Anti-NMDA receptor encephalitis

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Anti-NMDA receptor encephalitis

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Anti-NMDA (N-methyl D-aspartate) receptor encephalitis is an acute form of encephalitis, potentially lethal but with high probability for recovery, caused by autoimmune reaction against NR1- and NR2-subunits of the glutamate NMDA receptor.[1] Different descriptions and syndromal designations for this disease existed in the medical literature prior to 2007, when the cause was established and it received its current name.[2] The disease is associated with tumours, mostly teratomas of the ovaries, and thus is considered a paraneoplastic syndrome. Howerever, there are a substantial number of cases with no detectable cancerous tissue.

According to a review of 100 cases in The Lancet,[3] 91 of the 100 patients were women, mean age of all patients numbered 23 years (5-76 range); of the 98 patients that underwent an oncological screening 58 had a tumour, predominantly an ovarian teratoma. All patients presented with psychiatric symptoms or memory problems; 76 of them suffered from seizures, 88 developed unresponsiveness (decreased consciousness), 86 - dyskinesias, 69 - autonomic instability, and 66 - hypoventilation. Tumour treatment associated with better prognosis; 75 patients got well without residual problems and 25 died or were left with severe deficits.

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References

  1. ^ Iizuka T, Sakai F, Ide T, Monzen T, Yoshii S, Iigaya M, Suzuki K, Lynch DR, Suzuki N, Hata T, Dalmau J (February 2008). "Anti-NMDA receptor encephalitis in Japan: long-term outcome without tumor removal". Neurology 70 (7): 504–11. doi:10.1212/01.wnl.0000278388.90370.c3. PMC 2586938. PMID 17898324. http://www.neurology.org/cgi/pmidlookup?view=long&pmid=17898324. 
  2. ^ Dalmau J, Tüzün E, Wu HY, Masjuan J, Rossi JE, Voloschin A, Baehring JM, Shimazaki H, Koide R, King D, Mason W, Sansing LH, Dichter MA, Rosenfeld MR, Lynch DR (January 2007). "Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma". Ann. Neurol. 61 (1): 25–36. doi:10.1002/ana.21050. PMC 2430743. PMID 17262855. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2430743. 
  3. ^ Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, Dessain SK, Rosenfeld MR, Balice-Gordon R, Lynch DR (December 2008). "Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies". Lancet Neurol 7 (12): 1091–8. doi:10.1016/S1474-4422(08)70224-2. PMC 2607118. PMID 18851928. http://linkinghub.elsevier.com/retrieve/pii/S1474-4422(08)70224-2. 

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