Pediatric Anti-<i>N</i>-Methyl-<scp>D</scp>-Aspartate (NMDA) Receptor Encephalitis

Chakrabarty, Biswaroop; Tripathi, Manjari; Gulati, Sheffali; Yoganathan, Sangeetha; Pandit, Awadh Kishor; Sinha, Aditi; Rathi, Bhim Singh

doi:10.1177/0883073813494474

Cited by 25 publications

(3 citation statements)

References 20 publications

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“…[910] Treatment options are plasmapheresis, steroids, IVIGs, second-line immunomodulators when first line fails, and tumor removal whenever detected. [1112] The antibodies could be present or absent in the serum, CSF, or both, and therefore both need to be examined at least at the first instance. [131415]…”

Section: Anti-nmdar Antibody Syndromementioning

confidence: 99%

N-methyl-d-aspartate encephalitis our experience with diagnostic dilemmas, clinical features, and outcome

et al. 2018

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Introduction:A neuropsychiatric syndrome characterized by a wide spectrum of clinical manifestations. It is seen in patients with antibodies against NR1-NR2 heteromers of the NMDA receptor. As the spectrum is mainly psychiatric most patients are treated as psychiatric disease resulting in huge diagnostic delay.Patient and methods:Here we describe 29 patients with NMDA encephalitis seen by the authors in the last five years. Percentage of Transfected cells showing granular cytoplasmic florescence was considered for positivity and severity both in CSF and serum. Their presenting diagnosis, clinical features and the dilemmas, alarming gaps, laboratory data, response to treatment and relapses are discussed.Observations:All patients presented with a spectrum of psychiatric symptoms varying from panic to severe aggression, seizures, chorea, hemiplegia, catatonia, mitgehen, mutism, delirium, mania and memory problems. EEG is invariably abnormal as against imaging.Conclusion:NMDA receptor mediated encephalitis should be suspected in all children and females of adolescent age with refractory neuropsychiatric syndrome. Both CSF and serum should be tested and regular follow up for relapses and neoplasms is mandatory.

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Section: Anti-nmdar Antibody Syndromementioning

confidence: 99%

N-methyl-d-aspartate encephalitis our experience with diagnostic dilemmas, clinical features, and outcome

et al. 2018

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“…There are very few studies of AIE in children reported from India[56] and this is the first clinical series of seronegative AIE. Antibody-negative AIE behaves similarly in the initial clinical presentation, response to immunotherapy and long-term outcome.…”

Section: Discussionmentioning

confidence: 99%

Dilemmas and challenges in treating seronegative autoimmune encephalitis in Indian children

Sahoo

Jain

Mishra

et al. 2018

Indian Journal of Critical Care Medicine

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Objective:The main objective is to assess the challenges in diagnosis and treatment while managing seronegative cases of autoimmune encephalitis (AIE) in Indian children.Methods:A cohort study of patients with AIE was done where clinical presentations, investigations, management were analyzed and these patients were followed up to assess the evolution of the disease.Results:Nine patients were included in the study. Four patients presented with super-refractory status epilepticus (SRSE). Other presentations were behavioral change, hemiplegia, and autonomic dysfunction. Initial magnetic resonance imaging brain was suggestive of AIE in two patients. Only two were seropositive for cerebrospinal fluid (CSF) autoimmune panel. Five patients responded to the first-line immunotherapy and four required the second-line immunotherapy.Conclusion:The possibility of autoimmune encephalitis should be considered in patients with super-refractory status epilepticus. A large proportion of children with suspected AE may be “seronegative.” A trial of immunotherapy should be given to these children when there is a strong clinical suspicion of autoimmune encephalitis even in the absence of cerebrospinal fluid autoantibodies.

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“…59 A North Indian study of 11 children with NMDAR-AbE reported significant response to steroids and immunoglobulin in 58% of patients. 60 In a prospective Indian study of 15 patients aged 2-64 years with AIE, 67% of patients who were treated with IVIG, steroids or both showed significant improvement, with no further seizures or clinical relapse years at follow-up. 61 A single-arm, open-label study of 41 adult patients with possible autoimmune encephalitis demonstrated a clinical benefit from IVIG with significant improvement in neurological functional outcomes following IVIG treatment.…”

Section: Intravenous Immunoglobulin In Autoimmune Encephalitismentioning

confidence: 99%

Intravenous immunoglobulin treatment for encephalitis in children aged 6 months to 16 years: the IgNiTE RCT

Iro,

Sadarangani,

Absoud

et al. 2024

Efficacy Mech Eval

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Background There are data suggesting that intravenous immunoglobulin treatment has some benefit for certain forms of encephalitis but robust evidence from large randomised controlled trials in children with all-cause encephalitis is lacking. Objective To evaluate whether intravenous immunoglobulin treatment improves neurological outcomes in childhood encephalitis when given early in the illness. Design Phase 3b, investigator-initiated, randomised, double-blind, placebo-controlled trial of intravenous immunoglobulin for the treatment of encephalitis in children. Setting Twenty-one NHS Hospitals in the UK. Participants Children aged 6 months to 16 years with a diagnosis of acute or sub-acute encephalitis. Intervention Two doses (1 g/kg/dose) of either intravenous immunoglobulin or matching placebo, given 24–36 hours apart, in addition to standard treatment. Main outcome measure Participants were followed up for 12 months (+/– 4 weeks) after randomisation. The primary outcome measure was a ‘good recovery’ defined as a score of ≤ 2 on the Paediatric Glasgow Outcome Score Extended at 12 months after randomisation. Secondary outcomes The secondary outcomes were clinical, neurological, neuroimaging and neuropsychological results, identification of the proportion of children with immune-mediated encephalitis, and intravenous immunoglobulin safety data. Results We planned to recruit 308 children over a 42-month period. After enrolment of 18 participants (8 male; 44%) over 21 months (from December 2015 to September 2017), funding was withdrawn due to slow recruitment and the study was terminated. Ten participants were randomised to the intravenous immunoglobulin group, and eight to the placebo group, and all 18 participants were included in the analysis. At 12 months after randomisation, 9 participants [50%; intravenous immunoglobulin n = 5 (50%), placebo n = 4 (50%)] made good recovery and 5 participants [28%; intravenous immunoglobulin n = 3 (30%), placebo n = 2 (25%)] made a poor recovery. Three participants in the placebo group (43%) experienced a total of 10 serious adverse events compared with none in the intravenous immunoglobulin group but none of the adverse events were judged to be related to the study treatment. No deaths occurred during the study period. Conclusion ImmunoglobuliN in the Treatment of Encephalitis (IgNiTE) was halted prematurely due to slow recruitment. Given the small sample size, the study was underpowered to evaluate the effect of intravenous immunoglobulin when compared with placebo in childhood encephalitis. The study findings, albeit from a small sample size, support existing evidence that encephalitis results in poor neurological outcomes for many children. Lessons learned from the ImmunoglobuliN in the Treatment of Encephalitis trial would be valuable for the success of future trials set up to address the efficacy of early treatment with intravenous immunoglobulin in all-cause encephalitis in children. Study limitations and future work The study was underpowered to evaluate the efficacy of intravenous immunoglobulin in the treatment of childhood encephalitis due to the small sample size achieved. Future trials should seek to address this important question. Trial registration This trial is registered as Clinical Trials.gov (NCT02308982) and ISRCTN15791925. Funding This award was funded by the National Institute for Health and Care Research (NIHR) Efficacy and Mechanism Evaluation (EME) programme (NIHR award ref: 12/212/15) and is published in full in Efficacy and Mechanism Evaluation; Vol. 11, No. 6. See the NIHR Funding and Awards website for further award information.

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Pediatric Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis

Cited by 25 publications

References 20 publications

N-methyl-d-aspartate encephalitis our experience with diagnostic dilemmas, clinical features, and outcome

N-methyl-d-aspartate encephalitis our experience with diagnostic dilemmas, clinical features, and outcome

Dilemmas and challenges in treating seronegative autoimmune encephalitis in Indian children

Intravenous immunoglobulin treatment for encephalitis in children aged 6 months to 16 years: the IgNiTE RCT

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