Autoimmune Encephalitis beyond NMDA-R Antibodies

Autoimmune Encephalitis beyond NMDA-R Antibodies Josep Dalmau (SJD Barcelona Children's Hospital, Spain)


March 27, 2021
  • Josep Dalmau
CPD/CME Credits

Autoimmune Encephalitis beyond NMDA-R Antibodies
Josep Dalmau (SJD Barcelona Children's Hospital, Spain)

Autoimmune encephalitis is a new category of diseases in which autoantibodies against neuronal cell surface and synaptic proteins cause a wide variety of syndromes and diseases resulting in neuropsychiatric manifestations. The spectrum of diseases, comorbidities and triggers is different between adults and children and symptoms vary according to the type of autoantibody. In children the most frequent autoimmune encephalitis is anti-NMDAR followed by a few others such as anti-MOG (myelin oligodendrocyte glycoprotein), anti-GABAaR, and other. In this presentation I will review the spectrum of these diseases in children, some of the underlying pathogenic mechanisms involved, and the approaches to treatment.

Learning Objectives

  • Clinically recognize anti-NMDA receptor and other autoimmune encephalitis
  • Optimally manage the initial treatment approach of anti-NMDAR encephalitis
  • Describe the underlying antibody-mediated pathogenic mechanisms in anti-NMDAR and other types of autoimmune encephalitis.

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  • This commment is unpublished.
    Shahnaz Ibrahim · 26 days ago
    This is shahnaz ibrahim .Pakistan .it usually takes a week to get the results for antibodies . I In herpes ENCEPHALITIS should we start 1st line based on suspicion with acyclovir if the child has behavioural changes seizures and movement disorder
  • This commment is unpublished.
    Amal Ben kacem · 26 days ago
    if an anti-NMDA encephalitis is associated with anti-NMO or anti-MOG Abs,does this association affect the st rategy of treatement ?Or should we control the Abs after a period of time ?
  • This commment is unpublished.
    Sahar Hassanein · 26 days ago
    What immune therapy you recommend for treatment of AntiGABAa receptor antibody?
  • This commment is unpublished.
    Maryam Nabavi Nouri · 26 days ago
    Dr. Dalmau, Can you let me know what your experience has been with delayed (retrospective) diagnosis of NMDARE (diagnosed 10 years after in severely cognitively impacted child), is there room for immunotherapy (i.e. Rituximab)
  • This commment is unpublished.
    Yanwen Shen · 26 days ago
    Does there any biomarkers in children who would be more likely to develop anti-NMDA encephalitis after HSV infection
  • This commment is unpublished.
    Dr Sonal Bhatia · 26 days ago
    what is your experience on AchR ganglionic neuronal Ab mediated AE in children?
  • This commment is unpublished.
    Raghda Zaitoun · 26 days ago
    is it standard in post HSV anti NMDAR encephalitis to use combined Ritux and Cytx? i mean before giving one or the other a chance? In this same group of patients can we proceed with immune suppressive therapy in HSV PCR in CSF is still positive? Last: do we not try IVPMP/IVIG/PLEX before proceeding to either Cytx/Ritux or both?? Raghda Zaitoun, Cairo, Egypt
  • This commment is unpublished.
    Vrajesh Udani · 26 days ago
    Vrajesh Udani from Mumbai: In refractory AE how long do you treat with 2nd / 3rd line therapies - and how does one decide whether activity persists or the patient now has sequelae
  • This commment is unpublished.
    Alireza Tavasoli · 26 days ago
    Dear Prof. Dalmau, Thank you for outstanding presentation. As you mentioned, there are many unknown in this field especially in children. Could you tell us which criteria could be considered as first line treatment failure? continuing of seizures, persistent of movement disorders, psychiatry symptoms? and the other important question is how many days we have to stay for seeing response to first line treatment?
  • This commment is unpublished.
    Fous Sehu Lebbe · 26 days ago
    Dr Fous from Sri Lanka, If they present with myoclonic jerk and catatonia, we used to treat with 2nd line drugs earlier and see some good recovery. I would like have your expert opinion on this. Thanks.
  • This commment is unpublished.
    Shahnaz Ibrahim · 26 days ago
    What would be the first line in herpes simplex related NMDA R encephalitis .and would rituxamab and or cyclophosphamide be safe
  • This commment is unpublished.
    Farida Essajee · 26 days ago
    How would you define response to treatment in autoimmune encephalitis. Farida / South Africa
  • This commment is unpublished.
    Thashi Chang · 26 days ago
    What is the sensitivity and specificity of NMDAR antibodies in CSF? Thashi Chang Sri Lanka
  • This commment is unpublished.
    Dr Gouri Passi · 26 days ago
    What is the reason for sleep disturbances in anti nmdar encephalitis?
  • This commment is unpublished.
    Raghda Zaitoun · 26 days ago
    i can see that relapses are less frequent in patients who have 2nd line immune suppressive therapy...So should we always go for 2nd line even if the patients respond to the 1st line treatment? Raghda Zaitoun. Cairo, Egypt
  • This commment is unpublished.
    Minal Kekatpure · 26 days ago
    Should the CSF antibodies be rechecked before discontinuing treatment? Dr Minal , India
  • This commment is unpublished.
    Sehu Lebbe Fous · 26 days ago
    I am Dr Fous from Sri Lanka, What's your opinion on Methotrexate as a 2nd line treatment?
  • This commment is unpublished.
    Dipak Ram · 26 days ago
    After how long of giving 1st line therapy would you consider that someone has failed to respond - 2 weeks or less/more? Thanks, Dipak Ram, Manchester, UK
  • This commment is unpublished.
    Bidisha · 26 days ago
    Are there environmental or modifiable triggers to autoimmune encephalitis ? Dr. Bidisha Banerjee, India
  • This commment is unpublished.
    Ryan MCGinn · 26 days ago
    Just to clarify regarding 1st, 2nd line therapies - if you had given a steroid pulse and some maintenance, and this failed, would you then move to Rituximab/Cyclophosphamide, or would a trial of IVIG or PLEX still be warranted in 1st line therapy?