Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Case in a 34-yr-old male reported in Australia:

Started out with flu-like symptoms and a headache, ended up in an induced coma on a ventilator:
http://www.inspire.com/groups/encephalitis-global/discussion/my-story-so-far/

Got the diagnosis in the next thread. Note that the mother of one of the patients mentioned earlier in the thread comments and says that her daughter was told she had the flu for a month last year prior to the anti-NMDAR diagnosis:
http://www.inspire.com/groups/encephalitis-global/discussion/finally-a-diagnosis-for-pete/


The article below shows that there isn't consensus yet on whether this illness is new.

http://cumc.columbia.edu/features/quick-diagnosis-rare-disease-leads-remarkable-recovery

September 1, 2010

Quick Diagnosis of Rare Disease Leads to Remarkable Recovery

By Susan Conova
….
When Dr. Lee first saw Annalisa in the neuro ICU, he immediately suspected paraneoplastic anti-NMDAR encephalitis from his previous experiences with the disorder. But they’ve all been recent experiences, which makes some of Annalisa’s doctors wonder if the syndrome is something new and on the rise.

“I never saw these cases in my training, and once you see this movement disorder, you never forget it,” Dr. Lee says. “I’ve spoken to people with 40 and 50 years of experience, and they’ve said they never saw anything like it in the past. I think it’s something new, but it remains to be seen.”

Though the syndrome may turn out to be a common cause of encephalitis in young adults and children, Penn’s Dr. Dalmau believes it is simply becoming more recognized by clinicians. “When you look back at some older descriptions of mysterious cases of encephalitis, you realize they could have been describing anti-NMDAR encephalitis,” Dr. Dalmau says. “It’s like the story of the blind men and the elephant. A few different aspects of the disease have probably been described in the past, but now we know the whole elephant.”

The article below suggests that a disorder described in six children in 1992 as Sebire syndrome may have been anti-NMDAR encephalitis. The article also describes a phase in current cases of anti-NMDAR E where children are having difficulty recovering speech and other social interaction functions. The authors reports that this can be a transition phase that is part of long term recovery, rather than permanent loss of function.
http://onlinelibrary.wiley.com/doi/10.1111/j.1469-8749.2009.03542.x/full
Developmental Medicine & Child Neurology Volume 52, Issue 5, pages e78–e82, May 2010

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

This is a full text article from <em>Neurology</em> describing a case history for a 47-year-old man who was treated with ECT after being diagnosed with anti-NMDAR encephalitis.

NEUROLOGY 2010;75:e44-e46

Pearls & Oy-sters: Electroconvulsive therapy in anti-NMDA receptor encephalitis
H.M.H. Braakman, MD, V.M.P. Moers-Hornikx, MD, B.M.G. Arts, MD, PhD, R.M.M. Hupperts, MD, PhD and J. Nicolai, MD, PhD

From the Departments of Neurology (H.M.H.B., V.M.P.M.-H., J.N.) and Psychiatry (B.M.G.A.), Maastricht University Medical Center, Maastricht; and Department of Neurology (R.M.M.H.), Orbis Medical Center, Sittard, the Netherlands.

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Full text article about the case of a young 18-yr-old man with this disease, (in the state of Maryland, USA.)

NEUROLOGY 2010;74:e83-e86

? 2010 American Academy of Neurology
Resident and Fellow Section
Clinical Reasoning:
An 18-year-old man with subacute mental status change
Daniel Gold, DO, Rebecca Dougherty, MD and Donald Barone, DO
?.The patient had a tracheostomy and a percutaneous gastrostomy (PEG) tube placed. He remained on mechanical ventilation for 1 month. No tumor was identified on PET. He was hospitalized for 7 months and then discharged to rehabilitation, and as of 10 months he still requires 4 antiepileptics for generalized seizures. Repeat body CT scans and testicular ultrasounds have been negative. No surgical exploration has been performed in search of an occult tumor. He receives monthly maintenance IVIg and cyclophosphamide followed by CSF analysis to measure antibody titers, which reportedly continue to decrease?..

Another case in a 2-yr-old reported on Inspire.com.
http://www.inspire.com/BlessedMommy2/journal/nmda-receptor-encephalitis/

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Here's another anti-NMDA case in a 35-yr-old woman who had a teratoma. (I read about this on the Facebook support group.)

http://www.urmc.rochester.edu/news/story/index.cfm?id=3011
Canandaigua Woman?s Ordeal Illuminates Rare Form of Seizures October 19, 2010
The remarkable story of a Canandaigua, N.Y. women who emerged from a more than six month long coma is now being used to educate physicians about how to recognize and treat her extremely rare condition. The elusive source of her uncontrolled seizures, which physicians at the University of Rochester Medical Center (URMC) ultimately traced to ovarian cancer, and her subsequent recovery are the subject of a paper appearing today in the journal Neurology.....

And this might be the first case reported where a patient developed anti-NMDAR encephalitis after she had a teratoma removed. She was initially diagnosed with somatoform disorder with psychosis, but fortunately when the anti-NMDAR E was diagnosed she responded to treatment.

Military Medicine, Volume 175, Number 8, August 2010 , pp. 616-618(3)
Limbic Encephalitis Presenting With Seizures, Anterograde Amnesia, and Psychosis in a Patient Seven Weeks Status Post Immature Ovarian Teratoma

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

http://onlinelibrary.wiley.com/doi/10.1002/ana.21928/pdf

In Search of Lost Time From “Demonic Possession” to Anti–N-Methyl-DAspartate Receptor Encephalitis
Guillaume Se´bire, MD, PhD
The fascinating discovery of anti–N-methyl-D-aspartate receptor (NMDAR) pediatric encephalitis creates new connections between past and present, but likely identical, syndromes.1,2 There was first the strange story of a 14-year-old boy admitted in 1949 at the Alexian Brothers Hospital (Saint Louis, MO) for frightening behavioral disturbances (original description attributed to Rev.
William S. Bowdern, SJ3). Clinical manifestations included explosive episodes of rage, extreme auto- and heteroaggressivity, speech deterioration, sudden “shouts in unknown tongue,” and bouts of contorted postures into seemingly impossible shapes,” propagating vibrations, and even “movements to surrounding pieces of furniture.” 3 The patient recovered soon after an exorcism procedure.
This case report, popularized by the famous novel and movie The Exorcist, presents striking clinical similarities with most manifestations of anti-NMDAR encephalitis....

The news below could be related to the increase in anti-NMDAR cases.

http://www.nytimes.com/2010/11/13/us/13exorcism.html

New York Times
By LAURIE GOODSTEIN
Published: November 12, 2010
There are only a handful of priests in the country trained as exorcists, but they say they are overwhelmed with requests from people who fear they are possessed by the Devil.

Now, American bishops are holding a conference on Friday and Saturday to prepare more priests and bishops to respond to the demand. The purpose is not necessarily to revive the practice, the organizers say, but to help Catholic clergy members learn how to distinguish who really needs an exorcism from who really needs a psychiatrist, or perhaps some pastoral care.

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

http://www.nzma.org.nz/journal/abstract.php?id=4335

Anti-NMDA-receptor autoimmune encephalitis without neoplasm: a rare condition?
Mark Schmiedeskamp, Pietro Cariga, Annemarei Ranta
[snip]
Here we present two patients seen in a secondary care hospital that presented in quick succession and were not associated with a tumour. To the best of our knowledge these are the first non-paraneoplastic anti-NMDA-receptor encephalitides reported in New Zealand.

http://timesfreepress.com/news/2009/jun/11/chattanooga-teen-has-miraculous-recovery-unusual-t/?local

Thursday, June 11, 2009
Chattanooga: Teen has ?miraculous? recovery from unusual tumor disorder
By: Emily Bregel
In mid-March, Dottie Davis thought something was off when her typically unsentimental 13-year-old daughter sobbed with joy while riding a new horse on the family's farm in Hixson.....

Below is an abstract that has a link to a free full text PDF case report that explores why women might be more prone to this disease than men even if they don't have teratomas.

http://www.jstage.jst.go.jp/article/internalmedicine/49/19/49_2167/_article

Internal Medicine
Vol. 49 (2010) , No. 19 pp.2167-2173
Expression of Various Glutamate Receptors Including N-Methyl-D-Aspartate Receptor (NMDAR) in an Ovarian Teratoma Removed from a Young Woman with Anti-NMDAR Encephalitis

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Here's a new report from a hospital in Japan:

This is about a 10-yr-old girl.
http://ukpmc.ac.uk/abstract/MED/20803970
Anti-NMDA receptor antibody encephalitis with recurrent optic neuritis and epilepsy

Here is an abstract for a retrospective paper seeking to get an idea of the prevalence of anti-NMDAR encephalitis in recent years by testing stored serum and CSF samples. Charts of 505 patients aged 18 to 35 years admitted to the intensive care unit (ICU) at a single institution, (I think in Germany), during a 5-year period were reviewed for criteria of encephalitis of unknown etiology. Six out of seven found to match the criteria were positive for the anti-NMDAR antibodies. Anti-NMDAR encephalitis represented 1% of all patients? admissions to the ICU for any cause in the age group examined:

Neurology November 8, 2010 vol. 75 no. 19 1735-1739
Retrospective analysis of NMDA receptor antibodies in encephalitis of unknown origin

That sounds like a significant portion of ICU admissions, and a professor of neurology with UCSF seems impressed with the numbers and does a professional call to arms in the short Medscape blog article he wrote linked to below. He writes that this is an important syndrome and the ICU cases are only part of the total picture. (You can join Medscape for free to read the article.)


Anti-NMDA receptor antibodies in encephalitis
Samuel Pleasure, MD, Neurology, 01:29PM Nov 22, 2010

 

[sharon sanders]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Thank you Emily for this series.

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

http://www.thelancet.com/journals/laneur/article/PIIS1474-4422%2810%2970253-2/fulltext

The Lancet Neurology, Volume 10, Issue 1, Pages 63 - 74, January 2011

Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis

Prof Josep Dalmau MD a, Eric Lancaster MD a, Eugenia Martinez-Hernandez MD a, Prof Myrna R Rosenfeld MD a, Prof Rita Balice-Gordon PhD b

Summary
Since its discovery in 2007, the encephalitis associated with antibodies against the N-methyl-D-aspartate receptor (NMDAR) has entered the mainstream of neurology and other disciplines. Most patients with anti-NMDAR encephalitis develop a multistage illness that progresses from psychosis, memory deficits, seizures, and language disintegration into a state of unresponsiveness with catatonic features often associated with abnormal movements, and autonomic and breathing instability. The disorder predominantly affects children and young adults, occurs with or without tumour association, and responds to treatment but can relapse. The presence of a tumour (usually an ovarian teratoma) is dependent on age, sex, and ethnicity, being more frequent in women older than 18 years, and slightly more predominant in black women than it is in white women. Patients treated with tumour resection and immunotherapy (corticosteroids, intravenous immunoglobulin, or plasma exchange) respond faster to treatment and less frequently need second-line immunotherapy (cyclophosphamide or rituximab, or both) than do patients without a tumour who receive similar initial immunotherapy. More than 75% of all patients have substantial recovery that occurs in inverse order of symptom development and is associated with a decline of antibody titres. Patients' antibodies cause a titre-dependent, reversible decrease of synaptic NMDAR by a mechanism of crosslinking and internalisation. On the basis of models of pharmacological or genetic disruption of NMDAR, these antibody effects reveal a probable pathogenic relation between the depletion of receptors and the clinical features of anti-NMDAR encephalitis.

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

The 'mystery illness' of the little girl in post #5 was ultimately diagnosed as anti-NMDAR encephalitis. Doctors had suggested surrendering the 3-yr-old to hospice prior to this discovery after extensive treatments, including immune therapies, had failed to improve her condition.

Fortunately after being diagnosed, trying a chemo drug has helped and doctors are hoping that in 1-2 years, she will greatly recover.

http://www.abqjournal.com/upfront/2308102752upfront04-23-10.htm

Here is a more recent article. Both articles mention that the girl was tested for swine flu and numerous other infectious diseases and that genetic testing had been done for other causes.

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

A Young Man with Anti-NMDAR Encephalitis following Guillain-Barr? Syndrome
Case Rep Neurol. 2011 Jan?Apr; 3(1): 7?13.
Published online 2011 January 11. doi: 10.1159/000323751.

PMCID: PMC3037987
Copyright ? 2011 by S. Karger AG, Basel
Kana Tojo,a Kazuhito Nitta,a Wataru Ishii,a Yoshiki Sekijima,a Hiroshi Morita,a Yukitoshi Takahashi,b Keiko Tanaka,c and Shu-ichi Ikedaa
aDepartment of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan
bDepartment of Pediatrics, National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan
cDepartment of Neurology, Kanazawa Medical University, Ishikawa, Japan

This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No-Derivative-Works License (http://creativecommons.org/licenses/by-nc-nd/3.0/). Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.

Abstract
A 19-year-old man developed rapidly progressive muscle weakness and dysesthesia in the extremities, and dyspnea after a flu-like episode. Nerve conduction studies showed reduced motor nerve conduction velocities with conduction block, and sensory nerve action potentials could not be evoked. The patient was diagnosed as having Guillain-Barr? syndrome (GBS), and was treated with 2 cycles of intravenous immunoglobulin (IVIg) therapy and was assisted by mechanical ventilation. During the recovery course of the illness, he experienced several attacks of psychomotor agitation from the 37th hospital day, and generalized tonic convulsive seizures suddenly developed on the 42nd hospital day. Brain MRI showed high-intensity lesions in the bilateral thalamus and medial temporal lobes. The convulsions were controlled by continuous thiopental infusion (until the 50th hospital day) and mechanical ventilation (until the 84th hospital day). Intravenous methylprednisolone pulse therapy (1,000 mg/day) for 3 days followed by dexamethasone (16 mg/day) was added. After relief of convulsive seizures, prominent orolingual dyskinesia appeared, and on MRI marked atrophy of the bilateral medial temporal lobes was seen. Anti-N-methyl-D-aspartate receptor (NMDAR) antibodies in serum and cerebrospinal fluid were positive on the 92nd hospital day. Anti-NMDAR encephalitis usually affects young females but a small number of male cases with this disease have been reported. Our male patient was unique in having GBS, a post-infectious autoimmune disease, as a preceding disease, suggesting that anti-NMDAR encephalitis itself is caused by a parainfectious autoimmune mechanism....

Full text available at link.

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

A couple of case history reports from the Facebook support group:

http://www.facebook.com/topic.php?uid=116113262677&topic=15516

ARTICLE FROM 24 November, 2010 from the Netherlands (NL)
Complex Illness: A young female resident from Steenbergen (NL), TOGETHER WITH HER PARENTS IS BATTLING against Anti NMDAR Encephalites.

Wendy goes down only one step at a time

Simply like that, out of the blue, misfortune struck....

http://www.facebook.com/topic.php?uid=116113262677&topic=15610

A Mother's Account of her daughter's battle with Anti NMDA
The mother of a teen who wishes to remain anonymous, has sent me the following account of her daughter's illness. It is well documented and what struck me, is how quickly the physicians intervened, in comparison with other case reports and accounts I have read.

My sincere thanks to the contributor, whose hope it is that this may help someone in some way....

Both stories can be read in full at the links.

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

This is an audio documentary made by Aviva Ziegler about NMDAR encephalitis. Patients, family members, and Dr. Dalmau are interviewed.

http://mpegmedia.abc.net.au/rn/podcast/2011/06/hrt_20110606_0830.mp3

Here's a recent article about a patient who recovered in Japan:
(The full text and a movie are available.)

http://www.jstage.jst.go.jp/article/internalmedicine/50/6/50_627/_article
Akiko Uchino; Takahiro Iizuka; Yoshiaki Urano; Masahide Arai; Atsuko Hara; Junichi Hamada; Ryuichi Hirose; Josep Dalmau and Hideki Mochizuki, Pseudo-Piano Playing Motions and Nocturnal Hypoventilation in Anti-NMDA Receptor Encephalitis: Response to Prompt Tumor Removal and Immunotherapy . Intern. Med. 50: 627-630, 2011 .

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

http://mynorthwest.com/?nid=11&sid=504515

Updated Jun 29, 2011 - 12:57 pm
Rare brain disease puzzles doctors

By Ursula Reutin
97.3 KIRO FM Reporter
(Audio interview and report at link)
Imagine losing your mind for a month or even longer. It's a terrifying reality for a growing number of people who have a newly-recognized disease that attacks their brain, plunging them into madness. KIRO Radio's URSULA REUTIN reports it especially affects young women.
A young woman begins acting strangely, her parents think she's depressed, or maybe on drugs. Instead, it turns out she's suffering from a newly-recognized disease....

Turned out to be anti-NMDAR encephalitis. Concern is expressed that there may be thousands of people suffering undiagnosed.

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

http://www.capmh.com/content/5/1/15

Child and Adolescent Psychiatry and Mental Health 2011, 5:15 doi:10.1186/1753-2000-5-15

The electronic version of this article is the complete one and can be found online at: http://www.capmh.com/content/5/1/15

Received: 21 January 2011
Accepted: 13 May 2011
Published: 13 May 2011

? 2011 Consoli et al; licensee BioMed Central Ltd.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Malignant catatonia due to anti-NMDA-receptor encephalitis in a 17-year-old girl: case report


Ang?le Consoli1, Karine Ronen1, Isabelle An-Gourfinkel2, Martine Barbeau1, Donata Marra3, Nathalie Costedoat-Chalumeau3, Delphine Montefiore5, Philippe Maksud4, Olivier Bonnot1, Adrien Didelot6, Zahir Amoura3, Marie Vidailhet2 and David Cohen1*

Abstract

Anti-NMDA-Receptor encephalitis is a severe form of encephalitis that was recently identified in the context of acute neuropsychiatric presentation. Here, we describe the case of a 17-year-old girl referred for an acute mania with psychotic features and a clinical picture deteriorated to a catatonic state. Positive diagnosis of anti-NMDA-receptor encephalitis suggested specific treatment. She improved after plasma exchange and immunosuppressive therapy. Post-cognitive sequelae (memory impairment) disappeared within 2-year follow-up and intensive cognitive rehabilitation.

[snip]
Background
[snip]
...The exact incidence of anti-NMDAR encephalitis is unknown, but it seems to be more frequent than any other known paraneoplastic encephalitis [4].

Conclusions
[snip]
This case report emphasizes the importance to search for a medical condition in catatonic syndrome of young people to treat and avoid severe neurological sequelae or death. The proposal of the DSM-V workgroup to make catatonia a "specifier" added as a fifth digit to other diagnoses seems likely to reduce rather than enhance clinician awareness of importance of recognizing this syndrome and researching for medical condition, particularly during psychiatric training. By contrast, a large group of experts advocated a unique and broadly-defined code for catatonia as a syndrome, which can be diagnosed acutely in addition to any suspected or established associated disorders [24]. In this case, the initial psychiatric clinical presentation was complicated by a malignant catatonic state, which is now well-described in anti-NMDA-Receptor encephalitis. Child psychiatrists need to know that anti-NMDA-Receptor encephalitis occurs frequently in children and adolescents. Plasma exchanges and immunosuppressive therapy treatments can dramatically improve catatonic syndrome associated with autoimmune dysfunction such as SLE [17,18], PANDAS [25] and NMDA-receptor encephalitis.

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

http://www.thelancet.com/journals/lancet/article/PIIS0140-6736%2811%2960548-5/fulltext

The Lancet, Volume 378, Issue 9785, Page 98, 2 July 2011
<Previous Article
doi:10.1016/S0140-6736(11)60548-5Cite or Link Using DOI
Late onset autism and anti-NMDA-receptor encephalitis
Caroline Creten MD a, Sanne van der Zwaan BSc a, Roos J Blankespoor BSc a, Arjen Maatkamp MD b, Joost Nicolai PhD b, Prof Jim van Os PhD a, Dr Jan NM Schieveld PhD

In December, 2009, a 9-year-old boy was admitted to our hospital with an acute onset of secondary generalised seizures. He had no medical or psychiatric history and functioned very well socially and academically. He presented with speech and swallowing difficulties, which after 10 days developed into a severely agitated catatonic state with opisthotonic posturing, tonic posturing of limbs, insomnia, and dyskinesia....

Same article - HTML copy available:
http://74.125.127.132/scholar?q=cache:XTFETmwlFu0J:scholar.google.com/&hl=en&as_sdt=0,48&as_ylo=2011

Childhood disintegrative disorder, early onset schizo-
phrenia, late onset autism and all stages of anti-NMDA-
receptor encephalitis share core symptoms, as in our
patient. We suggest that anti-NMDA-receptor encephalitis
might be a possible organic cause underlying these three
disorders. Patients previously diagnosed with these
diagnoses might need to be re-examined for anti-NMDA-
receptor encephalitis. We suggest that forthcoming
editions of DSM-5 and ICD-11 exclude and define cases of
regressive autism spectrum disorders due to anti-NMDA-receptor encehpalitis.

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

22 yr-old Kiera Echols, her family, and surgeon Dr. Ed Richards continue their work to educate others about anti-NMDAR encephalitis:

http://nky.cincinnati.com/article/AB/20110725/NEWS01/107260317/-Monster-tumor-survivor-tells-nation-her-story

"The reason God had this happen is for me to help get the word out," Echols said Saturday as 10 hours of filming came to an end. "This doesn't need to be something that nobody knows about."

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

In December of 2009 an 8-year-old girl in Schwenksville, PA got anti-NMDAR encephalitis and is still having a difficult time battling the disease, even though an ovarian teratoma was removed in January 2010:


http://www.pottsmerc.com/articles/2010/08/10/news/srv0000009012950.txt
Annual 'If you build it, they will run' race supports local girl

Published: Tuesday, August 10, 2010; Last Updated: Tue. Aug 10, 2010, 3:13pm

Updates:
http://www.newhanoverumc.org/updates_on_charlotte_wendel

http://pottsmerc.com/articles/2011/07/30/life/srv0000012785708.txt?viewmode=fullstory

 

[Anne]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

http://www.medicalforum.ch/pdf/pdf_d/2011/2011-22/2011-22-112.PDF

in this paper, you can see some RMI, easy to understand.
( White " hyppocampus" on the floor of brain )

 

[Emily]

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

Thanks for sharing that paper, Anne. It seems that the young woman in that case was diagnosed with a related autoimmune disorder involving antibodies to the AMPA receptors in the brain:

An antibody syndrome
antiphospholipid has been eliminated, a dosage of autoantibodies
antineuronaux nuclear (anti-VGKC,
anti-NMDA, anti-GABA , Hu, Ri, Yo, Amphysin, CV2,
Ta/Ma2, Ma and recoverin) is entirely negative. finally,
assay of antibody-anti-AMPA-R is positive.
A third MRI (2 months) shows a
again a shift of epicenters of achieving
with bilateral limbic reached a higher
the left side and a change in the achievement
right (Fig. 3 X). We retain finally the diagnosis
limbic encephalitis of autoimmune origin.

The paper below has a chart on page 2 that describes characteristics of all of the known antibody diseases affecting the brain. Unfortunately the case of the 8-yr-old with anti-NMDA receptor encephalitis would seem to have the best chance of recovery with the immune treatment and tumor removal that was done, yet so far that didn't happen.


Lancet Neurol 2011; 10: 759–72

Autoantibodies associated with diseases of the CNS: new
developments and future challenges
Angela Vincent, Christian G Bien, Sarosh R Irani, Patrick Waters
Several CNS disorders associated with specific antibodies to ion channels, receptors, and other synaptic proteins have
been recognised over the past 10 years, and can be often successfully treated with immunotherapies. Antibodies to
components of voltage-gated potassium channel complexes (VGKCs), NMDA receptors (NMDARs), AMPA receptors
(AMPARs), GABA type B receptors (GABABRs), and glycine receptors (GlyRs) can be identified in patients and are
associated with various clinical presentations, such as limbic encephalitis and complex and diffuse encephalopathies.
These diseases can be associated with tumours, but they are more often non-paraneoplastic, and antibody assays can
help with diagnosis. The new specialty of immunotherapy-responsive CNS disorders is likely to expand further as
more antibody targets are discovered. Recent findings raise many questions about the classification of these diseases,
the relation between antibodies and specific clinical phenotypes, the relative pathological roles of serum and intrathecal
antibodies, the mechanisms of autoantibody generation, and the development of optimum treatment strategies.