Spectrum of Anti-NMDA Receptor Antibody Encephalitis: Clinical Profile, Management and Outcomes.
Anti NMDAR
autoimmune
encephalitis
immunotherapy
Journal
Annals of Indian Academy of Neurology
ISSN: 0972-2327
Titre abrégé: Ann Indian Acad Neurol
Pays: India
ID NLM: 101273955
Informations de publication
Date de publication:
Historique:
received:
27
07
2020
revised:
02
08
2020
accepted:
14
10
2020
entrez:
27
8
2021
pubmed:
28
8
2021
medline:
28
8
2021
Statut:
ppublish
Résumé
Anti-N-methyl D-aspartate receptor (anti NMDAR) antibody encephalitis is an immune-mediated entity characterised by a constellation of neuro-psychiatric symptoms. To describe clinical profile and treatment outcomes of patients with anti NMDAR antibody encephalitis. Subjects were selected by screening for all patients satisfying Graus A prospective, longitudinal study was conducted by identifying 25 patients with anti NMDAR antibodies in CSF and or serum, between September 2018 to February 2020. Chi square test was used to compare variables. Out of 98 patients screened, 25 subjects (14 females: 11 male) were positive for anti NMDAR autoantibodies, with a mean age of 17 years. 13 subjects belonged to paediatric age group. Most common presenting feature was memory/learning deficit (88%) followed by behavioural abnormalities (84%) and seizures (68%). 11 patients (44%) patients needed escalation to second line therapy, rituximab. Seven (28%) and twelve (48%) patients underwent complete (mRS 0-1) and partial recovery (mRS 2-3) respectively, while 4 (16%) became disabled (mRS 4-5). Mortality was 8%. Paediatric population had a better outcome in terms of disability (p = 0.043). Anti NMDAR-Ab encephalitis is the most common cause of antibody positive autoimmune encephalitis worldwide. There are important clinical markers and investigational profiles which carry prognostic significance.
Sections du résumé
BACKGROUND
BACKGROUND
Anti-N-methyl D-aspartate receptor (anti NMDAR) antibody encephalitis is an immune-mediated entity characterised by a constellation of neuro-psychiatric symptoms.
OBJECTIVE
OBJECTIVE
To describe clinical profile and treatment outcomes of patients with anti NMDAR antibody encephalitis.
SETTINGS AND DESIGN
METHODS
Subjects were selected by screening for all patients satisfying Graus
MATERIALS AND METHODS
METHODS
A prospective, longitudinal study was conducted by identifying 25 patients with anti NMDAR antibodies in CSF and or serum, between September 2018 to February 2020.
STATISTICAL ANALYSIS
METHODS
Chi square test was used to compare variables.
RESULTS
RESULTS
Out of 98 patients screened, 25 subjects (14 females: 11 male) were positive for anti NMDAR autoantibodies, with a mean age of 17 years. 13 subjects belonged to paediatric age group. Most common presenting feature was memory/learning deficit (88%) followed by behavioural abnormalities (84%) and seizures (68%). 11 patients (44%) patients needed escalation to second line therapy, rituximab. Seven (28%) and twelve (48%) patients underwent complete (mRS 0-1) and partial recovery (mRS 2-3) respectively, while 4 (16%) became disabled (mRS 4-5). Mortality was 8%. Paediatric population had a better outcome in terms of disability (p = 0.043).
CONCLUSION
CONCLUSIONS
Anti NMDAR-Ab encephalitis is the most common cause of antibody positive autoimmune encephalitis worldwide. There are important clinical markers and investigational profiles which carry prognostic significance.
Identifiants
pubmed: 34447002
doi: 10.4103/aian.AIAN_817_20
pii: AIAN-24-383
pmc: PMC8370154
doi:
Types de publication
Journal Article
Langues
eng
Pagination
383-389Informations de copyright
Copyright: © 2006 - 2021 Annals of Indian Academy of Neurology.
Déclaration de conflit d'intérêts
There are no conflicts of interest.
Références
Neurology. 2011 Sep 6;77(10):996-9
pubmed: 21865579
BJPsych Bull. 2015 Feb;39(1):32-5
pubmed: 26191422
J Pediatr Neurosci. 2018 Oct-Dec;13(4):423-428
pubmed: 30937083
Brain. 2010 Jun;133(Pt 6):1655-67
pubmed: 20511282
Eur J Pediatr. 2014 May;173(5):681-3
pubmed: 23703469
Arq Neuropsiquiatr. 2018 Jan;76(1):41-49
pubmed: 29364393
Neurologia. 2007 Oct;22(8):526-37
pubmed: 18000762
Eur J Paediatr Neurol. 2015 Jul;19(4):453-63
pubmed: 25792293
Clin Infect Dis. 2012 Apr;54(7):899-904
pubmed: 22281844
Lancet Infect Dis. 2010 Dec;10(12):835-44
pubmed: 20952256
Lancet Neurol. 2013 Feb;12(2):157-65
pubmed: 23290630
Am J Respir Crit Care Med. 2017 Feb 15;195(4):491-499
pubmed: 27552490
Pediatr Neurol. 2014 May;50(5):507-10
pubmed: 24656207
Dev Med Child Neurol. 2014 Aug;56(8):794-6
pubmed: 24641688
J Neurol. 2018 Feb;265(2):362-369
pubmed: 29249055
Epilepsy Behav. 2017 Mar;68:57-65
pubmed: 28109991
Eur J Neurol. 2016 Mar;23(3):621-9
pubmed: 26563553
Lancet Neurol. 2011 Jan;10(1):63-74
pubmed: 21163445
Lancet Psychiatry. 2019 Mar;6(3):235-246
pubmed: 30765329
Ann Neurol. 2007 Jan;61(1):25-36
pubmed: 17262855
Pediatr Neurol. 2016 Oct;63:71-72
pubmed: 27590992
Epilepsia. 2017 Dec;58(12):2104-2111
pubmed: 29098690
Lancet Neurol. 2016 Apr;15(4):391-404
pubmed: 26906964
Neurology. 2014 Feb 18;82(7):556-63
pubmed: 24443452
Neurol Neuroimmunol Neuroinflamm. 2019 Jun 04;6(4):e579
pubmed: 31355315
N Engl J Med. 2018 Mar 1;378(9):840-851
pubmed: 29490181
Front Neurol. 2019 Jun 06;10:596
pubmed: 31244759
Indian J Community Med. 2012 Oct;37(4):240-51
pubmed: 23293439
Lancet Neurol. 2014 Feb;13(2):167-77
pubmed: 24360484