Abstract
Autoimmune encephalitis associated with antibodies targeting neural cell surface antigens have emerged in the past 10 years as a major cause of encephalitis. Those associated with antibodies against the N-methyl-D-aspartate receptor (NMDAR) is the most frequent. This newly recognized disease is characterized by a stereotyped clinical phenotype. The clinical course usually begins in the majority of cases by psychiatric symptoms, sometimes preceded by prodromal symptoms. The psychiatric stage is usually followed by severe fluctuations in consciousness with neurologic involvement with cognitive impairment, speech impairment, movement disorders, seizures, and behavioral problems. Typically the disease affects young women, and an ovarian teratoma is frequently associated, but cases have been reported in children, in men, and in patients without tumors. The treatment consists of early immunotherapy and, if necessary, tumor removal. The outcome is good in many cases if the treatment is started early, but severe sequelae or death is possible. The management of psychiatric symptoms can be difficult.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Graus F, Delattre JY, Antoine JC, Dalmau J, Giometto B, Grisold W, et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. 2004;75:1135–40.
Honnorat J, Cartalat-Carel S, Ricard D, Camdessanche JP, Carpentier AF, Rogemond V, et al. Onco-neural antibodies and tumour type determine survival and neurological symptoms in paraneoplastic neurological syndromes with Hu or CV2/CRMP5 antibodies. J Neurol Neurosurg Psychiatry. 2009;80:412–6.
Graus F, Saiz A, Dalmau J. Antibodies and neuronal autoimmune disorders of the CNS. J Neurol. 2010;257:509–17.
Zuliani L, Graus F, Giometto B, Bien C, Vincent A. Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition. J Neurol Neurosurg Psychiatry. 2012;83:638–45.
Coutinho E, Harrison P, Vincent A. Do neuronal autoantibodies cause psychosis? A neuroimmunological perspective. Biol Psychiatry. 2014;75:269–75.
Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 2000;123:1481–94.
Dalmau J, Tüzün E, Wu HY, Masjuan J, Rossi JE, Voloschin A, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61:25–36.
Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol. 2011;10:63–74.
Kayser MS, Dalmau J. Anti-NMDA receptor encephalitis in psychiatry. Curr Psychiatry Rev. 2011;7:189–93.
Kayser MS, Dalmau J. The emerging link between autoimmune disorders and neuropsychiatric disease. J Neuropsychiatry Clin Neurosci. 2011;23:90–7.
Irani SR, Alexander S, Waters P, Kleopa KA, Pettingill P, Zuliani L, et al. Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan’s syndrome and acquired neuromyotonia. Brain. 2010;133:2734–48.
Gable MS, Sheriff H, Dalmau J, Tilley DH, Glaser CA. The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project. Clin Infect Dis. 2012;54:899–904.
Buckley C, Oger J, Clover L, Tüzün E, Carpenter K, Jackson M, et al. Potassium channel antibodies in two patients with reversible limbic encephalitis. Ann Neurol. 2001;50:73–8.
Lai M, Huijbers MG, Lancaster E, Graus F, Bataller L, Balice-Gordon R, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol. 2010;9:776–85.
Takahashi Y, Mori H, Mishina M, Watanabe M, Fujiwara T, Shimomura J, et al. Autoantibodies to NMDA receptor in patients with chronic forms of epilepsia partialis continua. Neurology. 2003;61:891–6.
Vitaliani R, Mason W, Ances B, Zwerdling T, Jiang Z, Dalmau J. Paraneoplastic encephalitis, psychiatric symptoms, and hypoventilation in ovarian teratoma. Ann Neurol. 2005;58:594–604.
Ances BM, Vitaliani R, Taylor RA, Liebeskind DS, Voloschin A, Houghton DJ, et al. Treatment-responsive limbic encephalitis identified by neuropil antibodies: MRI and PET correlates. Brain. 2005;128:1764–77.
Dalmau J, Tüzün E, Wu HY, Masjuan J, Rossi JE, Voloschin A, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61:25–36.
Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol. 2011;10:63–74.
Miya K, Takahashi Y, Mori H. Anti-NMDAR autoimmune encephalitis. Brain Dev. 2014;36:645–52.
Rosenthal-Simons A, Durrant AR, Heresco-Levy U. Autoimmune-induced glutamatergic receptor dysfunctions: conceptual and psychiatric practice implications. Eur Neuropsychopharmacol. 2013;23:1659–71.
Farber NB. The NMDA receptor hypofunction model of psychosis. Ann N Y Acad Sci. 2003;1003:119–30.
Gruber O, Chadha Santuccione A, Aach H. Magnetic resonance imaging in studying schizophrenia, negative symptoms, and the glutamate system. Front Psychiatry. 2014;5:32.
Prüss H, Dalmau J, Harms L, Höltje M, Ahnert-Hilger G, Borowski K, et al. Retrospective analysis of NMDA receptor antibodies in encephalitis of unknown origin. Neurology. 2010;75(19):1735–9.
Granerod J, Ambrose HE, Davies NW, Clewley JP, Walsh AL, Morgan D, et al. Causes of encephalitis and differences in their clinical presentations in England: a multicentre, population-based prospective study. Lancet Infect Dis. 2010;10:835–44.
Gable MS, Sheriff H, Dalmau J, Tilley DH, Glaser CA. The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project. Clin Infect Dis. 2012;54:899–904.
Sansing LH, Tüzün E, Ko MW, Baccon J, Lynch DR, Dalmau J. A patient with encephalitis associated with NMDA receptor antibodies. Nat Clin Pract Neurol. 2007;3:291–6.
Iizuka T, Sakai F, Ide T, Monzen T, Yoshii S, Iigaya M, et al. Anti-NMDA receptor encephalitis in Japan: long-term outcome without tumor removal. Neurology. 2008;70:504–11.
Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, et al. Anti-NMDA Receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008;7:1091–8.
Florance NR, Davis RL, Lam C, Szperka C, Zhou L, Ahmad S, et al. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents. Ann Neurol. 2009;66:11–8.
Niehusmann P, Dalmau J, Rudlowski C, Vincent A, Elger CE, Rossi JE, et al. Diagnostic value of N-methyl-D-aspartate receptor antibodies in women with new-onset epilepsy. Arch Neurol. 2009;66:458–64.
Rubio-Agustà I, Dalmau J, Sevilla T, Burgal M, Beltrán E, Bataller L. Isolated hemidystonia associated with NMDA receptor antibodies. Mov Disord. 2011;26:351–2.
Gresa-Arribas N, Titulaer MJ, Torrents A, Aguilar E, McCracken L, Leypoldt F, et al. Antibody titres at diagnosis and during follow-up of anti-NMDA receptor encephalitis: a retrospective study. Lancet Neurol. 2014;13:167–77.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer International Publishing Switzerland
About this chapter
Cite this chapter
Brochet, B. (2015). Psychiatric Presentation of Brain Inflammation. In: Brochet, B. (eds) Neuropsychiatric Symptoms of Inflammatory Demyelinating Diseases. Neuropsychiatric Symptoms of Neurological Disease. Springer, Cham. https://doi.org/10.1007/978-3-319-18464-7_7
Download citation
DOI: https://doi.org/10.1007/978-3-319-18464-7_7
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-18463-0
Online ISBN: 978-3-319-18464-7
eBook Packages: MedicineMedicine (R0)