Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is a life-threatening disorder that often occurs as a paraneoplastic encephalitis and usually begins with neuropsychological or psychiatric symptoms. We report a case of NMDA receptor encephalitis due to an ovarian teratoma, which began with severe and progressive amnesia and behavioral changes, reversed after surgical treatment and plasmapheresis. Using a battery of cognitive tests, its neuropsychological profile before treatment showed a complete alteration of the short and long term memory of both verbal and visual fixation, with clear improvement with cues and with intrusions, and saving other cognitive domains, such as working, episodic and semantic memory, executive, visuospatial, praxical thinking and language functions. These deficits reverted to normalcy with treatment. So, we can conclude that anti-NMDA receptor encephalitis is a rare entity that can be potentially serious depending on early management and diagnosis. We must suspect this entity in children or young people presenting with behavioural disturbances and crisis, with a cognitive pattern of complete alteration in short and long term memory improving with cues, and respecting other cognitive domains.
Alexopoulos, H., Kosmidis, M. L., Dalmau, J., & Dalakas, M. C. (2011) Paraneoplastic anti-NMDAR encephalitis: Long term follow-up reveals persistent serum antibodies. Journal of Neurology, 258, 1568-1570. http://dx.doi.org/10.1007/s00415-011-5982-4
Armangue, T., Petit-Pedrol, M., Dalmau, J. (2012). Autoimmune encephalitis in children. Journal Child Neurology, volume 11, pages 1460-9. http://dx.doi.org/10.1177/0883073812448838
Boeck, A.L., Logemann, F., Krauß, T., Hussein, K., Bültmann, E., Trebst, C., & Stangel, M. (2013). Ovarectomy despite negative imaging in anti-NMDA receptor encephalitis: Effective even late. Case Reports in Neurological Medicine, 2013, Article ID: 843192.
Buschke, H., Kuslansky, G., Katz, M., Stewart, W. F., Sliwinski, M. J., Eckholdt, H. M., & Lipton, R. B. (1999). Screening for dementia withthe memory impairment screen. Neurology, 2, 231-238. http://dx.doi.org/10.1212/WNL.52.2.231
Carnero, C., & Montoro, M. T. (2004). El Test de las Fotos. Revista de Neurología, 9, 801-806.
Dalmau, J., Gleichman, A. J., Hughes, E. G., et al. (2008) Anti-NMDA-receptor encephalitis: Case series and analysis of the effects of antibodies. The Lancet Neurology, 7, 1091-1098. http://dx.doi.org/10.1016/S1474-4422(08)70224-2
Dabner, M., McCluggage, W. G., Bundell, C., Carr, A., Leung, Y., Sharma, R., & Stewart, C. J. (2012). Ovarian teratoma associated with anti-N-methyl D-aspartate receptor encephalitis: A report of 5 cases documenting prominent intratumoral lymphoid infiltrates. International Journal of Gynecological Pathology, 31, 429-437. http://dx.doi.org/10.1097/PGP.0b013e31824a1de2
Dabner, M., McCluggage, W. G., Bundell, C., Carr, A., Leung, Y., Sharma, R., & Stewart, C. J. (2012). Ovarian teratoma associated with anti-N-methyl D-aspartate receptor encephalitis: A report of 5 cases documenting prominent intratumoral lymphoid infiltrates. International Journal of Gynecological Pathology, 31, 429-437. http://dx.doi.org/10.1097/PGP.0b013e31824a1de2
Finke, C., Kopp, U. A., Prüss, H., Dalmau, J., Wandinger, K. P., & Ploner, C. J. (2012). Cognitive deficits following anti-NMDA receptor encephalitis. Journal of Neurology, Neurosurgery & Psychiatry, 83, 195-198. http://dx.doi.org/10.1136/jnnp-2011-300411
Iizuka, T., Yoshii, S., Kan, S., et al. (2010). Reversible brain atrophy in anti-NMDA receptor encephalitis: A long-term observational study. Journal of Neurology, 257, 1686-1691. http://dx.doi.org/10.1007/s00415-010-5604-6
Johnson, N., Henry, C., Fessler, A. J., & Dalmau, J. (2010). Anti-NMDA receptor encephalitis causing prolonged nonconvulsive status epilepticus. Neurology, 75, 1480-1482. http://dx.doi.org/10.1212/WNL.0b013e3181f8831a
Lebon, S., Mayor-Dubois, C., Popea, I., Poloni, C., Selvadoray, N., Gumy, A., & Roulet-Perez, E. (2012). Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis mimicking a primary psychiatric disorder in an adolescent. Journal of Child Neurology, 27, 1607-1610. http://dx.doi.org/10.1177/0883073812438099
Peery, H. E., Day, G. S., Doja, A., Xia, C., Fritzler, M. J., & Foster, W. G. (2013). Anti-NMDA receptor encephalitis in children: The disorder, its diagnosis, and treatment. Handbook of Clinical Neurology, 112, 1229-1233. http://dx.doi.org/10.1016/B978-0-444-52910-7.00045-3
Pham, H. P., Daniel-Johnson, J. A., Stotler, B. A., Stephens, H., & Schwartz, J. (2011). Therapeutic plasma exchange for the treatment of anti-NMDA receptor encephalitis. Journal of Clinical Apheresis, 26, 320-325. http://dx.doi.org/10.1002/jca.20311
Prüss, H., Dalmau, J., Harms, L., Holtje, M., Ahnert-Hilger, G., Borowski, K., Stoecker, W., & Wandinger, K. P. (2010). Retrospective analysis of NMDA receptor antibodies in encephalitis of unknown origin. Neurology, 75, 1735-1739. http://dx.doi.org/10.1212/WNL.0b013e3181fc2a06
Rosenfeld, M. R., Dalmau, J. (2011). Anti-NMDA-receptor encephalitis and other synaptic autoimmune disorders. Current Treatment Options in Neurology, 13, 324-332. http://dx.doi.org/10.1007/s11940-011-0116-y
Rosenfeld, M. R., & Dalmau, J. O. (2012). Paraneoplastic disorders of the CNS and autoimmune synaptic encephalitis. Continuum (Minneaplis, Minn), 2, 366-383. http://dx.doi.org/10.1212/01.CON.0000413664.42798.aa
Rosenfeld, M. R., Titulaer, M. J., & Dalmau, J. (2012). Paraneoplastic syndromes and autoimmune encephalitis: Five new things. Neurology Clinical Practice, 2, 215-223. http://dx.doi.org/10.1212/CPJ.0b013e31826af23e
Suzuki, S., Seki, M., & Suzuki, N. (2013). Recent concept of limbic encephalitis: Progress in anti-NMDA receptor encephalitis. Japanese Journal of Clinical Immunology, 36, 86-94. http://dx.doi.org/10.2177/jsci.36.86
Tanyi, J. L., Marsh, E. B., Dalmau, J., & Chu, C. S. (2012). Reversible paraneoplastic encephalitis in three patients with ovarian neoplasms. Acta Obstetricia et Gynecologica Scandinavica, 91, 630-634.
Titulaer, M. J., McCracken, L., Gabilondo, I., et al. (2013). Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: An observational cohort study. The Lancet Neurology, 12, 157-165. http://dx.doi.org/10.1016/S1474-4422(12)70310-1
Young, P. J., Baker, S., Cavazzoni, E., Erickson, S. J., Krishnan, A., Kruger, P. S., Rashid, A. H., & Wibrow, B. A. (2013). A case series of critically ill patients with anti N-methyl-D-aspartate receptor encephalitis. Critical Care and Resuscitation, 1, 8-14.
Waas, J. A., & Storm, A. H. (2012). Anti-NMDA-receptor encephalitis: A neuropsychiatric illness requiring further study. Tijdschrift voor Psychiatrie, 54, 279-283.
Zuliani, F., Graus, F., Giometto, B., et al. (2012). Central nervous system neuronal surface antibody associated syndromes: Review and guidelines for recognition. Journal of Neurology, Neurosurgery & Psychiatry, 83, 638-645. http://dx.doi.org/10.1136/jnnp-2011-301237