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Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

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  • Emily
    A probable case of anti-NMDAR encephalitis from 1830
    Josep Dalmau

    Neurol Neuroimmunol Neuroinflamm Nov 2020, 7 (6) e901; DOI: 10.1212/NXI.0000000000000901
    • Copyright ? 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
    This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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    For those of you who enjoy a good detective story and
    medical mystery, I will end this Editor’s Corner with the
    contribution of T?enyi et al.6 While reviewing the historical
    medical literature, these authors found reference to the same
    case report in medical journals published in Hungarian, German,
    and Italian. The case was presented at the 3rd meeting of
    the Italian Scientists held in Florence in 1841. The patient
    presented in 1830. She was a previously healthy 18-year-old
    woman who developed seizures, followed by a 6-day-long
    clinical state characterized by catalepsy, unresponsiveness, and
    shallow breathing. These symptoms apparently resolved but
    returned over the following year and a half during which a
    progressively growing abdominal tumor was noted. One day,
    she suddenly developed emesis that included pus and blood
    mixed with more than 100 bone fragments. At the same time,
    she had evacuation from the anus and vagina of a membranous
    substance mixed with blood and pus. After this event, the
    seizures and the catalepsy-like episodes disappeared. Over the
    following years, she gave birth to 3 healthy children and was
    still well 11 years later. The authors suggest that this represents
    a case of anti-NMDA receptor (NMDAR) encephalitis. They
    postulate that the catalepsy-like state was catatonia, which was
    not defined as an entity for more than 40 years after this
    patient’s presentation. They suggest that the mass was an
    ovarian teratoma, which can reach large sizes, contain bone and
    perforate into the bowel and vagina, whereas the shallow
    breathing was likely because of hypoventilation. The authors
    remind us that this description of a likely paraneoplastic syndrome
    from 1830 predates by 35 years that of Trousseau
    syndrome, which is considered the first paraneoplastic syndrome
    reported. The case is fascinating and reminds us of
    complications in patients with anti-NMDAR encephalitis
    (continuous tumor growth despite partial neurologic improvement
    and bowel perforation)7 that were included in the
    first description of this disease and are now rarely encountered
    due early recognition of this disorder...

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  • Emily
    Copyright ? 2020 Vijay Renga. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

    Case Report | Open Access
    Volume 2020 |Article ID 3796807 | 3 pages |
    Into a Shaking Limbo: Case Report of a Nonneoplastic Limbic Encephalitis with Faciobrachial Dystonic Seizures and Parkinsonism
    Vijay Renga Department of Neurology, Dartmouth Hitchcock Medical Center, Geisel School of Medicine at Dartmouth, One Medical Center Drive, Lebanon, NH 03756, USA
    Academic Editor: Majaz Moonis
    Received26 Aug 2019
    Revised25 Jan 2020
    Accepted11 Feb 2020
    Published15 Mar 2020


    This case report describes a rare but classic presentation of a non-paraneoplastic, antibody-mediated limbic encephalitis. The clinical course did put us in a limbo as it evolved from seizure to Parkinsonism and then from metastasis to stroke, before it finally announced itself by its pathognomonic finding. Knowledge of this rare condition is important as early identification and treatment can change the course...
    Doctors Completely Missed My Brain Swelling and Sent Me to a Mental Health Facility for Schizophrenia Instead
    When Olivia Palermo, 19, noticed a twitch in her finger, she blamed dehydration.

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  • Emily
    After Five Months in a Coma, Young Nurse Wakes Up

    Chicago RN with rare autoimmune encephalitis responds to unusual treatment

    by Judy George, Senior Staff Writer, MedPage Today January 10, 2020

    VanHaerents kept searching for an answer, calling colleagues around the country. He found case reports demonstrating how bortezomib (Velcade), a drug approved for multiple myeloma, had been used to treat several refractory cases of anti-NMDA receptor encephalitis. "Bortezomib is a proteasome inhibitor; it predisposes both short- and long-term plasma cells to apoptotic death," VanHaerents explained.

    At least two case series had been published about severe encephalitis patients who had been treated with the drug in Germany, including one in JAMA Neurology and one in Neurology.

    Then VanHaerents heard about another case at a large academic U.S. hospital. They told him their patient started to wake up after bortezomib therapy. "Their 'n of 1' was good," he said.

    "I had to convince our pharmacy and our critical care team that I wanted to try this medication based on these cases," he recalled. "They agreed. And so we did."

    And it worked.

    Braker received her first dose of bortezomib on August 9, 2018. A few weeks after starting bortezomib and 5 months after she first became comatose -- she began to wake up.
    ECT was then used to enhance her recovery.

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  • Emily
    AUTHOR=Tzang Ruu-Fen, Chang Chuan-Hsin, Chang Yue-Cune, Lane Hsien-Yuan

    Autism Associated With Anti-NMDAR Encephalitis: Glutamate-Related Therapy

    Frontiers in Psychiatry
    VOLUME=10 YEAR=2019 PAGES=440


    DOI=10.3389/fpsyt.2019.00440 ISSN=1664-0640

    ABSTRACT=The purpose of this review is to correlate autism with autoimmune dysfunction in the absence of an explanation for the etiology of autism spectrum disorder. The anti-N-methyl-D-aspartate receptor (anti-NMDAR) autoantibody is a typical synaptic protein that can bind to synaptic NMDA glutamate receptors, leading to dysfunctional glutamate neurotransmission in the brain that manifests as psychiatric symptoms (psychosis, hallucinations, and personality changes). Detection of autoantibodies, cytokines, decreased lymphocytes, serum immunoglobulin level imbalance, T-cell mediated immune profile, maternal infection history, and children’s infection history can all be vital biological markers of autoimmune autism. Diagnosing autoimmune encephalitis sooner can increase the effectiveness of curative treatments—such as immune therapy or immune modulatory therapy—that may prevent the long-term consequence of being misdiagnosed with autism spectrum disorder. Glutamate therapy primarily normalizes glutamate neurotransmission and can be a new add-on intervention alongside antipsychotics for treating autoimmune autism.

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  • Emily
    Cai X, Zhou H, Xie Y, Yu D, Wang Z, Ren H. Anti-N-methyl-d-aspartate receptor encephalitis associated with acute Toxoplasma gondii infection: A case report. Rosca. EC, ed. Medicine. 2018;97(7):e9924. doi:10.1097/MD.0000000000009924. Abstract

    Go to:

    Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis has been recognized as the most frequent autoimmune encephalitis in children. Several infectious agents have been implicated in anti-NMDA encephalitis.

    Go to:
    Patient concerns:

    A previously healthy immunocompetent 9-year-old girl first presented with seizures, headaches and vomiting. Cerebrospinal fluid and brain magnetic resonance imaging were normal. After one week onset, the patient gradually developed unexplained personality and behavior changes, accompanied by fever and seizures again. Repeated CSF analysis revealed a slightly lymphocytic predominant pleocytosis and positive anti-NMDAR antibody. A variety of pathogenic examinations were negative, except for positive toxoplasma IgM and IgG.

    Go to:

    The patient was diagnoses for anti-NMDA encephalitis associated with acute acquired toxoplasma gondii infection.

    Go to:

    The patient received 10 days azithromycin for treatment of acquired toxoplasma infection. The parents refuse immunotherapy because substantial recovery from clinical symptoms.

    Go to:

    The patient was substantially recovered with residual mild agitation after therapy for acquired toxoplasma gondii infection. Two months later, the patient was completely devoid of symptoms, and the levels of serum IgM and IgG of toxoplasma gondii were decreased.

    Go to:

    Acquired toxoplasma gondii infection may trigger anti-NMDAR encephalitis in children, which has not been reported previously. Clinicians should assess the possibility of toxoplasma gondii infection when evaluating a patient with anti-NMDA encephalitis.

    Keywords: anti-NMDA receptor encephalitis, children, Toxoplasma gondii infection
    Full text at link.

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  • Emily
    Hoang, Mai Nguyen Thi, et al. "First reported cases of anti-NMDA receptor encephalitis in Vietnamese adolescents and adults." Journal of the Neurological Sciences 373 (2017): 250-253. Open Access funded by Wellcome Trust
    Under a Creative Commons license

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  • Emily
    Family pay tribute to 'beautiful, selfless and loving' woman who died in A1(M) crash near Stevenage

    By Matthew_MH | Posted: April 21, 2017

    Five years ago, the close-knit family endured heartache when Amelia's cousin Yasmin Johnson died at the age of 18.
    She had a rare illness called anti NMDA receptor encephalitis, which means antibodies produced by the body's own immune system attack nerve receptors in the brain.
    Olivia, who recently moved out of the family home with Nina, said: "I know Amelia never got over that.
    "She and Yasmin were so close, like the best of friends. They grew up together, and Yasmin was like another sister to us. It's a comfort to know that they are together again now."..

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  • Emily
    Antecedent anti-NMDA receptor encephalitis in two patients with multiple sclerosis

    Baheerathan, A. et al.
    Multiple Sclerosis and Related Disorders , Volume 12 , 20 - 22

    • ?Overlap has been shown between anti-NMDAR encephalitis and demyelinating disorders.
    • ?NMDAR antibody testing should be considered in atypical relapses of MS.


    Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder characterised by psychiatric symptoms, movement disorder and seizures often evolving into a severe encephalopathy. An overlap has recently been recognised between anti-NMDAR encephalitis and inflammatory demyelinating disorders, particularly neuromyelitis optical spectrum disorder (NMOSD).
    In this case report, we describe two patients with an initial presentation consistent with anti-NMDAR encephalitis who have subsequently developed relapsing-remitting multiple sclerosis (MS) and discuss the literature pertaining to potential overlap between NMDAR encephalitis and inflammatory demyelinating disorders.

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  • Emily
    Galli, J., Clardy, S.L. & Piquet, A.L. Curr Infect Dis Rep (2017) 19: 1. doi:10.1007/s11908-017-0556-y
    NMDAR Encephalitis Following Herpes Simplex Virus Encephalitis


    Purpose of Review

    Herpes simplex virus encephalitis (HSVE) is often associated with significant morbidity and mortality, and despite appropriate treatment with antivirals, worsening of neurological symptoms or relapse occurs in a subset of patients. Recent data suggests that many relapses are likely caused by a secondary immune response, with the N-methyl-D-aspartate receptor (NMDAR) antibody being the most commonly associated autoantibody. We provide a review of the relevant literature, examining the relationship between HSVE and development of autoimmunity.

    Recent Findings

    Autoantibodies, including pathogenic NMDAR antibody, have been demonstrated in the cerebrospinal fluid (CSF) of patients following HSVE. This occurs usually several weeks following initial HSV infection.


    There is growing evidence of a relationship between HSVE and the subsequent development of NMDAR encephalitis. Possible mechanisms include molecular mimicry or an immune response to direct neuronal damage. Future studies should address if the use of immunotherapy can prevent the development of autoimmunity following HSVE.


    N-methyl-D-aspartate receptor (NMDAR) antibody Herpes simplex virus Encephalitis Autoimmune Cell surface antibody
    This article is part of the Topical Collection on Neurological Infections

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  • Emily
    Excellent article by a mother and founder of Canada's Anti-NMDA Receptor Encephalitis Foundation support group.
    Not So Rare But Rarely Diagnosed: From Demonic Possession to Anti-NMDA Receptor Encephalitis

    Nesrin Shaheen
    August 24, 2016

    There is growing consensus in the medical community that many prior accounts of ?demonic possession? may have represented original accounts of what is now broadly known as ?autoimmune encephalitis?. This term unifies a broad number of autoimmune diseases, which all result in severe symptomatic brain inflammation owing to an antibody-mediated attack on central nervous system tissues that is sufficient to account for the clinical presentation. Perhaps the best understood medical surrogate for demonic possession is the recently characterized diagnosis of anti-NMDA receptor encephalitis (NMDARE). To the patient or their loved ones, it is a malady like no other, characterized by the rapid onset of symptoms so fierce and so encompassing, that the patient is often described ?as if possessed?.

    The first diagnosed case of NMDARE in Canada occurred in a 12-year-old girl seen at the Children?s Hospital of Eastern Ontario (Ottawa, Ontario) in 2008. Symptoms began insidiously with memory loss following a flu-like illness. The child would ask a question of her mother, and then following an explanation, would ask it again. Routine blood tests and neurological testing in the local emergency department were unrevealing; the symptoms were attributed to anxiety and the patient was discharged to the care of her general practitioner...

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  • Ronan Kelly

    GUNTUR, July 22, 2016
    Updated: July 22, 2016 06:05 IST
    Girl battles Rasmussen?s encephalitis


    A four-year-old girl diagnosed with a rare autoimmune disorder, Rasmussen?s encephalitis, is being treated by neurologists at the Government General Hospital here.

    Rasmussen, 92, a neurosurgeon had done extensive research on the disease, and identified the disease as having a poor prognosis. The symptoms of the disease, also known as chronic focal encephalitis, include seizures and paralysis of left upper and lower limb. The patient will continue to have seizures which is termed as epilepsia partialis continua despite using multiple anti-epileptic drugs.

    After conducting a series of clinical tests and collaborating with imaging evidence, Head of Department of Neurology N.V. Sundarachary said that though the exact cause was not known, the disorder could be due to sub-acute chronic infection or autoimmune attack on Glutamate 3 receptors in the brain.

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  • Emily
    Colban's story - a boy with a rare disease, in a rural area


    Colban Mcleod was a healthy 5 year old boy.
    But last August, everything changed.
    Sorin says it was hard to watch her grandson suffer, "it was just so overwhelming...his seizures started getting really bad. One morning he had more than 50 seizures in 5 hours - they lost count after 50.?
    His MRI came back normal. But a lumbar puncture, otherwise known as a spinal tap, revealed Colban had anti-NMDA receptor encephalitis ? a rare auto-immune disease...
    Canada's Anti-NMDA Receptor Encephalitis Foundation, founded by mother whose daughter was the first positively identified case in Canada in 2008:

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  • Emily
    Seronegative autoimmune encephalitis - struggle to find diagnosis and treatment:
    The Wonder Years: Inside a Medical Mystery

    We spent the prime of our daughter?s childhood in doctors? offices and hospital clinics. No one could tell us what was wrong with Rory?a medical mystery that not only took a toll on her health, but on our family as well.

    April 26, 2016Alicia Garceau
    The Autoimmune Encephalitis Alliance:

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  • Emily
    Almuslamani A, Mahmood F. First Bahraini adolescent with anti-NMDAR-Ab encephalitis. Qatar Medical Journal. 2015;2015(1):2. doi:10.5339/qmj.2015.2. Abstract

    Anti-N-methyl-D-aspartate-receptor (NMDA-R) encephalitis is a new autoimmune, often paraneoplastic disorder that presents with complex neuropsychiatric symptoms. It was first described in 2007 by Dalmau et al. Our patient presented with headache, behavioral changes and then seizures with hallucinations. She was initially misdiagnosed to have schizophrenia and was prescribed antipsychotics. She deteriorated and developed further seizures with hypoventilation and choreoathetosis. Her blood investigations were positive for mycoplasma IGM. Her CSF studies showed high white cell counts, predominantly lymphocytes, and high anti-NMDA-R titre. Her brain MRI scans showed high tbl2 and FLAIR intensities in the grey and white matter of the left cerebellar hemisphere suggestive of acute disseminated encephalomyelitis. She responded to treatment with antibiotics, multiple antiepileptics, steroids and needed five sessions of plasmapheresis. There was no underlying malignancy on repeated scanning of the abdomen. She needed around one year for full recovery with intensive rehabilitation. The objective of this paper was to highlight the occurrence of this fairly new, challenging, easily missed, not-so-rare form of encephalitis often occurring in the absence of fever.

    Keywords: anti-N-methyl-D-aspartate receptor antibody (anti-NMDAR-Ab), encephalitis, central nervous system (CNS), acute disseminated encephalomyelitis (ADEM)
    Full text at link.

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  • Emily
    The Tohoku Journal of Experimental Medicine Vol. 235 (2015) No. 3 March p. 223-231

    Expression of N-Methyl-D-Aspartate Receptor Subunits in the Bovine Ovum: Ova as a Potential Source of Autoantigens Causing Anti-NMDAR Encephalitis

    Naoko Tachibana1), Michiaki Kinoshita2), Fuyuki Kametani3), Keiko Tanaka4) 5), Yumi Une6), Yotaro Komatsu7), Yukihiro Kobayashi8), Shu-ichi Ikeda2) Abstract

    Autoimmune synaptic encephalitis is characterized by the presence of autoantibodies against synaptic constituent receptors and manifests as neurological and psychiatric disorders. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is such an autoimmune disorder that predominantly affects young women. It is associated with antibodies against the extracellular region of the NR1 subunit of postsynaptic NMDAR. Each NMDAR functions as a heterotetrameric complex that is composed of four subunits, including NR1 and NR2A, NR2B, or NR2C. Importantly, ovarian teratoma is a typical complication of anti-NMDAR encephalitis in female patients and may contain antigenic neural tissue; however, antigenic sites remain unknown in female patients without ovarian teratoma. The purpose of this study was to investigate the expression of NMDARs in the ovum. We detected NR1 and NR2B immunoreactivity in protein fractions extracted from the bovine ovary and ova by SDS-polyacrylamide gel electrophoresis and immunoblotting analysis. Immunoprecipitates digested with trypsin were analyzed by reverse phase liquid chromatography coupled to tandem mass spectrometry. We obtained the following five peptides: SPFGRFK and KNLQDR, which are consistent with partial sequences of human NR1, and GVEDALVSLK, QPTVAGAPK, and NEVMSSK, which correspond to those of NR2A, NR2B and NR2C, respectively. Immunocytochemical analysis revealed that the bovine ovum was stained with the immunoglobulin G purified from the serum of a patient with anti-NMDAR encephalitis. Taken together, we propose that the normal ovum expresses NMDARs that have strong affinity for the disease-specific IgG. The presence of NMDARs in ova may help explain why young females without ovarian teratomas are also affected by anti-NMDAR encephalitis.
    Full text at link.

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