Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

[Emily]

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5839864/

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. [h=2]Abstract[/h] Go to:
[h=2]Rationale:[/h] 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.

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[h=2]Patient concerns:[/h] 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.

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[h=2]Diagnoses:[/h] The patient was diagnoses for anti-NMDA encephalitis associated with acute acquired toxoplasma gondii infection.

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[h=2]Interventions:[/h] The patient received 10 days azithromycin for treatment of acquired toxoplasma infection. The parents refuse immunotherapy because substantial recovery from clinical symptoms.

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[h=2]Outcomes:[/h] 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.

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[h=2]Lessons:[/h] 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.

 

[Emily]

https://www.frontiersin.org/articles/10.3389/fpsyt.2019.00440/full

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

URL=https://www.frontiersin.org/article/10.3389/fpsyt.2019.00440

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.

 

[Emily]

https://www.medpagetoday.com/neurology/generalneurology/84296

[h=1]After Five Months in a Coma, Young Nurse Wakes Up[/h] [h=2]Chicago RN with rare autoimmune encephalitis responds to unusual treatment[/h] 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.

 

[Emily]

https://www.hindawi.com/journals/crinm/2020/3796807/

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 | https://doi.org/10.1155/2020/3796807
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

Abstract

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...

https://headtopics.com/us/doctors-co...hreni-12148316

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]

https://nn.neurology.org/content/7/6/e901.abstract

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...