Anti-NMDA receptor encephalitis

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The Anti-NMDA receptor encephalitis is an inflammation of the brain ( encephalitis ), in which antibodies against the NMDA receptor can be detected.

Classification, frequency and risk groups

Anti-NMDA receptor encephalitis was first described in 2007. The disease ranks among other previously unrecognized inflammatory diseases of the brain ( e.g. PIBS , PANS ) that have been researched and described since 2000. Reliable data on the frequency of anti-NMDA receptor encephalitis are not yet available. About 80% of those affected are women and girls. The average age of onset is 23 years.

Other mammals can also develop anti-NMDA receptor encephalitis. The seizure that led to the drowning of the Berlin polar bear Knut in 2011 can also be traced back to this disease.

Symptoms

The illness often begins with a pre-flu-like stage, insomnia, loss of appetite, confusion, followed by psychological symptoms such as anxiety, excitement, bizarre behavior, delusions and hallucinations. A large proportion of the patients initially receive psychiatric treatment. Epileptic seizures and catatonia- like disturbances of consciousness occur within a few weeks .

At this stage, other symptoms can include involuntary movements, swallowing disorders (food and drinks can no longer be consumed), inability to move, breathing disorders, cardiac arrhythmias, blood pressure and temperature fluctuations, as well as speech interruptions (complete loss of speech). It is a serious illness and patients are often treated in intensive care units for long periods of time.

According to individual case reports, anti-NMDA receptor encephalitis can lead to acute onset, autism-like symptoms ( late onset autism ) in older children .

Cause, diagnosis

Anti-NMDA receptor encephalitis is an autoimmune disease . The body forms defense substances ( antibodies ) against the NMDA receptor , a protein that plays an important role in signal transmission in the brain. A teratoma that contains nerve cells, among other things, is found in around 60% of adult patients . In these patients, anti-NMDA receptor encephalitis is a paraneoplastic syndrome . Teratomas are very rarely found in children and adolescents. No underlying disease is found in other patients.

The suspicion is based on the clinical syndrome and a mostly increased number of cells in the cerebrospinal fluid ( liquor cerebrospinalis ) and confirmed by the detection of antibodies against NMDA receptors in the liquor or serum (blood).

Many of the sick people have EEG changes. Only about half of the patients show changes in the brain on magnetic resonance imaging .

Treatment, prognosis

One tries to suppress the misdirected defense reaction of the body with the help of immunosuppressive drugs . Glucocorticoids and intravenous immunoglobulin administration are most commonly used. If a tumor is found, it is removed. The antibodies are filtered out of the blood via a so-called plasmapheresis and replaced by an antibody substitute.

Approximately 75% of patients will recover or survive with only mild neurological problems. Around 21% of those affected survive with severe neurological damage, and an estimated 4% of patients die as a result of the disease. According to more recent studies, a good half of the patients who are treated with immunosuppressive therapy show an improvement after just four weeks. A full or at least very good recovery was observed in over 80% in this study, and 12% had a relapse within two years.

The prognosis is better in patients who have a tumor found and removed. Most sufferers have a permanent memory loss ( amnesia ) for the duration of the illness. Relapses are possible. If the disease is recognized early and treated correctly, the prospect of full recovery is very good. The patients have to learn everything again in various therapies, physiotherapy (movement), speech therapy (speaking and swallowing) and occupational therapy (visual perception and memory).

A study by the Charité and the DZNE in 2016 showed that the protease inhibitor bortezomib leads to rapid clinical improvement in severe disease courses. The drug eliminates the antibody-producing plasma cells , which reduces the number of antibodies and thus improves the symptoms.

Trivia

During the autopsy of the polar bear Knut , it was found that he suffered from NMDA encephalitis until he drowned in an epileptic seizure. This is the first reported case of NMDA encephalitis in an animal.

See also

Web links

literature

  • Susannah Calahan: Brain on Fire. My Month of Madness. New York 2012, ISBN 978-1-4516-2137-2 .
  • Susannah Calahan: Fire in the head. My time of madness. Translation: Christa Trautner-Suder, Munich 2013, ISBN 978-3-86882-467-4 .
  • Josep Dalmau u. a .: Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. In: The Lancet Neurology . Volume 7, December 2008, pp. 1091-1098.
  • Josep Dalmau, E. Lancaster, E. Martinez-Hernandez, MR Rosenfeld, R. Balice-Gordon: Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. In: The Lancet Neurology. Volume 10, Number 1, January 2011, pp. 63-74. ISSN  1474-4465 . doi: 10.1016 / S1474-4422 (10) 70253-2 . PMID 21163445 . (Review).
  • Fritz Habekuss: inflamed soul . In: Die Zeit , No. 31/2014, p. 29.

Individual evidence

  1. ↑ Josep Dalmau, Tüzün Erdem, Wu HaiYan u. a .: Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. In: Annals of Neurology. Volume 61, Issue 1, 2007, pp. 25-36, PMC 2430743 (free full text).
  2. ^ H. Prüss et al .: Anti-NMDA Receptor Encephalitis in the Polar Bear (Ursus maritimus) Knut . In: Nature Scientific Reports (2015): 5, 12805; doi: 10.1038 / srep12805 (open full text)
  3. ↑ Caroline Creten et al. a .: Case Report: Late onset autism and anti-NMDA-receptor encephalitis. In: The Lancet. Volume 378, Issue 9785, 2-8 July 2011, p. 98, PMID 21724038
  4. ↑ MJ Titulaer, L. McCracken, J. Gabilondo et al. a .: Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. In: Lancet Neurology. Volume 12, Issue 2, 2013, pp. 157-165, PMID 23290630 .
  5. ↑ Franziska Scheibe, Harald Prüss, Annerose M. Mengel, Siegfried Kohler, Astrid Nümann: Bortezomib for treatment of therapy-refractory anti-NMDA receptor encephalitis . In: Neurology . tape 88 , no. 4 , January 24, 2017, ISSN  0028-3878 , p. 366-370 , doi : 10.1212 / WNL.0000000000003536 , PMID 28003505 ( neurology.org [accessed January 13, 2018]).
  6. ^ H. Prüss, J. Leubner, NK Wenke, G. Czirják, CA Szentiks, AD Greenwood: Anti-NMDA Receptor Encephalitis in the Polar Bear (Ursus maritimus) Knut. In: Scientific reports. Volume 5, 2015, p. 12805, doi: 10.1038 / srep12805 , PMID 26313569 , PMC 4551079 (free full text).