paediatric case series of anti-N-methyl-d-aspartate receptor encephalitis. Methods To contribute to define the features of this condition, we describe retrospectively a new nationwide case series of 20 children (50% females), referred by 13 Italian centres. Results Mean age at onset was 8 years (range 3-17). Prodromal symptoms were reported in 31.6%; onset was with neurological symptoms in 70%, and with behavioural/psychiatric disturbances in 30%. Most patients developed a severe clinical picture (90%), and 41% experienced medical complications; children 12-18 years old seemed to be more severe and symptomatic than younger patients. All children received first-line immune therapy; second-line treatment was administered to 45%. Relapses occurred in 15%. At last follow-up (mean 23.9 months, range 5-82), 85% patients had mRS 0-1; this rate was higher among older patients, and in those receiving first immune therapy within 1 month. Conclusions Our case series confirms a symptomatologic core of paediatric anti-N-methyl-d-aspartate receptor encephalitis, even though displaying some distinctive features that may be explained by a specific genetic background or by the limited number of patients. The growing incidence of this condition, the relative age-dependent variability of its manifestations, the availability of immunotherapy and the possible better outcome with early treatment impose a high index of clinical suspicion be maintained. In the absence of data suggesting other specific etiologies, paediatricians should consider this diagnosis for children presenting with neurological and/or behavioural or psychiatric disturbances, regardless of age and gender.

Original languageEnglish
Pages (from-to)453-463
Number of pages11
JournalEuropean Journal of Paediatric Neurology
Volume19
Issue number4
DOIs
Publication statusPublished - Jul 1 2015

Fingerprint

Encephalitis
Pediatrics
Psychiatry
Prodromal Symptoms
Therapeutics
Age of Onset
Immunotherapy
aspartic acid receptor
Recurrence
Incidence

Keywords

  • Antibodies
  • Children
  • Encephalitis
  • Italy
  • NMDAR
  • Paediatric

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

Cite this

Sartori, S., Nosadini, M., Cesaroni, E., Falsaperla, R., Capovilla, G., Beccaria, F., ... Suppiej, A. (2015). Paediatric anti-N-methyl-d-aspartate receptor encephalitis: The first Italian multicenter case series. European Journal of Paediatric Neurology, 19(4), 453-463. https://doi.org/10.1016/j.ejpn.2015.02.006

Paediatric anti-N-methyl-d-aspartate receptor encephalitis : The first Italian multicenter case series. / Sartori, Stefano; Nosadini, Margherita; Cesaroni, Elisabetta; Falsaperla, Raffaele; Capovilla, Giuseppe; Beccaria, Francesca; Mancardi, Maria Margherita; Santangelo, Giuseppe; Giunta, Leandra; Boniver, Clementina; Cantalupo, Gaetano; Cappellari, Alberto; Costa, Paola; Dalla Bernardina, Bernardo; Dilena, Robertino; Natali Sora, Maria Grazia; Pelizza, Maria Federica; Pruna, Dario; Serino, Domenico; Vanadia, Francesca; Vigevano, Federico; Zamponi, Nelia; Zanus, Caterina; Toldo, Irene; Suppiej, Agnese.

In: European Journal of Paediatric Neurology, Vol. 19, No. 4, 01.07.2015, p. 453-463.

Research output: Contribution to journalArticle

Sartori, S, Nosadini, M, Cesaroni, E, Falsaperla, R, Capovilla, G, Beccaria, F, Mancardi, MM, Santangelo, G, Giunta, L, Boniver, C, Cantalupo, G, Cappellari, A, Costa, P, Dalla Bernardina, B, Dilena, R, Natali Sora, MG, Pelizza, MF, Pruna, D, Serino, D, Vanadia, F, Vigevano, F, Zamponi, N, Zanus, C, Toldo, I & Suppiej, A 2015, 'Paediatric anti-N-methyl-d-aspartate receptor encephalitis: The first Italian multicenter case series', European Journal of Paediatric Neurology, vol. 19, no. 4, pp. 453-463. https://doi.org/10.1016/j.ejpn.2015.02.006
Sartori, Stefano ; Nosadini, Margherita ; Cesaroni, Elisabetta ; Falsaperla, Raffaele ; Capovilla, Giuseppe ; Beccaria, Francesca ; Mancardi, Maria Margherita ; Santangelo, Giuseppe ; Giunta, Leandra ; Boniver, Clementina ; Cantalupo, Gaetano ; Cappellari, Alberto ; Costa, Paola ; Dalla Bernardina, Bernardo ; Dilena, Robertino ; Natali Sora, Maria Grazia ; Pelizza, Maria Federica ; Pruna, Dario ; Serino, Domenico ; Vanadia, Francesca ; Vigevano, Federico ; Zamponi, Nelia ; Zanus, Caterina ; Toldo, Irene ; Suppiej, Agnese. / Paediatric anti-N-methyl-d-aspartate receptor encephalitis : The first Italian multicenter case series. In: European Journal of Paediatric Neurology. 2015 ; Vol. 19, No. 4. pp. 453-463.
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T2 - The first Italian multicenter case series

AU - Sartori, Stefano

AU - Nosadini, Margherita

AU - Cesaroni, Elisabetta

AU - Falsaperla, Raffaele

AU - Capovilla, Giuseppe

AU - Beccaria, Francesca

AU - Mancardi, Maria Margherita

AU - Santangelo, Giuseppe

AU - Giunta, Leandra

AU - Boniver, Clementina

AU - Cantalupo, Gaetano

AU - Cappellari, Alberto

AU - Costa, Paola

AU - Dalla Bernardina, Bernardo

AU - Dilena, Robertino

AU - Natali Sora, Maria Grazia

AU - Pelizza, Maria Federica

AU - Pruna, Dario

AU - Serino, Domenico

AU - Vanadia, Francesca

AU - Vigevano, Federico

AU - Zamponi, Nelia

AU - Zanus, Caterina

AU - Toldo, Irene

AU - Suppiej, Agnese

PY - 2015/7/1

Y1 - 2015/7/1

N2 - Background Given the rarity of this condition, especially in children, there is a paucity of large reported paediatric case series of anti-N-methyl-d-aspartate receptor encephalitis. Methods To contribute to define the features of this condition, we describe retrospectively a new nationwide case series of 20 children (50% females), referred by 13 Italian centres. Results Mean age at onset was 8 years (range 3-17). Prodromal symptoms were reported in 31.6%; onset was with neurological symptoms in 70%, and with behavioural/psychiatric disturbances in 30%. Most patients developed a severe clinical picture (90%), and 41% experienced medical complications; children 12-18 years old seemed to be more severe and symptomatic than younger patients. All children received first-line immune therapy; second-line treatment was administered to 45%. Relapses occurred in 15%. At last follow-up (mean 23.9 months, range 5-82), 85% patients had mRS 0-1; this rate was higher among older patients, and in those receiving first immune therapy within 1 month. Conclusions Our case series confirms a symptomatologic core of paediatric anti-N-methyl-d-aspartate receptor encephalitis, even though displaying some distinctive features that may be explained by a specific genetic background or by the limited number of patients. The growing incidence of this condition, the relative age-dependent variability of its manifestations, the availability of immunotherapy and the possible better outcome with early treatment impose a high index of clinical suspicion be maintained. In the absence of data suggesting other specific etiologies, paediatricians should consider this diagnosis for children presenting with neurological and/or behavioural or psychiatric disturbances, regardless of age and gender.

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