Autoimmune Limbic Encephalitis – literature review
DOI:
https://doi.org/10.12775/JEHS.2024.56.006Keywords
autoimmune limbic encephalitis, autoantibodies, immunotherapyAbstract
Autoimmune Limbic Encephalitis is a rare disease occurring with a frequency of approximately 13.7/100,000 people, mainly among middle-aged women. The disease is caused by autoantibodies, either synthesised due to the presence of a tumour or having an idiopathic nature. Depending on the type of autoantibodies detected, the disease is divided into subtypes, which differ in the frequency of specific clinical symptoms (most commonly psychosis, mood changes, memory problems, cognitive impairment and seizures) and can direct to detection of a specific type of cancer. Making a prompt diagnosis and initiating treatment is crucial as it ensures a reduction in clinical symptoms and improves survival rate. Broad immunotherapy is used - intravenous immunoglobulin, corticosteroids, azathioprine, rituximab, cyclophosphamide, anti-epileptic drugs and plasmapheresis. The efficacy is high - 80% of patients recover, and relapses occur in only 10% of cases. If a patient is diagnosed with cancer, effective oncological treatment is necessary to achieve complete remission.
References
Tüzün E, Dalmau J. Limbic encephalitis and variants: classification, diagnosis and treatment. Neurologist. 2007;13(5):261-271. doi:10.1097/NRL.0b013e31813e34a5
Höftberger R, Titulaer MJ, Sabater L, et al. Encephalitis and GABAB receptor antibodies: novel findings in a new case series of 20 patients. Neurology. 2013;81(17):1500-1506. doi:10.1212/WNL.0b013e3182a9585f
Dubey D, Pittock SJ, Kelly CR, et al. Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis. Ann Neurol. 2018;83(1):166-177. doi:10.1002/ana.25131
Honnorat J, Didelot A, Karantoni E, et al. Autoimmune limbic encephalopathy and anti-Hu antibodies in children without cancer. Neurology. 2013;80(24):2226-2232. doi:10.1212/WNL.0b013e318296e9c3
Graus F, Titulaer MJ, Balu R, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016;15(4):391-404. doi:10.1016/S1474-4422(15)00401-9
Schermann H, Ponomareva IV, Maltsev VG, Yakushev KB, Sherman MA. Clinical variants of limbic encephalitis. SAGE Open Med Case Rep. 2019;7:2050313X19846042. Published 2019 May 2. doi:10.1177/2050313X19846042
Oyanguren B, Sánchez V, González FJ, et al. Limbic encephalitis: a clinical-radiological comparison between herpetic and autoimmune etiologies. Eur J Neurol. 2013;20(12):1566-1570. doi:10.1111/ene.12249
Lancaster E, Dalmau J. Neuronal autoantigens--pathogenesis, associated disorders and antibody testing. Nat Rev Neurol. 2012;8(7):380-390. Published 2012 Jun 19. doi:10.1038/nrneurol.2012.99
Budhram A, Leung A, Nicolle MW, Burneo JG. Diagnosing autoimmune limbic encephalitis. CMAJ. 2019;191(19):E529-E534. doi:10.1503/cmaj.181548
Munshi SK, Thanvi B, Chin SK, Hubbard I, Fletcher A, Vallance TR. Paraneoplastic limbic encephalitis--case report and review of literature. Age Ageing. 2005;34(2):190-193. doi:10.1093/ageing/afi017
Dalmau J, Gleichman AJ, Hughes EG, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008;7(12):1091-1098. doi:10.1016/S1474-4422(08)70224-2
Titulaer MJ, McCracken L, Gabilondo I, 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):157-165. doi:10.1016/S1474-4422(12)70310-1
Spatola M, Sabater L, Planagumà J, et al. Encephalitis with mGluR5 antibodies: Symptoms and antibody effects. Neurology. 2018;90(22):e1964-e1972. doi:10.1212/WNL.0000000000005614
Laurido-Soto O, Brier MR, Simon LE, McCullough A, Bucelli RC, Day GS. Patient characteristics and outcome associations in AMPA receptor encephalitis. J Neurol. 2019;266(2):450-460. doi:10.1007/s00415-018-9153-8
Irani SR, Alexander S, Waters P, 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(9):2734-2748. doi:10.1093/brain/awq213
Irani SR, Michell AW, Lang B, et al. Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Ann Neurol. 2011;69(5):892-900. doi:10.1002/ana.22307
van Sonderen A, Ariño H, Petit-Pedrol M, et al. The clinical spectrum of Caspr2 antibody-associated disease. Neurology. 2016;87(5):521-528. doi:10.1212/WNL.0000000000002917
van Coevorden-Hameete MH, de Bruijn MAAM, de Graaff E, et al. The expanded clinical spectrum of anti-GABABR encephalitis and added value of KCTD16 autoantibodies. Brain. 2019;142(6):1631-1643. doi:10.1093/brain/awz094
Mansoor, S., Murphy, K. Anti-GAD-associated limbic encephalitis: an unusual clinical manifestation from northwest of Ireland. Egypt J Neurol Psychiatry Neurosurg 56, 23 (2020). https://doi.org/10.1186/s41983-020-0160-1
Dalmau J, Graus F, Rosenblum MK, Posner JB. Anti-Hu--associated paraneoplastic encephalomyelitis/sensory neuronopathy. A clinical study of 71 patients. Medicine (Baltimore). 1992;71(2):59-72. doi:10.1097/00005792-199203000-00001
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 ( Pt 7):1481-1494. doi:10.1093/brain/123.7.1481
Dalmau J, Graus F, Villarejo A, et al. Clinical analysis of anti-Ma2-associated encephalitis. Brain. 2004;127(Pt 8):1831-1844. doi:10.1093/brain/awh203
Castle J, Sakonju A, Dalmau J, Newman-Toker DE. Anti-Ma2-associated encephalitis with normal FDG-PET: a case of pseudo-Whipple's disease. Nat Clin Pract Neurol. 2006;2(10):566-573. doi:10.1038/ncpneuro0287
Yu Z, Kryzer TJ, Griesmann GE, Kim K, Benarroch EE, Lennon VA. CRMP-5 neuronal autoantibody: marker of lung cancer and thymoma-related autoimmunity. Ann Neurol. 2001;49(2):146-154.
Baumgartner, A., Rauer, S., Hottenrott, T. et al. Admission diagnoses of patients later diagnosed with autoimmune encephalitis. J Neurol 266, 124–132 (2019). https://doi.org/10.1007/s00415-018-9105-3
Granerod J, Ambrose HE, Davies NW, et al.: Causes of encephalitis and differences in their clinical presentations in England: a multicentre, population-based prospective study. Lancet Infect Dis. 2010,
:835-44. 10.1016/s1473-3099(10)70222-x
van Sonderen A, Thijs RD, Coenders EC, et al. Anti-LGI1 encephalitis: clinical syndrome and long-term follow-up. Neurology 2016;87:1449-56.
Chow FC, Glaser CA, Sheriff H, et al. Use of clinical and neuroimaging characteristics to distinguish temporal lobe herpes simplex encephalitis from its mimics. Clin Infect Dis 2015;60:1377-83.
Vogrig A, Joubert B, Ducray F, et al. Glioblastoma as differential diagnosis of autoimmune encephalitis. J Neurol 2018;265:669-77.
Hosoki S, Satoi H, Matsumoto S. Bilateral hippocampal infarction mimicking limbic encephalitis. Intern Med 2018;57:911-2.
Kim JA, Chung JI, Yoon PH, et al. Transient MR signal changes in patients with generalized tonicoclonic seizure or status epilepticus: periictal diffusionweighted imaging. AJNR Am J Neuroradiol 2001;22:1149-60.
Newey CR, Sarwal A, Hantus S. [(18)F]-fluoro-deoxy-glucose positron emission tomography scan should be obtained early in cases of autoimmune encephalitis. Autoimmune Dis 2016;2016:9450452.
Pessa ME, Janes F, Gigli GL. Electroencephalographic evaluation for early diagnosis of limbic encephalitis. Clin EEG Neurosci 2016;47:207-10.
Vincent A, Buckley C, Schott JM, et al. Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain 2004;127:701-12.
Malter MP, Elger CE, Surges R. Diagnostic value of CSF findings in antibody-associated limbic and anti-NMDAR-encephalitis. Seizure. 2013;22(2):136-140. doi:10.1016/j.seizure.2012.12.013
Weil AA, Glaser CA, Amad Z, Forghani B. Patients with suspected herpes simplex encephalitis: rethinking an initial negative polymerase chain reaction result. Clin Infect Dis. 2002;34(8):1154-1157. doi:10.1086/339550
Gadoth A, Pittock SJ, Dubey D, et al. Expanded phenotypes and outcomes among 256 LGI1/CASPR2-IgG-positive patients. Ann Neurol. 2017;82(1):79-92. doi:10.1002/ana.24979
Lancaster E, Lai M, Peng X, et al. Antibodies to the GABA(B) receptor in limbic encephalitis with seizures: case series and characterisation of the antigen. Lancet Neurol. 2010;9(1):67-76. doi:10.1016/S1474-4422(09)70324-2
Mueller C, Elben S, Day GS, et al. Review and meta-analysis of neuropsychological findings in autoimmune limbic encephalitis with autoantibodies against LGI1, CASPR2, and GAD65 and their response to immunotherapy. Clin Neurol Neurosurg. 2023;224:107559. doi:10.1016/j.clineuro.2022.107559
Viaccoz A, Desestret V, Ducray F, et al. Clinical specificities of adult male patients with NMDA receptor antibodies encephalitis. Neurology. 2014;82(7):556-563. doi:10.1212/WNL.0000000000000126
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2024 Szymon Pokora, Karolina Pokora, Alicja Poloczek, Jakub Szczerba
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
The periodical offers access to content in the Open Access system under the Creative Commons Attribution-NonCommercial-ShareAlike 4.0
Stats
Number of views and downloads: 321
Number of citations: 0