Autoimmune Encephalitis: A Literature Review

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Tatjana Deleva-Stoshevska
Sofija Nikoloska
Bojan Stoshevski
Marko Nikoloski
Dimitar Veljanovski
Sandra Dejanova-Panov


Autoimmune encephalitis (AIE) defines brain inflammation caused by a misdirected immune response against self-antigens expressed in the central nervous system. AIE encompasses a group of non-infectious immune-mediated inflammatory disorders of the brain parenchyma often involving the cortical or deep gray matter with or without involvement of the white matter, meninges, or the spinal cord. Suggested mechanisms that may trigger AIE include tumors (paraneoplastic), infections (para-infectious), or it may be cryptogenic. This study represents a review of the common forms of AIE, exploring their causes, diagnostic approaches, and management strategies. The previous and ongoing investigations in this field have been driven by the identification of several pathogenic autoantibodies that cause polysymptomatic neuropsychiatric and neurological diseases. AIE comprises a heterogeneous group of disorders that are at least as common as infectious causes of encephalitis. Early treatment is associated with better prognosis and is crucial for the prevention of severe complications. The underlying mechanisms for activation and autoimmune response in the CNS are still unclear. Further investigations are needed to better explain how immune mechanisms affect nervous system functions.


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Deleva-Stoshevska T, Nikoloska S, Stoshevski B, Nikoloski M, Veljanovski D, Dejanova-Panov S. Autoimmune Encephalitis: A Literature Review. SEE J Immunol [Internet]. 2023 Jul. 15 [cited 2024 Jun. 16];6(1):29-33. Available from:
Clinical Immunology


Graus F, Titulaer MJ, Balu R, Benseler S, Bien CG, Cellucci T, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016;15(4):391-404. S1474-4422(15)00401-9 PMid:26906964

Singh TD, Fugate JE, Rabinstein AA. The spectrum of acute encephalitis: Causes, management, and predictors of outcome. Neurology. 2015;84:359-66. PMid:25540320

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(7):899-904. PMid:22281844

Lancaster E. Paraneoplastic disorders. Continuum (Minneap Minn). 2017;23(6):1653-79. PMid:29200116

Abboud H, Rossman I, Mealy MA, Hill E, Thompson N, BanerjeeA, et al. Neuronal autoantibodies: Differentiating clinically relevant and clinically irrelevant results. J Neurol. 2017;264(11):2284-92. PMid:28975404

Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: An observational cohort study. Lancet Neurol. 2013;12(2):157-65. PMid:23290630

Vincent A, Bien CG, Irani SR, Waters P. Autoantibodies associated with diseases of the CNS: New developments and future challenges. Lancet Neurol. 2011;10(8):759-72. PMid:21777830

Titulaer MJ, Höftberger R, Iizuka T, Leypoldt F, McCracken L, Cellucci T, et al. Overlapping demyelinating syndromes and anti-N-methyl-D-aspartate receptor encephalitis. Ann Neurol. 2014;75(3):411-28. PMid:24700511

Tobin WO, Guo Y, Krecke KN, Parisi JE, Lucchinetti CF, Pittock SJ, et al. Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. 2017;140(9):2415-25. PMid:29050399

Kumar N, Abboud H. Iatrogenic CNS demyelination in the era of modern biologics. Mult Scler. 2019;25(8):1079-85. PMid:30767720

Dubey D, Britton J, McKeon A, Gadoth A, Zekeridou A, Chiriboga SA, et al. Randomized placebo-controlled trial of intravenous immunoglobulin in autoimmune LGI1/CASPR2 epilepsy. Ann Neurol. 2020;87(2):313-23. PMid:31782181

Nguyen MK, Rastogi A, Kurtz I. True hyponatremia secondary to intravenous immunoglobulin. Clin Exp Nephrol. 2006;10(2):124-6. PMid:16791398

Abboud H, Petrak A, Mealy M, Sasidharan S, Siddique L, Levy M. Treatment of acute relapses in neuromyelitis optica: Steroids alone versus steroids plus plasma exchange. Mult Scler. 2016;22(2):185-92. PMid:25921047

DeSena AD, Noland DK, Matevosyan K, King K, Phillips L, Qureshi SS, et al. Intravenous methylprednisolone versus therapeutic plasma exchange for treatment of anti-N-methyl-D- aspartate receptor antibody encephalitis: A retrospective review. J Clin Apher. 2015;30(4):212-16. PMid:25664728

Höftberger R, Titulaer MJ, Sabater L, Dome B, Rózsás A, Hegedus B, et al. Encephalitis and GABAB receptor antibodies: Novel findings in a new case series of 20 patients. Neurol. 2013;81(17):1500-6. PMid:24068784