Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

[Emily]

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

This was not diagnosed as NMDARE but an autoimmune mechanism is suspected.

http://www.therecord.com/news-story/...local-teacher/

Mysterious illness incapacitates local teacher

Susan Benedetto of Waterloo.
ByLiz Monteiro

WATERLOO REGION ? For nearly 18 months, Susan Benedetto has said precious little and hasn't taken a step.

The 48-year-old St. Mary's High School teacher can't speak or walk, and she's fed through a tube.

It's been like this since May 2012.
...
The closest Susan got to a diagnosis is autoimmune encephalitis, also known as inflammation of the brain. Susan had a pre-existing autoimmune disorder, but was able to manage the symptoms for 20 years, her husband said.
...
Dr. Alfonso Fasano, a neurologist at Toronto Western and an associate professor at the University of Toronto, said the encephalitis of the brain is likely caused by a virus.

Fasano said Susan had two MRIs. The first MRI showed a huge lesion around the brain stem and severe inflammation. The second MRI done since Susan has been at the Toronto hospital now shows atrophy, which is death of neurons in the brain, he said.

The inflammation is no longer present, Fasano said.
...
Fasano said the medication and treatment Susan has been on to deal with her autoimmune illness over the years may have contributed to the imbalance in the autoimmune system and lead to the abnormal reaction to the virus...

[Emily]

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

http://jcn.sagepub.com/content/early...01875.abstract

Anti?N-Methyl-d-Aspartate (NMDA) Receptor Encephalitis
An Unusual Cause of Autistic Regression in a Toddler
J Child Neurol 0883073813501875, first published on October 3, 2013 doi:10.1177/0883073813501875

Abstract

Anti N-methyl-d-aspartate (NMDA) receptor encephalitis in children is associated with psychiatric changes, seizures, and dyskinesias. We present the first report of autistic regression in a toddler caused by this entity. A 33-month-old boy presented with decreased appetite, irritability, and insomnia following an upper respiratory tract infection. Over the next few weeks he lost language and social skills, and abnormal movements of his hand developed. Within a month, this patient came to fit the diagnostic criteria for autistic spectrum disorder. Upon investigation, anti-NMDA receptor antibodies were found in the boy's cerebrospinal fluid. He was treated with intravenous immunoglobulins and steroids, resulting in reacquisition of language and social skills and resolution of movements. Our case emphasizes the significance of suspecting anti-NMDA receptor encephalitis as the cause of autistic regression, even in an age group where the diagnosis of autistic spectrum disorder is typically made, and especially when presentation follows a febrile illness.

Received July 4, 2013.
Revision received July 11, 2013.
Accepted July 23, 2013.

Journal of the Neurological Sciences

Available online 30 October 2013
Anti-NMDA receptor encephalitis presenting with total insomnia ? A case report

In?s Br?s Marques,
Rute Teot?nio,
Catarina Cunha,
Concei??o Bento,
Francisco Sales

Department of Neurology, Coimbra University Hospital, Praceta Prof. Mota Pinto, 3000-075 Coimbra, Portugal

Fatal insomnia (FI) is the first diagnosis to be considered by most neurologists when approaching a patient presenting with total insomnia followed by personality and cognitive changes, disturbance of alertness, autonomic hyperactivation and movement abnormalities.

We report the case of a 30 year-old male patient who presented with total insomnia followed by episodes of psychomotor restlessness resembling anxiety attacks. Twenty days later, he developed refractory convulsive status epilepticus with admission to Intensive Care Unit. He progressed to a state of reduced alertness and responsiveness, presenting periods of agitation with abnormal dyskinetic movements, periods of autonomic instability and central hypoventilation. Workup revealed antibodies against N-methyl-d-aspartate receptor (NMDAR). Immunotherapy treatment led to a very significant improvement with the patient presenting only slight frontal lobe dysfunction after one year of recovery.

To the best of our knowledge this is the first report of a patient with anti-NMDAR encephalitis first presenting with total insomnia. Our aim is to alert that anti-NMDAR encephalitis must be considered in the differential diagnosis of FI, especially in sporadic cases. Distinguishing the two conditions is very important as, contrarily to the fatal disclosure of FI, anti-NMDAR encephalitis is potentially reversible with adequate treatment even after severe and prolonged disease.

[Emily]

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

http://www.hkjpaed.org/details.asp?id=930&show=1234

HK J Paediatr (New Series)
Vol 18. No. 4, 2013
HK J Paediatr (New Series) 2013;18:226-229

Case Report

Anti-N-Methyl-D-Aspartate Receptor Encephalitis: A Potentially Treatable Cause of Neuropsychiatric Syndrome

KKH Ho, EKC Yau, WL Yiu, NC Fong

Abstract

A young adolescent girl with no history of psychiatric illness presented with acute onset of delusional ideas and behavioural changes that were followed by recurrent seizures, involuntary movement and oromotor dyskinesia. Anti-N-methyl-D-aspartate receptor (NMDAR) antibodies were present in the serum and she was successfully treated with immunotherapy. The presence of neurological features including convulsions, movement disorder and autonomic disturbance are important clues to the diagnosis of anti-NMDAR encephalitis in patients who initially present with acute psychiatric symptoms. Early diagnosis and treatment is warranted as aggressive immunotherapy improves clinical outcome.

Keyword : Autoimmune; Encephalitis; Movement disorder; N-methyl-D-aspartate receptor

Abstract in Chinese
Introduction

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis was first described as a syndrome of neuropsychiatric symptoms in young women with ovarian teratoma. Since the discovery of the pathogenic anti-NMDAR antibodies in 2007,1 increasingly more children and adults with or without underlying tumour were identified with this syndrome, bringing to light a new association between neuronal autoantibodies and psychosis. Recent studies suggest that anti-NMDAR encephalitis is not as uncommon as previously thought.2 Yet, anti-NMDAR encephalitis is not widely reported in our local population. We here describe a Chinese adolescent girl with antibodies to the NMDAR who presented with neuropsychiatric symptoms, seizures and movement disorder...

http://onlinelibrary.wiley.com/doi/1...nticated=false

N-methyl-D-aspartate receptor antibody-associated movement disorder without encephalopathy

Yael Hacohen1, Nomazulu Dlamini2,
Tammy Hedderly2, Elaine Hughes2,
Martin Woods2, Angela Vincent1,
Ming Lim1,2,*
Developmental Medicine & Child Neurology

Early View (Online Version of Record published before inclusion in an issue)
Article first published online: 16 OCT 2013

DOI: 10.1111/dmcn.12321

© 2013 Mac Keith Press

N-methyl-D-aspartate receptor (NMDAR) antibody encephalitis is a well-recognized clinico-immunological syndrome that presents with a movement disorder, cognitive decline, psychiatric symptoms, and epileptic seizures. A pure monosymptomatic presentation is rare; however, some patients present predominantly with a movement disorder in the absence of encephalopathy. Here, we describe three paediatric patients with an NMDAR antibody-mediated movement disorder: a 5-year-old female with acute onset hemichorea, a 10-year-old female with generalized chorea, and a 12-year-old male with abdominal myoclonus. These patients did not develop the characteristic encephalopathy syndrome seen in NMDAR encephalitis, but all three had other associated subtle cognitive deficits. The patients demonstrated good responses to immunotherapy.

[Emily]

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

http://journals.lww.com/greenjournal/Abstract/2014/02002/Anti_N_Methyl_Aspartate_Receptor_Encephalitis_in.7.aspx

Obstetrics & Gynecology:
February 2014 - Volume 123 - Issue 2, PART 2 - p 433?435
doi: 10.1097/AOG.0000000000000078
Case Report
Anti-N-Methyl-Aspartate Receptor Encephalitis in Identical Twin Sisters: Role for Oophorectomy

Masghati, Salome MD; Nosratian, Mana; Dorigo, Oliver MD, PhD
Collapse Box
Abstract

BACKGROUND: Anti-N-methyl-aspartate receptor encephalitis is a potentially fatal form of encephalitis and frequently associated with ovarian teratomas. Surgical removal of ovarian teratomas improves clinical outcome, but it is unclear whether bilateral salpingo-oophorectomy for normal-appearing ovaries is of clinical benefit.

CASE: Our report describes a unique clinical scenario of identical twin sisters with anti-N-methyl-aspartate receptor encephalitis. Neither patient responded to immunosuppressive therapy. Imaging studies showed normal-appearing ovaries. The first twin continued on medical therapy only and died of the disease. The second twin underwent a bilateral salpingo-oophorectomy followed by gradual recovery.

CONCLUSION: Based on our experience in two genetically identical individuals, we suggest considering the removal of normal-appearing ovaries in patients with anti-N-methyl-aspartate receptor encephalitis who fail to respond to medical treatment.

? 2014 by The American College of Obstetricians and Gynecologists.

[Emily]

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

http://journals.lww.com/neurotodayon...spx?PostID=282

Thursday, January 16, 2014
New Evidence for Narcolepsy as an Autoimmune Disorder

BY TOM VALEO

...

Now researchers at the Stanford University School of Medicine have found compelling evidence that human narcolepsy is an autoimmune disease that results when people with certain gene variants are exposed to a virus or a vaccine that induces the body to launch an attack on hypocretin-producing cells.

Also, signs of this autoimmune attack appear very early in the course of the disease, which may enable a prompt diagnosis with a simple blood test that would allow treatment to begin sooner, according to veteran sleep researcher Emmanuel Mignot, MD, PhD, director of the Stanford Center for Sleep Sciences and Medicine, the lead author of the study published in the Dec. 18 issue of Science Translational Medicine.

...

Dr. Mignot suspects that narcolepsy exists on a spectrum, with cases at the milder end now potentially identifiable with a blood test.

?This is speculation on my part, but I think onset in some cases is probably milder and harder to detect, and progressive,? he said. ?People learn to live with it.?

A blood test could allow T cells from a subject to be exposed in vitro to cells containing the HLA variant associated with narcolepsy. ?Then you add the piece of hypocretin that binds to [the haplotype] DQB1*06:02,? said Dr. Mignot. ?T cells from normal people won?t proliferate, but if you put T cells from patients (with narcolepsy), they will get activated.?

See what sleep experts had to say about this research in our Feb. 6 issue of Neurology Today....

http://www.ncbi.nlm.nih.gov/pmc/arti...MC3099489/#B12

Sleep. 2011 June 1; 34(6): 689?690.
doi: 10.5665/SLEEP.1024
PMCID: PMC3099489
Comment on the Letter to the Editor By Dr. Marcus on the Association between Narcolepsy and H1N1 Exposure
Emmanuel Mignot, MD, PhD,1 Yves Dauvilliers, MD, PhD,2 and Jacques Montplaisir, MD, PhD3,4

...

Since we first reported this observation, the association between Pandemrix and narcolepsy has become even more likely. Of 33 million Europeans vaccinated with Pandemrix, approximately 160 have already developed narcolepsy, leading to an incidence of 0.36/100,000, a figure close to habitual yearly incidence of narcolepsy. As it takes years before narcolepsy is diagnosed, many more cases are likely to be present in this sample. Reviewing current evidence, the WHO is now suspecting a 9-fold increase in incidence in Finish children and adolescents.3

Surprisingly, we also found that many post-H1N1 subjects were positive for ASO2 (as in prior samples4) suggesting a role for recent strep throat infections.4,5 We suggest that two factors are needed for the development of narcolepsy: (1) a specific immune?mimicry component, mediated through the presentation by DQB1*0602/DQA1*01026 of a particular antigen to a specific TCR idiotype,7 and (2) nonspecific factors, such as adjuvants, flu or strep infections, streptococcus superantigens, and other factors. These nonspecific effects may reactivate dormant T cell cones, increase blood brain penetration of peripheral immune responses, or could involve novel HLA immune interactions specific to the brain, as suggested recently by HLA class I expression in neurons and interactions with natural killer cell or immunoglobulin-like receptors.8

...

...Intriguingly, it has long been forgotten that Von Economo's encephalitis lethargica, a seasonal disease that followed the great 1918 H1N1 flu pandemic, was a very polymorphic disorder at the symptomatic level.11 Symptoms included extreme somnolence and ophthalmoplegia (associated with lesions of the posterior hypothalamus and upper brainstem), insomnia and sleep inversion (associated with lesions of the anterior hypothalamus), psychosis, movement disorders (chorea type, as seen in post-streptococcal Sydenham's Chorea, with lesion of the basal ganglia) and a residual form: post-encephalitis parkinsonism. These recent findings increasingly suggest that narcolepsy may be the tip of the iceberg and that a much broader phenotype involving post-infectious autoimmune pathologies of the brain persists in the population at large. Recent cases of Von Economo's-like encephalitis have also shown increased ASO titers.12

[Emily]

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

www.karger.com/Article/PDF/358820

Case Rep Neurol 2014;6:38?43
DOI: 10.1159/000358820
Published online: February 8, 2014
? 2014 S. Karger AG, Basel
1662‒680X/14/0061‒0038$39.50/0

https://www.karger.com/crn

This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) (www.karger.com/OAlicense), applicable to the online version of the article only. Distribution permitted for noncommercial purposes only.
Salvatore Monaco, MD
Department of Neurological and Movement Sciences
University of Verona, Policlinico G.B. Rossi, P. le L.A. Scuro 10
IT?37134 Verona (Italy)
E-Mail [see article]
Anti-N-Methyl-D-Aspartate Receptor Encephalitis Causing a Prolonged Depressive Disorder Evolving to Inflammatory Brain Disease
Sara Mariottoa Stefano Tamburina Alessandro Salviatia Sergio Ferraria
Marco Zoccaratoc Bruno Giomettoc Laura Bertolasia Franco Alessandrinib Maria Donata Benedettia Salvatore Monacoa aDepartment of Neurological and Movement Sciences, University of Verona, and bDivision of Neuroradiology, AOUI, Verona, and cDepartment of Neurology, Regional Hospital ?Ca? Foncello?, Treviso, Italy

Key Words
Anti-N-methyl-D-aspartate receptor encephalitis ? Optic neuritis ? Depressive disorder

Abstract
Background: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a rapidly evolving condition that combines psychiatric and neurologic manifestations. Much remains unclear about its clinical onset and subsequent course. Although successful treatment depends on diagnosing the disorder early and therefore minimizing long-term complications, this is a difficult task owing to the atypical onset of this condition and the prolonged clinical course that has been observed in some patients. This report, illustrating a patient with slowly progressing psychiatric manifestations, unusual imaging and electrophysiological features, extends the information on varied clinical phenotypes.
Case Report: A 32-year-old woman suffered from an isolated depressive disorder for 4 months before undergoing psychiatric evaluation. During the following 5 months, she manifested hypersexuality, dysarthria, imbalance, dyskinesias and decreased word output. Brain magnetic resonance
imaging (MRI) showed multifocal hyperintense T2/FLAIR lesions, a few contrast-enhanced, involving the corona radiata, the periventricular white matter, the deep gray nuclei, the optic nerves and the brainstem. MRI spectroscopy disclosed confluent bilateral demyelination and focal optic nerve involvement suggesting widespread encephalitis. Visual evoked potential
studies indicated a demyelinating disorder. Serological screening and total body positronemission tomography yielded negative findings for malignancies. Cerebrospinal fluid examination disclosed IgG oligoclonal bands and nti-NMDAR antibodies. Corticosteroids and intravenous immunoglobulin provided only slight improvement, whereas switching to cyclophosphamide markedly improved her neurological status.
Conclusion: In patients with a prolonged clinical course, including psychiatric and neurological symptoms, the differential diagnosis should be anti-NMDAR encephalitis. This report expands the known disease phenotypes in this emerging condition. ? 2014 S. Karger AG, Basel

Full text at link.

[Emily]

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

http://onlinelibrary.wiley.com/doi/1...12204/abstract

Title: CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders

Author: A. Dudesek,F. Rimmele,S. Tesar,S. Kolbaske,P. S. Rommer,R. Benecke,U. K. Zettl
Publication: Clinical & Experimental Immunology
Publisher: John Wiley and Sons
Date: Feb 4, 2014
? 2013 British Society for Immunology

Summary

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently defined inflammatory central nervous system (CNS) disorder, prominently involving the brainstem and in particular the pons. The condition features a combination of clinical symptoms essentially referable to brainstem pathology and a characteristic magnetic resonance imaging (MRI) appearance with punctate and curvilinear gadolinium enhancement ?peppering? the pons. The radiological distribution is focused in the pons and adjacent rhombencephalic structures such as the cerebellar peduncles, cerebellum, medulla and the midbrain. While the lesion burden with a perivascular pattern is typically most dense in these pontine and peripontine regions, enhancing lesions may additionally extend into the spinal cord and supratentorial structures such as the thalamus, basal ganglia, capsula interna, corpus callosum and the cerebral white matter. Another core feature is clinical and radiological responsiveness to glucocorticosteroid (GCS)-based immunosuppression. As withdrawal of GCS treatment results commonly in disease exacerbation, long-term immunosuppressive therapy appears to be mandatory for sustained improvement. Diagnosis of CLIPPERS is challenging, and requires careful exclusion of alternative diagnoses. A specific serum or cerebrospinal fluid (CSF) biomarker for the disorder is currently not known. Pathogenesis of CLIPPERS remains poorly understood, and the nosological position of CLIPPERS has still to be established. Whether CLIPPERS represents an independent, actual new disorder or a syndrome that includes aetiologically heterogeneous diseases and/or their prestages remains a debated and not finally clarified issue. Clinicians and radiologists should be aware of this condition and its differential diagnoses, given that CLIPPERS constitutes a treatable condition and that patients may benefit from an early introduction of GCS ensued by long-term immunosuppression. Based on previous reports in literature ? currently encompassing more than 50 reported cases of CLIPPERS ? this review addresses clinical features, diagnostic criteria, differential diagnoses and therapeutic management of this peculiar disorder.

See also:
Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids Following Influenza Vaccination

[Emily]

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

http://tbonescafe.com/we-recommend/a...rday-march-1st

A Concert for Coco ? Saturday, March 1st

Posted: February 21, 2014 at 8:46 pm
...Coco is a Hattiesburg native and is a senior at Southern Miss. She was recently diagnosed with anti-NMDA receptor encephalitis, a very rare disease. While the road to recovery will be long, we know that Coco is a fighter. By bringing her love for music to one of her favorite venues, we hope to gather both morale and financial support that will help alleviate the financial burden that her journey may have...

Very rare or relatively common now?

www.texasneurologist.org (Meeting notice in Word doc format)

Schedule of Events
Friday, February 28, 2014
Pediatric Neurology
Moderators: Gary Clark, MD and Eli Mizrahi, MD

8 ? 9 AM
Approach to Autoimmune Encephalitis
Amber Stocco, MD and Eyal Muscal, MD

Objectives: Describe the relatively common but recently discovered entity: NMDA Receptor Antibody Encephalitis; Recognize clinically and medically the typical semiology of associated movement disorders of NMDA Encephalitis: dystonia, chorea, myoclonus, ataxia and stereotypes; Detail long term prognosis and prolonged care needed for this disorder.

http://www.asianjournalofpsychiatry....372-9/abstract

Asian Journal of Psychiatry
Volume 7, Complete , Pages 92-94, February 2014
Anti-NMDA receptor encephalitis: A neurological disease in psychiatric disguise

Bhawna Sharma, Rahul Handaemail, Swayam Prakash, Kadam Nagpal,
Pankaj Gupta

Received 9 November 2013; received in revised form 21 November 2013; accepted 23 November 2013. published online 19 December 2013.

Abstract

Anti-NMDA receptor encephalitis was first described in 2005 when psychiatric features, memory loss and altered consciousness were found in four women with ovarian teratoma. We report a case of anti-NMDA receptor encephalitis in a 16-year-old female who presented with psychiatric features followed by autonomic dysfunction and orofacial dyskinesias that showed drastic improvement to intravenous immunoglobulin. As many patients of anti-NMDAR encephalitis initially present with psychiatric features, it is important for psychiatrists to have high index of suspicion for this disease and thus avoid the delay in diagnosing this treatable condition which may be otherwise fatal.

http://www.researchgate.net/publicat...and_immunology

Autoimmune synaptic protein encephalopathy syndromes and the interplay between mental health, neurology and immunology

Gregory S Day, Harry E Peery
Health Science Inquiry 06/2013; 4(1):89-92.

ABSTRACT Indirect evidence supporting the organic underpinnings of mental illness has accumulated over the past five decades, driven by advances in neuropharmacology, neurochemistry, neuroimaging, and neuropathology, and by expanding knowledge of the structural and ultra-structural changes that occur within the brains of patients with behavioural and affective symptoms. 1 Yet, despite a growing body of evidence, a distinction continues to be drawn between psychological anomalies of the mind and organic disorders of the nervous system. The recent isolation and characterization of pathogenic autoantibodies within the blood and cerebrospinal fluid of patients with symptoms and signs commonly associated with 'mental illness' raises new questions about the origins and pathogenesis of behavioural and affective disorders, providing a timely and welcome challenge to the ideology that divides mental illness and neurological disorders. ? Autoimmune synaptic protein encephalopathy syndromes and the interplay between mental health, neurology and immunology

[Emily]

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

http://www.wtov9.com/news/features/t...tal-3768.shtml

Teen returns home from hospital

Updated: Thursday, April 3 2014, 08:08 AM EDT

TORONTO, Ohio --

A homecoming queen’s senior year was cut short after she came down with a rare disease in December but this week she was able to finally go home to be with her family.

Hannah Leasure, a Toronto High School senior, was a healthy teen who was hospitalized on Christmas Eve after developing anti-NMDA receptor encephalitis, a rare condition that affects the brain...

http://iospress.metapress.com/content/e78n0861qlv220x7/

Neuroimmune diseases are increasing. Is there a possible vaccine link?

Journal Journal of Pediatric Neurology
Publisher IOS Press
ISSN 1304-2580 (Print)
1875-9041 (Online)
Subject Pediatrics, Neurology, Neuroradiology, Neurosciences, Child Psychiatry and Epilepsy
Issue Volume 11, Number 4 / 2013
Category Review Article
Pages 201-209
DOI 10.3233/JPN-130623
Subject Group Medicine and Health
Online Date Tuesday, January 07, 2014
Publisher's Copyright Statement
Authors
Alisan Yildiran1, Süleyman Kaplan2, Mehmet Emin Önger2

1Department of Pediatric Immunology-Allergy, School of Medicine, Ondokuz Mayıs University, Samsun, Turkey
2Department of Histology-Embryology, School of Medicine, Ondokuz Mayıs University, Samsun, Turkey
Abstract

Autoimmune and neuroimmune diseases prevalence rates are rising enormously. It would be difficult to explain with genetic and environmental factors. Noteworthy, this rising maybe related with childhood vaccine programs. There is also a lack of information about this topic in pediatric and family medicine practice in some countries like Turkey. Prevention and treatment of these diseases is possible if pathogenesis could be well understood. We attempt to review this topic in immunological and neurological perspectives for this purpose. We searched Pubmed from year 2000 to date with neuroimmune, autoimmune, measles and vaccine link keywords in some combinations and related topics. The obtained information from this search dealt with some important subheadings. There is a need for further investigations of neuroimmune disease and the possible vaccine link.

Keywords
Neuroimmune, autoimmune, measles, vaccine link

[Emily]

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

http://www.neurology.org/content/82/...t/P5.179.short

April 30, 2014
Poster Session V
CNS Diseases and Differential Diagnosis

Anti-NMDA Receptor Antibodies in Neurologic Disease (P5.179)

Jessica Panzer3, Bailey Baumann2, Hiro Furukawa4, Noriko Simorowski5,
Amy Gleichman1 and David Lynch1

Neurology April 8, 2014 vol. 82 no. 10 Supplement P5.179

Abstract

OBJECTIVE:To understand the role of anti-NMDAR antibodies in the pathogenesis of autoimmune encephalitis and other neurological disorders.BACKGROUND:Anti-NMDAR encephalitis, a recently discovered syndrome associated with psychosis, altered consciousness, seizures, dyskinesias, and autonomic dysfunction, is now known to be one of the most commonly identified causes of encephalitis. IgG antibodies in this syndrome may have increased affinity for the open conformation of the n-methyl-D-aspartate receptor (NMDAR), and transiently stabilize this conformation. Over the longer term, there is NMDAR hypofunction due to receptor cross-linking and internalization. Recently, anti-NMDAR IgA antibodies were found both in a progressive dementia and also in some patients with anti-NMDAR encephalitis. Anti-NMDAR antibodies have also been noted in relapse of herpes simplex encephalitis, Creutzfeldt-Jakob disease, and neuropsychiatric lupus...

http://onlinelibrary.wiley.com/doi/1...mds.25623/full

Mohammad, S. S., Sinclair, K., Pillai, S., Merheb, V., Aumann, T. D., Gill, D., Dale, R. C. and Brilot, F. (2014), Herpes simplex encephalitis relapse with chorea is associated with autoantibodies to N-Methyl-D-aspartate receptor or dopamine-2 receptor. Mov. Disord., 29: 117?122. doi: 10.1002/mds.25623

http://onlinelibrary.wiley.com/doi/1...mds.25626/full

Hacohen, Y., Deiva, K., Pettingill, P., Waters, P., Siddiqui, A., Chretien, P., Menson, E., Lin, J.-P., Tardieu, M., Vincent, A. and Lim, M. J. (2014), N-methyl-D-aspartate receptor antibodies in post?herpes simplex virus encephalitis neurological relapse. Mov. Disord., 29: 90?96. doi: 10.1002/mds.25626

[Emily]

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

http://www.mercurynews.com/health/ci...rds?source=pkg

Misdiagnosed bipolar: One girl's struggle through psych wards before Stanford doctors make bold diagnosis and treatment
Julia Prodis Sulek
Posted: 04/19/2014 04:16:41 PM PDT57 Comments | Updated: 5 days ago

...

Controversial diagnosis

What Frankovich, a pediatric rheumatologist, and Dr. Kiki Chang, a child psychiatrist, concluded was that Tessa likely had an infection or other trigger that caused her immune system to mistakenly attack her brain, dramatically changing Tessa's behavior overnight. It's a condition called PANS -- pediatric acute-onset neuropsychiatric syndrome -- that in some cases, if caught early enough, could be cured by commonly used antibiotics. Without early treatment, they say, children can suffer needlessly.
...
"To know how many patients are in mental institutions that have treatable diseases," Frankovich said, "we can't even wrap our head around this."...

[Emily]

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

Detailed report of a battle with anti-NMDAR encephalitis:

http://www.washingtonpost.com/nation...14b_story.html

The doctor and the teenager

By Sandra G. Boodman, Published: April 28

As the jet hurtled toward New York?s John F. Kennedy International Airport on New Year?s Day 2013, the clinical psychologist watched her 16-year-old daughter warily, praying there would be no recurrence of the girl?s inexplicable and bizarre behavior.

...

Carmen remembers feeling elated by this turn of events. ?You think, ?Well, that?s it, they know what it is and they?re going to cure her and she?s going to be out of here in three days,? she said.

But Marcuse knew that nothing could be further from the truth. Mia?s illness is ?like walking someone through Hades,? the neurologist said. ?And most people get worse before they get better.?...

[Emily]

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

http://news.pennmedicine.org/blog/20...ew-center.html

Obscure Neurologic Diseases Discovered at Penn to be Focus of New Center
Posted by Kim Menard on January 29, 2014
...
The new center, developed through Penn?s department of Neurology, will be run by leaders in the field including center director Josep Dalmau, MD, PhD, adjunct professor of Neurology and Eric Lancaster, MD, PhD, assistant professor of Neurology. The clinic will focus on consultations and long-term care of patients with antibody mediated neurological diseases such as anti-NMDA receptor encephalitis. Physicians from outside hospitals can refer patients to the center, and patients with a positive antibody diagnosis can make appointments for follow-up care. Additionally, Penn neurologists will handle in-patient consults of those hospitalized at the Hospital of the University of Pennsylvania with autoantibody disorders...

Pediatric Neurology
Available online 29 May 2014
Three phenotypes of anti-NMDA receptor antibody encephalitis in children: prevalence of symptoms and prognosis

Allen D. DeSena, MD, MPHa, b, Benjamin M. Greenberg, MD, MHSa, b,
Donna Graves, MDa, b

...

Results

We note that the poorest responders, even to aggressive immunotherapies, are the catatonia persistent type anti-NMDA receptor antibody encephalitis, which has as its hallmark, prolonged periods of severe encephalopathy. Patients with predominantly psychiatric symptoms, which we call the psychiatric predominant NMDA receptor antibody encephalitis, have had excellent responses to plasma exchange or other immunotherapies and appear to have the least residual deficits at follow-up. Patients with fairly equal representations of periods of altered mental status, behavior problems, and movement disorders appear to have an intermediate prognosis and likely require early aggressive immunotherapy...

[Emily]

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

http://www.tennessean.com/story/news...fight/9860755/

Donations help girl, 6, in encephalitis fight
Maranda Faris; 7:26 a.m. CDT June 2, 2014

The mother of a 6-year-old South Nashville girl has taken to the Internet to raise money for the medical bills her daughter has accumulated after she developed a disease that began to attack her brain.

On April 17, Callie Hulsey had a seizure at school.
...
A week before her hospitalization, Callie had an ear infection. That, doctors told her mother, caused viral meningitis. The bout of meningitis, in turn, caused encephalitis to attack Callie?s brain, the doctors said.

Last week, Callie?s doctors told the Hulsey family that their daughter has anti-NMDA receptor antibody encephalitis, an autoimmune form of encephalitis that causes inflammation in the brain...

[Emily]

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

http://philadelphia.cbslocal.com/201...ing-outbursts/

Health: Mysterious Brain Illness Causes Terrifying Outbursts
June 10, 2014 1:27 PM

PHILADELPHIA (CBS) ? It?s a mysterious brain illness that triggers psychotic and violent behavior.

It is rare, and it is often misdiagnosed.

One desperate family grabbed a video camera to document what was happening.

...

A blood test finally revealed PANS ? Pediatric Acute-onset Neuropsychiatric Syndrome. It?s marked by sudden and dramatic outbursts and obsessive behavior, and it?s unusual.

We discovered that he actually had an autoimmune disease attacking his blood and blood vessels,? explains Dr. Jennifer Frankovitch.

It was also attacking the brain, triggering psychiatric problems.

A lot isn?t known about the newly identified syndrome.

?Maybe about 14 to 40 percent of the cases, we think strep triggers it?we just don?t know yet,? Dr. Frankovitch says...

The symptoms started in 2009 at age 7.