Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.
ADVANCED SEARCH
Log in
Top
Neurological Sciences
Published in: Neurological Sciences 2/2019

01-02-2019 | Original Article

Clinical variability of children with anti-N-methyl-d-aspartate receptor encephalitis in southern Brazil: a cases series and review of the literature

Authors: Daniel Almeida do Valle, Joselainy Stela Pires Galeazzi, Mayara de Rezende Machado, Vanessa Catarine Silva Abreu Ribeiro dos Santos, Alcir Francisco da Silva, Alfredo Lohr Júnior, Mara Lúcia Schmitz Ferreira Santos, Rosana Herminia Scola

Published in: Neurological Sciences | Issue 2/2019

Login to get access

Abstract

Purpose

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an immune-mediated disease of the central nervous system (CNS). The aim of this study was to describe the variability of clinical presentation in anti-NMDAR encephalitis, treatment and outcomes in a case series of children and adolescents.

Methods

Retrospectively analyse patients diagnosed with anti-NMDAR encephalitis, from 2010 to 2018.

Results

The study population consisted of nine children with anti-NMDAR encephalitis from southern Brazil, six females and three males, aged 5 months to 16 years (mean 5 years). The time of follow-up varied between 1 and 7 years, with a mean of 3 years. The most frequent first manifestation consisted of seizures. All patients described had psychiatric symptoms and a wide spectrum of neurologic findings. Five patients had unilateral symptoms. Magnetic resonance imaging and electroencephalogram were normal in most patients. Cerebrospinal fluid pleocytosis occurred in five patients. All patients were administered immunoglobulin and/or steroids. Seven patients (78%) required cyclophosphamide and/or rituximab. Almost half of the patients fully recovered from all symptoms.

Conclusions

A wide variety of symptoms were observed in this study and, although unilateral symptoms are rarely reported in the literature, a high frequency was observed among Brazilian children. Alternatives to first-line therapy should be considered in patients with clinical suspicion, even if they have not had a good response with first-line therapy.
Literature
1.
Dalmau J, Tüzün E, Wu HY, Masjuan J, Rossi JE, Voloschin A, Baehring JM, Shimazaki H, Koide R, King D, Mason W, Sansing LH, Dichter MA, Rosenfeld MR, Lynch DR (2007) Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol 61:25–36CrossRef
2.
Gresa-arribas N, Titulaer MJ, Torrents A et al (2015) Diagnosis and significance of antibody titers in anti-NMDA receptor encephalitis, a retrospective study. Lancet Neurol 13:167–177CrossRef
3.
Granerod J, Ambrose HE, Davies NWS, Clewley JP, Walsh AL, Morgan D, Cunningham R, Zuckerman M, Mutton KJ, Solomon T, Ward KN, Lunn MP, Irani SR, Vincent A, Brown DW, Crowcroft NS, UK Health Protection Agency (HPA) Aetiology of Encephalitis Study Group (2010) Causes of encephalitis and differences in their clinical presentations in England: a multicentre, population-based prospective study. Lancet Infect Dis 10:835–844CrossRef
4.
Konuskan B, Yildirim M, Topaloglu H, Erol I, Oztoprak U, Tan H, Gocmen R, Anlar B (2018) Clinical presentation of anti-N-methyl-D-aspartate receptor and anti-voltage-gated potassium channel complex antibodies in children: a series of 24 cases. Eur J Paediatr Neurol 22:135–142CrossRef
5.
Borlot F, Santos MLF, Bandeira M et al (2012) Anti-N-methyl D-aspartate receptor encephalitis in childhood. J Pediatr 88:275–278
6.
Ho AC, Chan SH, Chan E et al (2018) Anti-N-methyl-D-aspartate receptor encephalitis in children: incidence and experience in Hong Kong. Brain Dev 40(6):473–479
7.
Wright S, Hacohen Y, Jacobson L, Agrawal S, Gupta R, Philip S, Smith M, Lim M, Wassmer E, Vincent A (2015) N-methyl-D-aspartate receptor antibody-mediated neurological disease: results of a UK-based surveillance study in children. Arch Dis Child 100:521–526CrossRef
8.
de Bruijn MAAM, Aarsen FK, van Oosterhout MP et al (2018) Long-term neuropsychological outcome following pediatric anti-NMDAR encephalitis. Neurology 90:e1997–e2005CrossRef
9.
Florance NR, Davis RL, Lam C, Szperka C, Zhou L, Ahmad S, Campen CJ, Moss H, Peter N, Gleichman AJ, Glaser CA, Lynch DR, Rosenfeld MR, Dalmau J (2009) Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents. Ann Neurol 66:11–18CrossRef
10.
Dale RC, Irani SR, Brilot F, Pillai S, Webster R, Gill D, Lang B, Vincent A (2009) N-methyl-D-aspartate receptor antibodies in pediatric dyskinetic encephalitis lethargica. Ann Neurol 66:704–709CrossRef
11.
Hacohen Y, Wright S, Waters P, Agrawal S, Carr L, Cross H, de Sousa C, DeVile C, Fallon P, Gupta R, Hedderly T, Hughes E, Kerr T, Lascelles K, Lin JP, Philip S, Pohl K, Prabahkar P, Smith M, Williams R, Clarke A, Hemingway C, Wassmer E, Vincent A, Lim MJ (2013) Paediatric autoimmune encephalopathies: clinical features, laboratory investigations and outcomes in patients with or without antibodies to known central nervous system autoantigens. J Neurol Neurosurg Psychiatry 84:748–755CrossRef
12.
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. Eur J Paediatr Neurol 19:453–463CrossRef
13.
Brenton JN, Kim J, Schwartz RH (2016) Approach to the Management of Pediatric-Onset Anti-N-methyl-d-aspartate (Anti-NMDA) receptor encephalitis. J Child Neurol 31:1150–1155CrossRef
14.
Wang Y, Zhang W, Yin J, Lu Q, Yin F, He F, Peng J (2017) Anti-N-methyl-D-aspartate receptor encephalitis in children of Central South China: clinical features, treatment, influencing factors, and outcomes. J Neuroimmunol 312:59–65CrossRef
15.
Armangue T, Titulaer MJ, Málaga I et al (2013) Pediatric anti-NMDAR encephalitis-clinical analysis and novel findings in a series of 20 patients. J Pediatr 25:713–724
16.
Viaccoz A, Desestret V, Ducray F, Picard G, Cavillon G, Rogemond V, Antoine JC, Delattre JY, Honnorat J (2014) Clinical specificities of adult male patients with NMDA receptor antibodies encephalitis. Neurology 82:556–563CrossRef
17.
Ignacio R-A, Josep D, Teresa S et al (2011) Isolated hemidystonia associated with NMDA receptor antibodies. Mov Disord 26:265–275
18.
Hughes EG, Peng X, Gleichman AJ, Lai M, Zhou L, Tsou R, Parsons TD, Lynch DR, Dalmau J, Balice-Gordon RJ (2010) Cellular and synaptic mechanisms of anti-NMDAR encephalitis. J Neurosci 30:5866–5875CrossRef
19.
Barry H, Byrne S, Barrett E, Murphy KC, Cotter DR (2015) Anti-N-methyl-D-aspartate receptor encephalitis: review of clinical presentation, diagnosis and treatment. BJPsych Bull 39:19–23CrossRef
20.
Guasp M, Dalmau J (2018) Encefalitis por anticuerpos contra el receptor de NMDA. Med Clin (Barc) 150:1–9
21.
Iizuka T, Sakai F, Ide T, Monzen T, Yoshii S, Iigaya M, Suzuki K, Lynch DR, Suzuki N, Hata T, Dalmau J (2008) Anti-NMDA receptor encephalitis in Japan: long-term outcome without tumor removal. Neurology 70:504–511CrossRef
Metadata
Title
Clinical variability of children with anti-N-methyl-d-aspartate receptor encephalitis in southern Brazil: a cases series and review of the literature
Authors
Daniel Almeida do Valle
Joselainy Stela Pires Galeazzi
Mayara de Rezende Machado
Vanessa Catarine Silva Abreu Ribeiro dos Santos
Alcir Francisco da Silva
Alfredo Lohr Júnior
Mara Lúcia Schmitz Ferreira Santos
Rosana Herminia Scola
Publication date
01-02-2019
Publisher
Springer International Publishing
Published in
Neurological Sciences / Issue 2/2019
Print ISSN: 1590-1874
Electronic ISSN: 1590-3478
DOI
https://doi.org/10.1007/s10072-018-3648-z

Other articles of this Issue 2/2019

Neurological Sciences 2/2019 Go to the issue

Original Article

The clinical and imaging features of hypertrophic pachymeningitis: a clinical analysis on 22 patients

Original Article

Factors associated with freezing of gait in patients with Parkinson’s disease

Original Article

MR evaluation of encephalic leukoaraiosis in sudden sensorineural hearing loss (SSNHL) patients

Highlights

Highlights of the Issue 2, 2019

Brief Communication

The performance of patients with Parkinson’s disease on the Face-Name Associative Memory Examination

Original Article

A novel homozygous nonsense mutation in CCDC88A gene cause PEHO-like syndrome in consanguineous Saudi family