Announcement

**Emily** · February 1, 2012, 10:54 PM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

New study reports on the public health importance of anti-NMDAR encephalitis:

http://www.flutrackers.com/forum/sho...d.php?t=181368
The Frequency of Autoimmune N-Methyl-D-Aspartate Receptor Encephalitis Surpasses That of Individual Viral Etiologies in Young Individuals Enrolled in the California Encephalitis Project

**Anne** · February 8, 2012, 04:58 PM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

http://www.jim.fr/medecin/21_neuro/e...actu_med.phtml

I hope that the translation is OK...

Ophelia Syndrome
Posted on 07/02/2012

In 1982, Ian Carr Canadian pathologist reported in the Lancet severe psychiatric and neurological disorders observed in his daughter Jane discovered before his Hodgkin's disease. He proposed the term syndrome of Ophelia in reference to psychiatric disorders presented by Ophelia after the heartache caused by the conduct of Hamlet in the famous eponymous work of Shakespeare. Reading this article published in "personal paper" is recommended to all lovers of beautiful letters. This is an unfortunately rare example of literature where the patient is described in his daily life with their questions and emotions and not as a complex of genes, or biomarkers of images.

"We Walked and visited the small islands, and She Was her quaint, cool, remote choke, with the infinite promise of femininity lurking around the corner of her smile."

The girl had therefore presented a clinical picture involving severe subacute memory loss, depression and hallucinations. At that time, the origin of limbic encephalitis was uncertain (metastases, papovavirus) paraneoplastic but the mechanism was already mentioned. Ian Carr was thus suggested that the tumor secreted a substance having an effect neurotransmitter.

From this description, the knowledge of the pathophysiology of limbic encephalitis has made significant progress but the medical pen dried up. An article recently published in Lancet Neurology summarizes the progress in this area. The limbic encephalitis associated with antibodies to NMDA receptors is the most common and can manifest as a psychotic state followed by seizures, a catatonic state, a disintegration of language, movement disorders and a dysautonomia. A series of 400 cases has been well published. In 58% of cases, an ovarian teratoma is found and immunotherapy may lead to improvement in 75% of cases. Treatment can be guided by the demonstration of intrathecal synthesis of antibodies. More recently, we identified antibodies called anti potassium channels (VGKC) antibodies against proteins associated leucine-rich glioma inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2).

In this case, encephalic manifestations may be preceded by behavioral problems in REM sleep or insomnia realizing Morvan syndrome. Other antibodies have been described as directed against the GABA B and glutamic acid decarboxylase. But 30 years later can we explain what happened to Jane? The antibodies were probably guilty, in fact, since a team has identified recently demonstrated in patients with encephalitis and Hodgkin's disease antibodies directed against the mGlu5 (glutamate receptors) in 2 patients.

Thus, chemotherapy and radiotherapy did they help to overcome the Jane Hodgkin, limiting antibody production and preventing it the fate of Ophelia.

"When we cam back she Remembered what she HAD beens doing the day before. Not just a straw in the wind bending branches goal. Went on it. More chemotherapy. Went the snow and the summer meadow instant cam. Day by day She Was Remembering more and more. "

Dr. Christian Geny

Carr I: The Ophelia syndrome: memory loss in Hodgkin's disease. Lancet. 1982; 1: 844-5.

**Emily** · February 9, 2012, 06:05 AM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

Very interesting, Anne!

I'd never heard of Ophelia Syndrome. So this would be a neurological complication of Hodgkin Lymphoma that shares symptoms with anti-NMDAR encephalitis?

I see Dr. Dalmau is heading research into identfying an antibody to target for identifying this:
http://www.neurology.org/content/77/18/1698.short

Antibodies to metabotropic glutamate receptor 5 in the Ophelia syndrome Neurology November 1, 2011 77:1698-1701; published ahead of print October 19, 2011
E. Lancaster, E. Martinez-Hernandez, M.J. Titulaer, M. Boulos,
S. Weaver, J.-C. Antoine, E. Liebers, C. Kornblum,
C.G. Bien, J. Honnorat, S. Wong, J. Xu,
A. Contractor, R. Balice-Gordon, and J. Dalmau

**Anne** · February 9, 2012, 08:15 AM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

I had a paper with an old man : auto immune encephalitis and in fact Hodgkin..http://www.em-consulte.com/article/198975
Abstract

Introduction

An observation of limbic encephalitis associated with Hodgkin?s disease is compared with rare cases of the literature. The clinical presentation was relapsing episodes of febrile cognitive disorders with confusion and meningitis, curable after treatment of Hodgkin?s disease. Recent concepts on limbic encephalitis are discussed.
Case report

A seventy-five-year-old patient was hospitalized because of relapsing feverish confusion episodes with meningitis. During the year before his admission he had experienced four spontaneously regressive episodes of feverish confusion. Exploration of these episodes disclosed a paraneoplastic limbic encephalitis due to an underlying Hodgkin?s disease. The treatment of Hodgkin?s disease led to perfect recovery of cognitive function, so that the patient could drive his car.
Conclusion

Lymphoproliferative disease, such as Hodgkin?s disease, is a possible diagnosis in patients with limbic encephalitis. A dysimmune process underlying Hodgkin?s disease might be operating in this association.

I think that "Ophelia " was an autoimmune encephalitis, with anti body against mGlu5.

here : tourette as autoimmune disease against " putamen"

Antibodies against human putamen in children with Tourette syndrome

http://www.neurology.org/content/50/6/1618.abstract?ijkey=5c37e526f9b3d75313562473b29a031b3e7a75c4&keytype2=tf_ipsecsha

Background Similar to the model for Sydenham's chorea, antineuronal antibodies, which develop in response to a preceding streptococcal infection, have been speculated to have a role in the development of Tourette syndrome (TS). Methods Serum antibodies against human caudate, putamen, and globus pallidus (interna and externa) were assayed by enzyme-linked immunosorbent assay (ELISA) and Western blot techniques and results were correlated with clinical characteristics and markers of streptococcal infection. Subjects A total of 41 children with TS (mean age, 11.3 years) and 39 controls (mean age, 12.1 years) were included. Results Compared with controls, TS subjects had a significant increase in the mean ( p = 0.006) and median ( p = 0.002) ELISA optical density (OD) levels of serum antibodies against putamen, but not caudate or globus pallidus. Western blots on 20 control and 20 TS serum samples showed that specific antibodies to caudatelputamen occurred more frequently in TS subjects at 83, 67, and 60 kDa; antigens were present in a synaptosomal fraction. TS subjects with a positive family history of tics had higher OD values ( p ≤ 0.04), but no association was shown with age of tic onset, tic severity, sudden onset of tics, or presence of attention-deficit hyperactivity disorder or obsessive-compulsive disorder. Risk ratio calculations in TS and control groups and in study subjects dichotomized for high and low putamen OD values were similar for titers of antistreptolysin O ≥166 or antideoxyribonuclease B ≥ 170. A subgroup analysis limited to subjects with elevated streptococcal titers, however, showed a significantly ( p ≥ 0.004) larger number of TS subjects with elevated OD levels. Conclusion Children and adolescents with TS had significantly higher serum levels of antineuronal antibodies against putamen than did controls, but their relation to clinical characteristics and markers for streptococcal infection remains equivocal.

**Emily** · February 10, 2012, 04:01 AM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

I think that "Ophelia " was an autoimmune encephalitis, with anti body against mGlu5.

Thank you for clarify that, Anne.

That's interesting about the Tourette research, too. It makes me wonder how much of that might be autoimmune.

There was a news article about another case of anti-NMDAR encephalitis in Georgia that was published last December, though the 3-yr-old fell ill in June:
http://www2.wsav.com/news/2011/dec/0...se-ar-2837223/

Pooler Girl Battles Rare Disease
By: Tina Tyus-Shaw | WSAV-TV
Published: December 09, 2011

(Savannah, GA) In three beautiful years, Chloe Sills has accomplished so much.
Wearing the orange shirt, she's the smallest dancer at her church...

**Emily** · February 24, 2012, 04:31 AM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

A parent in El Paso, TX, resigns from school board presidency to attend to medical needs of daughter recently diagnosed with anti-NMDAR encephalitis:

http://www.elpasotimes.com/news/ci_20015613

By Alex Hinojosa \ EL PASO TIMES
Posted: 02/22/2012 12:00:00 AM MST

Socorro Independent School District officials unanimously approved $35 million for the construction of a new school and accepted the resignation of Board President Tony Ayub on Tuesday.

Ayub said he was stepping down immediately to be with his daughter Erin, who was diagnosed in December with anti-NMDA receptor encephalitis, a rare form of encephalitis. It is an acute inflammation of the brain caused by a viral infection or when the body's immune system mistakenly attacks brain tissue...

**Emily** · February 29, 2012, 02:51 AM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

More news on the El Paso case reported on in the previous post:

http://www.ktsm.com/news/local-high-...p-student-coma

Local High School Bands Fundraise for UTEP Student in Coma
News
Elena Arteaga - Producer
Friday, February 24, 2012 - 22:26
Erin Ayub, 21, and family
El Paso ? Two El Paso high schools have put down their rivalries for the moment to raise money for an ex-student who has been in an Arizona hospital since December.

Erin Ayub, 21, has been in an induced-coma in an Arizona hospital after being diagnosed with a rare illness-- anti-NMDA Receptor Encephalitis...

**Emily** · March 14, 2012, 05:46 AM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

Tetano posted an article about anti-NMDAR autoantibodies being found in these cases, (suggesting pandemic H1N1 was the trigger):

http://www.flutrackers.com/forum/sho...d.php?t=184208
Pediatrics: Late Delirious Behavior With 2009 H1N1 Influenza: Mild Autoimmune-Mediated Encephalitis?

**Emily** · March 16, 2012, 05:54 AM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

This is a case in a 27-yr-old woman in Romania. There is a PDF preview of the first page of the paper at the link.

http://www.springerlink.com/content/3m414v2672338515/

J Neurol. 2012 Mar 3. [Epub ahead of print]
Spontaneous recovery from anti-NMDAR encephalitis.
Evoli A, Spinelli P, Frisullo G, Alboini PE, Servidei S, Marra C.
Source

Institute of Neurology, Catholic University, Largo F. Vito 1, 00168, Rome, Italy

One of the theories about why some cases might spontaneously recover is that the immune system might have destroyed a tiny teratoma. Here's a case where a microscopic teratoma was found in a patient:

http://onlinelibrary.wiley.com/doi/1...365.x/abstract

Reversible paraneoplastic encephalitis in three patients with ovarian neoplasms

JANOS L. TANYI1,
EVELYN B. MARSH1,
JOSEP DALMAU2,
CHRISTINA S. CHU1

DOI: 10.1111/j.1600-0412.2012.01365.x

? 2012 The Authors AOGS? 2012 Nordic Federation of Societies of Obstetrics and Gynecology
bstract

Anti-NMDA receptor encephalitis is a recently described potentially lethal but treatable disorder that often occurs as a paraneoplastic manifestation of ovarian teratomas. We report three women with this disorder who presented with subacute onset of delirium, seizures and autonomic instability. Anti-NMDA receptor antibodies were detectable in the serum or CSF of each patient. Ovarian masses were detected in two patients, and subsequently excised. In the third patient, an empiric bilateral salpingo-oophorectomy was performed and revealed a microscopic neoplasm. All patients experienced slow reversal of the neurological symptoms following surgery and immunotherapy. Our experience supports that prompt syndrome recognition followed by tumor removal and immunotherapy usually results in neurological recovery.

Here's another paper reporting about how teratomas can be so small that they are difficult to find:

http://cat.inist.fr/?aModele=afficheN&cpsidt=25287870

Titre du document / Document title
?Benign? ovarian teratoma and N-methyl-D-aspartate receptor (NMDAR) encephalitis in a child
Auteur(s) / Author(s)
Frawley Kieran J. (1 4) ; Calvo-Garcia Maria A. (1) ; Krueger Darcy A. (2) ; McMasters Richard L. (3) ;
Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)
(1) Department of Radiology, Cincinnati Children?s Hospital Medical Center, OH, Cincinnati, USA
(4) Department of Radiology, Royal Children?s Hospital, Qld, Brisbane, Australia
(2) Department of Pediatrics, Division of Child Neurology, Cincinnati Children?s Hospital Medical Center, OH, Cincinnati, USA
(3) Department of Pathology and Laboratory Medicine, Cincinnati Children?s Hospital Medical Center, OH, Cincinnati, USA
R?sum? / Abstract
N-methyl-D-aspartate receptor (NMDAR) encephalitis is a life-threatening paraneoplastic neuropsychiatric encephalitis that predominantly affects young women and has a strong association with ovarian teratomas. Removal of the ovarian teratomas improves the prognosis and decreases the risk of recurrence. We present an 11-year-old girl with NMDAR encephalitis with small bilateral teratomas not initially appreciated on abdominal CT or pelvic MRI. A 12-mm teratoma was identified in the right ovary and a 7-mm teratoma was identified in the left ovary on US follow-up at 5 months. Intraoperative sonography was used to localize the teratomas for excision. In NMDAR encephalitis, the ovarian teratomas can be very small, particularly in children, and easily missed on cross-sectional imaging. Awareness of the association of NMDAR encephalitis and ovarian teratomas will improve the diagnostic accuracy and imaging interpretation. Periodic sonography and MRI might be warranted in children if the initial study is negative.
Revue / Journal Title
Pediatric radiology ISSN 1432-1998
Source / Source
2012, vol. 42, no1, pp. 120-123 [4 page(s) (article)]

**Anne** · March 29, 2012, 12:17 PM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

about PANDA and OCD... a good news

http://www.eurekalert.org/pub_releases/2012-03/niom-pco032312.php

Possible causes of sudden onset OCD in kids broadened

NIH immune-based treatment study underway

<table align="right" border="0" cellpadding="0" cellspacing="0" width="218"> <tbody><tr> <td colspan="5"></td> </tr> <tr> <td></td> <td align="left" bgcolor="#f2f2f2" height="4" valign="top" width="4"></td> <td bgcolor="#f2f2f2" height="4" width="210"></td> <td align="right" bgcolor="#f2f2f2" height="4" valign="top" width="4"></td> <td></td> </tr> <tr> <td></td> <td bgcolor="#f2f2f2"></td> <td bgcolor="#f2f2f2"> <center> </center> IMAGE: Children with PANS and PANDAS sometimes experience sudden loss of fine motor skills.

<center> Click here for more information. </center> </td> <td bgcolor="#f2f2f2"></td> <td></td> </tr> <tr> <td></td> <td align="left" bgcolor="#f2f2f2" height="4" valign="bottom" width="4"></td> <td bgcolor="#f2f2f2" height="4" width="202"></td> <td align="right" bgcolor="#f2f2f2" height="4" valign="bottom" width="4"></td> <td></td> </tr> <tr> <td colspan="5"></td> </tr> </tbody></table> Criteria for a broadened syndrome of acute onset obsessive compulsive disorder (OCD) have been proposed by a National Institutes of Health scientist and her colleagues. The syndrome, Pediatric Acute-onset Neuropsychiatric Syndrome (PANS), includes children and teens that suddenly develop on-again/off-again OCD symptoms or abnormal eating behaviors, along with other psychiatric symptoms ? without any known cause.
PANS expands on Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus (PANDAS), which is limited to a subset of cases traceable to an autoimmune process triggered by a strep infection. A clinical trial testing an immune-based treatment for PANDAS is currently underway at NIH and Yale University (see below).
"Parents will describe children with PANS as overcome by a 'ferocious' onset of obsessive thoughts, compulsive rituals and overwhelming fears ," said Susan Swedo, M.D., of the NIH's National Institute of Mental Health (NIMH), who first characterized PANDAS two decades ago. "Clinicians should consider PANS when children or adolescents present with such acute-onset of OCD or eating restrictions in the absence of a clear link to strep."
Swedo, James Leckman, M.D., of Yale University, and Noel Rose, M.D., Ph.D. of Johns Hopkins University, propose working criteria for PANS in February 2012 in the open source journal Pediatrics & Therapeutics.
"As the field moves toward agreement on this broadened syndrome, affected youth will be more likely to receive appropriate care, regardless of whether they are seen by a neurologist, pediatrician or child psychiatrist," said NIMH Director Thomas R. Insel, M.D.
Differing causes sharing a "common presentation"

The PANS criteria grew out of a PANDAS workshop convened at NIH in July 2010, by the NIMH Pediatric and Developmental Neuroscience Branch, which Swedo heads. It brought together a broad range of researchers, clinicians and advocates. The participants considered all cases of acute-onset OCD, regardless of potential cause.
Clinicians reported that evaluations of more than 400 youth diagnosed with PANDAS confirmed that affected boys outnumbered girls 2:1, with psychiatric symptoms, always including OCD, usually beginning before 8 years.
Although debate continues about the fine points, the field is now of one mind on the core concept of "acute and dramatic" onset of a constellation of psychiatric symptoms. There is also broad agreement on the need for a "centralized registry" that will enable the research community to analyze evidence from studies that will eventually pinpoint causes and treatments. Such a registry is currently under development by members of the International Obsessive Compulsive Foundation (IOCDF).
Since a diagnosis of PANS implies no specific cause, clinicians will have to evaluate and treat each affected youth on a case-by-case basis.
"PANS will likely turn out to include a number of related disorders with different causes that share a common presentation," explained Swedo.
The authors propose that a patient must meet 3 diagnostic criteria for a diagnosis of PANS:

Abrupt, dramatic onset of OCD or anorexia.
Concurrent presence of at least two additional neuropsychiatric symptoms with similarly severe and acute onset. These include: anxiety; mood swings and depression; aggression, irritability and oppositional behaviors; developmental regression; sudden deterioration in school performance or learning abilities; sensory and motor abnormalities; somatic signs and symptoms.
Symptoms are unexplainable by a known neurologic or medical disorder.

Among the wide range of accompanying symptoms, children may appear terror stricken or suffer extreme separation anxiety, shift from laughter to tears for no apparent reason, or regress to temper tantrums, "baby talk" or bedwetting. In some cases, their handwriting and other fine motor skills worsen dramatically. Leckman's team at the Yale Child Study Center is in the process of developing assessment tools for diagnosing the syndrome.
PANDAS treatment study targets errant antibodies
Meanwhile, Swedo, Leckman, and Madeleine Cunningham of the University of Oklahoma, and colleagues, are collaborating on a new, multi-site placebo-controlled study, testing the effectiveness of intravenous immunoglobulin (IVIG) for reducing OCD symptoms in children with PANDAS.
Previous human and animal research suggested mechanisms by which strep-triggered antibodies mistakenly attack specific brain circuitry, resulting in obsessional thoughts and compulsive behaviors.
"Strep bacteria has evolved a kind of camouflage to evade detection by the immune system," Swedo explained. "It does this by displaying molecules on its cell wall that look nearly identical to molecules found in different tissues of the body, including the brain. Eventually, the immune system gets wise to this 'molecular mimicry,' recognizes strep as foreign, and produces antibodies against it; but because of the similarities, the antibodies sometimes react not only with the strep, but also with the mimicked molecules in the human host. Such cross-reactive 'anti-brain' antibodies can cause OCD, tics, and the other neuropsychiatric symptoms of PANDAS."
IVIG, a medication derived from normal antibodies, neutralizes such harmful antibodies, restoring normal immune function. It is used to treat other autoimmune illnesses and showed promise in a pilot study with PANDAS patients.
"We predict that IVIG will have striking benefits for OCD and other psychiatric symptoms, and will prove most effective for children who show high levels of anti-brain antibodies when they enter the study," said Swedo.
Prospective study participants are first screened by phone by investigators at the NIH or the Yale Child Study Center. Those who meet eligibility requirements are then randomized to receive either active IVIG or a placebo procedure during a brief inpatient stay at the NIH Clinical Center. The researchers remain blind to which children received the active medication; after 6 weeks of placebo control, they give any children whose symptoms fail to improve the option to receive open-label active treatment.
In addition to assaying for antibodies that attack brain cells, the researchers use magnetic resonance imaging to see if the treatment reduces inflammation in an area of the brain known as the basal ganglia, which is thought to be the target of the errant antibodies. They also analyze levels of immune system chemical messengers (cytokines) in cerebrospinal fluid and blood ? with an eye to identifying biomarkers of disease activity and potential predictors of treatment response.

**Emily** · March 30, 2012, 12:09 PM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

about PANDA and OCD... a good news

That is good news, Anne. I think I read that it can be difficult to have some insurance companies pay for the IVIG, so a study could make the treatment more established and that might help patients get off some of the powerful medications that can have side effects long-term.

**Emily** · April 1, 2012, 05:05 AM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

http://www.ktvn.com/story/17270535/f...pitalized-girl

Fundraiser Wednesday to Help Hospitalized Reno Girl
Posted: Mar 27, 2012 5:53 PM PDT Updated: Mar 27, 2012 7:04 PM PDT
Michael Wolfe
Channel 2 News

Wednesday you can help the family of a 2-year-old Reno girl diagnosed with a rare disease.

Gillian Navarroza was diagnosed with anti-NMDA Receptor Encephalitis last October and has been hospitalized in Oakland for more than four months...

http://www.gulf-times.com/site/topic...8&parent_id=20

Latest Update: Sunday11/3/2012March, 2012, 11:52 PM Doha Time
Constable opens eyes after year in coma
London Evening Standard/London

A policewoman has opened her eyes for the first time since falling into a coma 18 months ago.
Pc Katie Habberley, 27, was taken to hospital in August 2010 after suffering severe headaches and went into intensive care two days later with brain inflammation caused by a rare form of encephalitis.
?She had a headache that got worse as the weeks went on,? Bronwen said.
[snip]
She suffered from NMDAR-antibody encephalitis, a rare form that causes confusion, memory loss and seizures. Her family are now raising money for the Encephalitis Society to help sufferers.

http://archneur.ama-assn.org/cgi/con...rol.2012.354v1

ONLINE FIRST
Autoantibodies in the Patient With Drug-Resistant Epilepsy
Are We Missing a Treatable Etiology?
Gregory K. Bergey, MD Arch Neurol. Published online March 26, 2012. doi:10.1001/archneurol.2012.354
AUTHOR INFORMATION Author Affiliation: Department of Neurology, The Johns Hopkins University School of Medicine, The Johns Hopkins Hospital, Baltimore, Maryland.

http://archneur.ama-assn.org/cgi/con...ol.2011.2985v1

ONLINE FIRST
Autoimmune Epilepsy

Clinical Characteristics and Response to Immunotherapy

Amy M. L. Quek, MBBS; Jeffrey W. Britton, MD; Andrew McKeon, MD; Elson So, MD; Vanda A. Lennon, MD, PhD; Cheolsu Shin, MD; Christopher J. Klein, MD; Robert E. Watson Jr, MD, PhD; Amy L. Kotsenas, MD; Terrence D. Lagerlund, MD; Gregory D. Cascino, MD; Gregory A. Worrell, MD, PhD; Elaine C. Wirrell, MD; Katherine C. Nickels, MD; Allen J. Aksamit, MD; Katherine H. Noe, MD; Sean J. Pittock, MD

Arch Neurol. Published online March 26, 2012. doi:10.1001/archneurol.2011.2985

Objective To describe clinical characteristics and immunotherapy responses in patients with autoimmune epilepsy.
Design Observational, retrospective case series.
Setting Mayo Clinic Health System.

[snip]

Main Outcome Measure Seizure frequency.

Results After a median interval of 17 months (range, 3-72 months), 22 of 27 (81%) reported improvement postimmunotherapy; 18 were seizure free. The median time from seizure onset to initiating immunotherapy was 4 months for responders and 22 months for nonresponders (P < .05). All voltage-gated potassium channel complex antibody?positive patients reported initial or lasting benefit (P < .05). One voltage-gated potassium channel complex antibody?positive patient was seizure free after thyroid cancer resection; another responded to antiepileptic drug change alone.

Conclusion When clinical and serological clues suggest an autoimmune basis for medically intractable epilepsy, early-initiated immunotherapy may improve seizure outcome.

**Emily** · April 11, 2012, 06:24 AM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

http://www.ncbi.nlm.nih.gov/pubmed/22426441

Epileptic Disord. 2012 Mar;14(1):90-3.
PET follow-up in a case of anti-NMDAR encephalitis: arguments for cingulate limbic encephalitis.
Chanson JB, Diaconu M, Honnorat J, Martin T, De Seze J, Namer IJ, Hirsch E.
Source

D?partement de Neurologie, CHRU de Strasbourg, France.
Abstract

Background. The lack of specific MRI abnormalities in anti-NMDA receptor encephalitis makes the identification of the most affected areas difficult. Functional neuroimaging could be useful to identify brain dysfunction associated with psychiatric symptoms, but few precise data are available up to now. Case study. A 27-year-old woman was referred for recent behavioural changes and jerks of the right foot. Serial left fronto-temporal seizures were recorded. Identification of anti-NMDA receptor antibodies in CSF indicated a diagnosis of anti-NMDA receptor encephalitis. Two foci of hypermetabolism, in the left prefrontal cortex and the anterior cingulate cortex, were identified using 18-fluorodeoxyglucose PET and both disappeared after treatment. Brain MRI was normal, except for a mild left prefrontal hypersignal. Conclusions. The increase in marker uptake in motor and premotor regions in our case probably corresponds to epileptic activity. Our data suggest that the anterior cingulate cortex could play an important role in psychiatric symptoms. Other studies are needed to better understand the pathophysiology of anti-NMDA receptor encephalitis.

Full text available for this article about another case in France:
http://www.ncbi.nlm.nih.gov/pmc/arti...3/?tool=pubmed

Child Adolesc Psychiatry Ment Health. 2011; 5: 15.
Published online 2011 May 13. doi: 10.1186/1753-2000-5-15
Consoli, A.
Ronen, K.
An-Gourfinkel, I.
Cohen, D
PMCID: PMC3121673
Copyright ?2011 Consoli et al; licensee BioMed Central Ltd.
Malignant catatonia due to anti-NMDA-receptor encephalitis in a 17-year-old girl: case report
[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.

Here's a report of a study starting in England:
https://blogs.monash.edu/bmedsc-et-al/2012/03/22/hi/

[snip]
Recently cases have been reported and ?Anti-NMDA encephalitis? has been described, where patients present with neuropsych symptoms, are found to have the antibodies, and respond very well to immunosuppresive treatment. We are taking a reverse approach, to look at how many patients present with First Episode Psychosis may have these antibodies present in serum...

**Ronan Kelly** · April 27, 2012, 06:00 PM

Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

Clinical Features, Treatment, and Outcome of 500 Patients with Anti-NMDA Receptor Encephalitis

March 14, 2012
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Speaker: Maarten Titulaer, MD, PhD

Lecture Abstract:
OBJECTIVE: To provide the clinical features and suggest treatment guidelines for anti-NMDA-receptor encephalitis.

BACKGROUND: Anti-NMDA-receptor encephalitis is the most common and best characterized antibody-mediated encephalitis. We provide the clinical features, treatment, and follow-up of 500 patients.
DESIGN/METHODS: Cohort study, analysis of demographics, onset, treatment, and long-term follow-up.

RESULTS: 82% were female. Median age was 21 years (range 1-85; 36% <18 and 4% >45 years). 42% had a tumor (95% teratomas). 55% of females >12 years had ovarian teratoma(s) versus 8% <12 years. In patients <12 years old the most frequent initial symptoms were abnormal behavior, seizures, and movement disorders (36%, 35%, 14%), while in adults were abnormal behavior and memory problems (70%, 13%). 90% of patients had >3 of the following: psychiatric symptoms, memory/speech disorder, seizures, dyskinesias, decreased level of consciousness, autonomic instability, or hypoventilation. Within the first month, movement disorders and ataxia were more frequent in children (92% and 17% vs 70% and 2%, p<0.001 both), while memory problems and hypoventilation predominated in adults (84% and 42% vs 68% and 16%, p=0.008 and p<0.001).Immunotherapy (93%) and tumor removal (when appropriate) resulted in full recovery or substantial improvement in 61% of patients at 8 months, and 77% at 24 month follow-up; 7% died. Early treatment (1st month) led to better outcome (75% vs 64%, p=0.001). If 1st line immunotherapy (steroids, immunoglobulins and/or plasma exchange) failed, 2nd line treatment (rituximab or cyclophosphamide) significantly improved outcome compared with no treatment or repeating 1st line drugs (56% vs 27%, p=0.006). Relapses occurred in 14%, 73% of them in patients without teratoma.

CONCLUSIONS: Anti-NMDA-receptor encephalitis is a severe but treatable disorder of predominantly young individuals. Prompt treatment improves outcome. If initial immunotherapy fails, second-line treatment is usually effective. 75% of patients have full/substantial improvement although the process of recovery can take >24 months.

...

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Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

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