Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

[Emily]

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

http://www.ncbi.nlm.nih.gov/pubmed/20016378

Crit Care Med. 2010 Feb;38(2):679-82.
Anti-N-methyl-D-aspartate receptor antibodies: a potentially treatable cause of encephalitis in the intensive care unit.

Davies G, Irani SR, Coltart C, Ingle G, Amin Y, Taylor C, Radcliffe J, Hirsch NP, Howard RS, Vincent A, Kullmann DM.

National Hospital for Neurology and Neurosurgery, and Institute of Neurology, University College London, London, UK.
Abstract

OBJECTIVE: To report the occurrence of an unusual neurologic disorder requiring admission to the intensive care unit. DESIGN: Analysis of an observational cohort study of 31 patients with encephalitis admitted over a 4-yr period. SETTING: Neurologic intensive care unit in a tertiary referral center. PATIENTS: We identified N-methyl-D-aspartate receptor antibodies in six patients (two male and four female). All seropositive patients presented with a psychiatric prodrome, before developing seizures and obtundation requiring intensive care unit admission. They exhibited limb and truncal stereotypies and orofacial dyskinesias upon weaning sedation. Two patients had ovarian tumors. INTERVENTIONS: Patients were treated with sedation, antiepileptic drugs, and immunotherapy. One patient received a magnesium infusion and ketamine. MEASUREMENTS AND MAIN RESULTS: N-methyl-D-aspartate receptor antibodies were identified in serum samples by an immunofluorescent cell-based assay. Three patients made a good but slow recovery; two were left with severe neurologic deficits; and one died after return to the referring hospital. These patients accounted for approximately 20% of all patients admitted with encephalitis to this referral center. CONCLUSIONS: N-methyl-D-aspartate receptor antibodies should be tested in patients with hyperkinetic encephalitis and neuropsychiatric prodrome admitted to the intensive care unit. The disorder is probably not rare and is potentially treatable.

PMID: 20016378 [PubMed - indexed for MEDLINE]

http://www.news-bulletin.com/nb/inde...y-illness.html

Mother seeks help with child's mystery illness
Written by Brent Ruffner/News-Bulletin
Wednesday, 07 April 2010 06:00

Desiree Egan-Jaramillo gently held her daughter's hand after putting her into a red Flyer wagon on a recent Thursday afternoon.

But her daughter, Fiona, wasn't on her way to an Easter egg hunt or an afternoon of playing in the park with her friends.

Instead, the 3-year old was asleep on the sixth floor of the University of New Mexico Children's Hospital, likely catching her breath from a day full of medical testing.

The pair have been at the hospital since Jan. 22, the day Fiona suddenly stopped breathing and was rushed to the Albuquerque hospital after sporadic spells of flu-like symptoms in the weeks before.....continued at link

[Emily]

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

http://journals.lww.com/co-neurology...ion_of.10.aspx

Current Opinion in Neurology:
April 2010 - Volume 23 - Issue 2 - p 144–150
doi: 10.1097/WCO.0b013e32833735fe
Seizure disorders: Edited by Michael Sperling
The growing recognition of immunotherapy-responsive seizure disorders with autoantibodies to specific neuronal proteins
Vincent, Angela; Irani, Sarosh R; Lang, Bethan

[sharon sanders]

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

Thanks Emily.

[Emily]

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

http://www.primarypsychiatry.com/asp...articleid=1677

Psychosis Associated with Anti-N-methyl-D-aspartate Receptor Antibodies
Kevin M. Nasky, DO, Douglas R. Knittel, MD, and Gail H. Manos, MD
CNS Spectr. 2008;13(8):699-702
Until recently, relatively few reports have associated the etiology of limbic encephalitis with the production of antibodies to the N-methyl-D-aspartate (NR)1/NR2 subunit heterodimers of the N-methyl-D-aspartate (NMDA) receptor. In fact, 13 out of the 23 total publications on the subject of anti-NMDA receptor encephalitis appeared in 2007, revealing a sharp increase in the incidence of this disorder, its recognition, or both.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2607118/

Lancet Neurol. 2008 December; 7(12): 1091–1098.
Published online 2008 October 11. doi: 10.1016/S1474-4422(08)70224-2.

Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies

In anti-NMDA-receptor encephalitis the high prevalence of prodromal viral-like symptoms is intriguing. Direct viral pathogenesis is unlikely because extensive studies of CSF samples, brain biopsies, and autopsies were negative for viruses (data not shown). Whether the prodromal symptoms form part of an early immune activation,17,18 or result from a non-specific infection that facilitates crossing of the blood–brain barrier by the immune response is unknown.19,20 Nevertheless, the immune response eventually predominates in the nervous system as suggested by the high frequency of pleocytosis, oligoclonal bands, and intrathecal synthesis of NR1 antibodies. In general, patients with an underlying tumour develop more robust immune responses than those without a tumour.
...
A characteristic feature of patients who recover from anti-NMDA-receptor encephalitis is a persisting amnesia of the entire process (data not shown). This feature is compatible with disruption of the mechanisms of synaptic plasticity, thought to underlie learning and memory, in which the NMDA receptors play a key part.
....
Anti-NMDA-receptor encephalitis represents a new category of immune-mediated disorder that is often paraneoplastic, treatable, and can be diagnosed serologically. Future studies should clarify the best type and duration of immunotherapy, the role of prodromal events in triggering the immune response, and the molecular mechanisms involved in decreasing the number of NMDA receptors.

http://www.ncbi.nlm.nih.gov/pubmed/2...gdbfrom=pubmed

Nervenarzt. 2010 Jan 31. [Epub ahead of print]
[Anti-NMDA-receptor encephalitis : An interdisciplinary clinical picture.]

[Article in German]

Prüß H, Dalmau J, Arolt V, Wandinger KP.

Experimentelle Neurologie und Klinik und Poliklinik für Neurologie, Charité – Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland.

Anti-NMDA-receptor encephalitis is a severe and considerably underdiagnosed form of encephalitis with characteristic clinical features including psychiatric symptoms, decreased levels of consciousness, hypoventilation, epileptic seizures, autonomic dysfunction and dyskinesias. Most patients are primarily seen by psychiatrists, often on the assumption of a drug-induced psychosis. Anti-NMDA-receptor encephalitis had initially been described in young women with ovarian teratoma, but is also common in women without tumour, in men and in children.

I see quite a few parents of very young children looking for support and information about this.
http://www.facebook.com/group.php?v=...d=116113262677

[Emily]

Springfield News-Sun:
http://www.springfieldnewssun.com/ne...rs-598733.html

Updated 8:57 AM Monday, March 15, 2010
SPRINGFIELD ? Last November, Kiera Echols was in hell.

Goblins. Demons with red eyes. A room that should be still was moving; a picture that should be silent was talking.

It seemed as if the 22-year-old had gone mad. Her family was advised to institutionalize her.

But Echols? parents, David and Chellie Givens, and her husband, Mike, couldn?t reconcile this delusional, incoherent person with the strong, motivated Kiera they knew.

Chellie Givens demanded a second opinion ? a move that saved Echols? life and possibly her sanity....continued at link.

http://www.youtube.com/neurofilmfest#p/c/52/dcd8_1CooZ8

Another case in a New York Post reporter:
http://www.nypost.com/p/news/local/i...6axZ8Uyig17QKL

My mysterious lost month of madness
I was a happy 24-year-old suddenly stricken by paranoia & seizures. Was I going crazy?

By SUSANNAH CAHALAN

Last Updated: 6:01 AM, October 4, 2009

Read more: http://www.nypost.com/p/news/local/i...#ixzz0jvVdjOwd
IT was a cold March day as I walked to work from my Hell's Kitchen studio. The weather was clear, people were out in their coats and scarves, but something did not feel right. The sky was so blue, it hurt my eyes. The billboards in Times Square assaulted me with violent reds, yellows and purples. It was like the world had become brighter, louder, more painful.

When I got to the Midtown newsroom of the New York Post, where I was a year into my first full-time reporting job, I asked a friend, "Have you ever not felt like yourself? Have you ever felt completely off?"....

Even more alarming is that more cases are being seen world-wide in younger children.

http://www.ncbi.nlm.nih.gov/pubmed/1...&ordinalpos=51

J Child Neurol. 2009 Oct 15. [Epub ahead of print]
Expanding Spectrum of Encephalitis With NMDA Receptor Antibodies in Young Children.

Lebas A, Husson B, Didelot A, Honnorat J, Tardieu M.

Assistance Publique-H?pitaux de Paris, Service de neurologie p?diatrique et Centre de R?f?rence des maladies inflammatoires du cerveau, H?pital Bic?tre, France.

The authors report here 2 cases of subacute-onset encephalitis with N-methyl-D-aspartate (NMDA) receptor antibodies. One had a paraneoplastic syndrome associated with a neuroblastoma, whereas the other had no primary tumor. This disease was originally described as a paraneoplastic syndrome in young women with ovarian teratoma. The clinical features of both children resembled the typical symptoms reported for older patients with this disease: psychomotor deterioration, movement disorders, and seizures. One of the reported cases is the first known case of paraneoplastic encephalitis with NMDA antibodies in a child with neuroblastoma. Both cases described here were younger than any of the previously reported cases. Consistent with recently published series, this report suggests that the spectrum of symptoms of encephalitis with NMDA receptor antibodies is probably wider than previously thought.

Doctors and patients are trying to spread the word to avoid people being institutionalized without proper treatment.