Rasmussen脑炎发病机制的研究现状
The Pathogenesis and Research Status of Rasmussen Encephalitis
DOI: 10.12677/HJS.2022.111001, PDF,    国家自然科学基金支持
作者: 刘 东：首都医科大学附属北京天坛医院，北京；首都医科大学三博脑科医院，北京；栾国明*：首都医科大学三博脑科医院神经外一科，北京；癫痫病临床医学研究北京市重点实验室，北京；北京脑重大疾病研究院癫痫研究所，北京
关键词: Rasmussen脑炎；癫痫；发病机制；病毒感染；自身免疫；Rasmussen Encephalitis； Pathogenesis； Epilepsy； Virus Infection； Autoimmunity
摘要: Rasmussen脑炎(Rasmussen encephalitis, RE)是一种罕见，儿童时期起病，常累及一侧大脑半球的慢性炎症性疾病，表现为难治性局灶性癫痫、部分性癫痫持续状态(epilepsia partialis continua, EPC)和进行性加重的神经功能缺损症状如偏瘫、偏盲和智力减退等。近半个世纪以来，尽管对于这种严重致残性疾病的研究不断取得重大进展，但该病的病因至今尚不明确，大脑半球离断术仍是临床治疗最重要的方法。本文就RE发病机制的研究现状做一综述，供同行参考。
Abstract: Rasmussen encephalitis (RE) is a rare, childhood onset, and chronic encephalitis involving one side of the hemisphere. This disease manifested as refractory focal epilepsy, epilepsia partialis continua (EPC) and progressive neurological deficits such as hemiplegia, hemianopia and mental retardation. For nearly half a century, the significant progress of the disease has been made significant progress, but the pathogenesis of the disease is not clear, the hemispherotomy is still the most effective clinical treatment. This paper will review the pathogenesis of RE for colleague’s reference.
文章引用：刘东, 栾国明. Rasmussen脑炎发病机制的研究现状[J]. 外科, 2022, 11(1): 1-6. https://doi.org/10.12677/HJS.2022.111001

参考文献

[1] Varghese, B., Aneesh, M., Singh, N. and Gilwaz, P. (2014) A Case of Rasmussen Encephalitis: The Differential Diagnoses and Role of Diagnostic Imaging. Oman Medical Journal, 29, 67-70. [Google Scholar] [CrossRef] [PubMed]
[2] Luan, G., Gao, Q., Zhai, F., Chen, Y. and Li, T. (2016) Upregulation of HMGB1, Toll-Like Receptor and RAGE in Human Rasmussen’s Encephalitis. Epilepsy Research, 123, 36-49. [Google Scholar] [CrossRef] [PubMed]
[3] Varadkar, S., Bien, C.G., Kruse, C.A., Jensen, F.E., Bauer, J., Pardo, C.A., Vincent, A., Mathern, G.W. and Cross, J.H. (2014) Rasmussen’s Encephalitis: Clinical Features, Pathobiology, and Treatment Advances. Lancet Neurology, 13, 195-205. [Google Scholar] [CrossRef] 
[4] Bien, C.G., Urbach, H., Deckert, M., Schramm, J., Wiestler, O.D., Lassmann, H. and Elger, C.E. (2002) Diagnosis and Staging of Rasmussen’s Encephalitis by Serial MRI and Histopathology. Neurology, 58, 250-257. [Google Scholar] [CrossRef] 
[5] Walter, G.F. and Renella, R.R. (1989) Epstein-Barr Virus in Brain and Rasmussen’s Encephalitis. Lancet, 333, 279-280. [Google Scholar] [CrossRef] 
[6] Farrell, M.A., Cheng, L., Cornford, M.E., Grody, W.W. and Vinters, H.V. (1991) Cytomegalovirus and Rasmussen’s Encephalitis. Lancet, 337, 1551-1552. [Google Scholar] [CrossRef] [PubMed]
[7] Jay, V., Becker, L.E., Otsubo, H., Cortez, M., Hwang, P., Hoffman, H.J. and Zielenska, M. (1995) Chronicencephalitis and Epilepsy (Rasmussen’s Encephalitis): Detection of Cytomegalovirus and Herpessimplex Virus 1 by the Polymerase Chain Reaction and in Situ Hybridization. Neurology, 45, 108-117. [Google Scholar] [CrossRef] 
[8] McLachlan, R.S., Levin, S. and Blume, W.T. (1996) Treatment of Rasmussen’s Syndrome with Ganciclovir. Neurology, 47, 925-928. [Google Scholar] [CrossRef] 
[9] Merkler, D., Horvath, E., Bruck, W., Zinkernagel, R.M., Del la Torre, J.C. and Pinschewer, D.D. (2006) “Viral déjà vu” Elicits Organ-Specific Immune Disease Independent of Reactivity to Self. Journal of Clinical Investigation, 116, 1254-1263. [Google Scholar] [CrossRef] 
[10] Takahashi, Y., Matsuda, K., Kubota, Y., Shimomura, J., Yamasaki, E., Kudo, T., Fukushima, K., Osaka, H., Akasaka, N., Imamura, A., Yamada, S., Kondo, N. and Fujiwara, T. (2006) Vaccination and Infection as Causative Factors in Japanese Patients with Rasmussen Syndrome: Molecular Mimicry and HLA Class I. Journal of Immunology Research, 13, Article ID: 872092. [Google Scholar] [CrossRef] [PubMed]
[11] Chen, S., Chen, S., Guan, Y., Zhang, Y., Qi, X., An, J., Wang, Y. and Luan, G. (2016) Elevated Expression of Human Papillomavirus Antigen in Brain Tissue of Patients with Rasmussen’s Encephalitis. Epilepsy Research, 126, 119-125. [Google Scholar] [CrossRef] [PubMed]
[12] Trippe, J., Steinke, K., Orth, A., Faustmann, P.M., Hollmann, M. and Haase, C.G. (2014) Autoantibodies to Glutamate Receptor Antigens in Multiple Sclerosis and Rasmussen’s Encephalitis. Neuroimmunomodulation, 21, 189-194. [Google Scholar] [CrossRef] [PubMed]
[13] Fukuyama, T., Takahashi, Y., Kubota, Y., Mogami, Y., Imai, K., Kondo, Y., Sakuma, H., Tominaga, K., Oguni, H. and Nishimura, S. (2015) Semi-Quantitative Analyses of Antibodies to N-Methyl-D-Aspartate Type Glutamate Receptor Subunits (GluN2B & GluN1) in the Clinical Course of Rasmussen Syndrome. Epilepsy Research, 113, 34-43. [Google Scholar] [CrossRef] [PubMed]
[14] Rogers, S.W., Andrews, P.I., Gahring, L.C., Whisenand, T., Cauley, K., Crain, B., Hughes, T.E., Heinemann, S.F. and McNamara, J.O. (1994) Autoantibodies to Glutamate Receptor GluR3 in Rasmussen’s Encephalitis. Science, 265, 648-651. [Google Scholar] [CrossRef] [PubMed]
[15] Watson, R., Jiang, Y., Bermudez, I., Houlihan, L., Clover, L., McKnight, K., Cross, J.H., Hart, I.K., Roubertie, A., Valmier, J., Hart, Y., Palace, J., Beeson, D., Vincent, A. and Lang, B. (2004) Absence of Antibodies to Glutamate Receptor Type 3 (GIuR3) in Rasmussen Encephalitis. Neurology, 63, 43-50. [Google Scholar] [CrossRef] 
[16] Yang, R., Puranam, R.S., Butler, L.S., Qian, W.H., He, X.P., Moyer, M.B., Blackburn, K., Andrews, P.I. and McNamara, J.O. (2000) Autoimmunity to Munc-18 in Rasmussen’s Encephalitis. Neuron, 28, 375-383. [Google Scholar] [CrossRef] 
[17] Alvarez-Barón, E., Bien, C.G., Schramm, J., Elger, C.E., Becker, A.J. and Schoch, S. (2008) Autoantibodies to Muncl8, Cerebral Plasma Cells and B-lymphocytes in Rasmussen Encephalitis. Epilepsy Research, 80, 93-97. [Google Scholar] [CrossRef] [PubMed]
[18] Pardo, C.A., Vining, E.P., Guo, L., Skolasky, R.L., Carson, B.S. and Freeman, J.M. (2004) The Pathology of Rasmussen syndrome: Stages of Cortical Involvement and Neuropathological Studies in 45 Hemispherectomies. Epilepsia, 45, 516-526. [Google Scholar] [CrossRef] [PubMed]
[19] Levite, M. and Hermelin, A. (1999) Autoimmunity to the Glutamate Receptor in Mice—A Model for Rasmussen’s Encephalitis? Journal of Autoimmunity, 13, 73-82. [Google Scholar] [CrossRef] [PubMed]
[20] Schneider-Hohendorf, T., Mohan, H., Bien, C.G., Breuer, J., Becker, A., Görlich, D., Kuhlmann, T., Widman, G., Herich, S., Elpers, C., Melzer, N., Dornmair, K., Kurlemann, G., Wiendl, H. and Schwab, N. (2016) CD8+ T-Cell Pathogenicity in Rasmussen Encephalitis Elucidated by Large-Scale T-Cell Receptor Sequencing. Nature Communications, 7, Article No. 11153. [Google Scholar] [CrossRef] [PubMed]
[21] Gahring, L., Carlson, N.G., Meyer, E.L. and Rogers, S.W. (2001) Granzyme B Proteolysis of a Neuronal Glutamate Receptor Generates an Autoantigen and Is Modulated by Glycosylation. The Journal of Immunology, 166, 1433-1438. [Google Scholar] [CrossRef] [PubMed]
[22] Bien, C.G. and Scheffer, I.E. (2011) Autoantibodies and Epilepsy. Epilepsia, 52, 18-22. [Google Scholar] [CrossRef] [PubMed]
[23] Bien, C.G., Granata, T., Antozzi, C., Cross, J.H., Dulac, O., Kurthen, M., Lassmann, H., Mantegazza, R., Villemure, J.G., Spreafico, R. and Elger, C.E. (2005) Pathogenesis, Diagnosis and Treatment of Rasmussen Encephalitis: A European Consensus Statement. Brain, 128, 454-471. [Google Scholar] [CrossRef] [PubMed]
[24] Woiciechowsky, C., Schöning, B., Stoltenburg-Didinger, G., Stock-hammer, F. and Volk, H.D. (2004) Brain-IL-1 Beta Triggers Astrogliosis through Induction of IL-6: Inhibition by Propranolol and IL-10. Medical Science Monitor, 10, BR325-BR330.
[25] Bauer, J., Elger, C.E., Hans, V.H., Schramm, J., Urbach, H., Lassmann, H. and Bien, C.G. (2007) Astrocytes Are a Specific Immunological Target in Rasmussen Encephalitis. Annals of Neurology, 62, 67-80. [Google Scholar] [CrossRef] [PubMed]
[26] Takahashi, Y., Mogami, Y., Mine, J., Imai, K., Koide, Y., Matsuda, K., Akasaka, N., Konishi, T., Imamura, A. and Inoue, Y. (2013) Genetic Variations of Immunoregulatory Genes Associated with Rasmussen Syndrome. Epilepsy Research, 107, 238-243. [Google Scholar] [CrossRef] [PubMed]
[27] Takahashi, Y., Mine, J., Kubota, Y., Yamazaki, E. and Fujiwara, T. (2009) A Substantial Number of Rasmussen Syndrome Patients Have Increased IgG, CD4+ T cells, TNFalpha, and Granzyme B in CSF. Epilepsia, 50, 1419-1431. [Google Scholar] [CrossRef] [PubMed]
[28] Mirones, I., de Prada, I., Gómez, A.M., Luque, A., Martín, R., Pérez-Jiménez, M.Á., Madero, L., García-Castro, J. and Ramírez, M. (2013) A Role for the CXCR3/CXCL10 Axis in Rasmussen Encephalitis. Pediatric Neurology, 49, 451-457. [Google Scholar] [CrossRef] [PubMed]
[29] Khojah, A. and Klein-Gitelman, M. (2016) Evidence for Resident Memory T cells in Rasmussen Encephalitis. ediatric Neurology Briefs, 30, 22. [Google Scholar] [CrossRef] [PubMed]
[30] Avberšek, A., Miserocchi, A., McEvoy, A.W., Patel, A.V., Aronica, E., Blümcke, I., Jacques, T.S., Acheson, J., Thom, M. and Sisodiya, S.M. (2015) Multiphasic Presentation of Rasmussen’s Encephalitis. Epileptic Disorders, 17, 315-320. [Google Scholar] [CrossRef] [PubMed]
[31] Shetty-Alva, N., Novotny, E.J., Shetty, T. and Kuo, P.H. (2007) Positron Emission Tomography in Rasmussen’s Encephalitis. Pediatric Neurology, 36, 112-114. [Google Scholar] [CrossRef] [PubMed]
[32] Pulsifer, M.B., Brandt, J., Salorio, C.F. and Vining, E.P., Carson, B.S. and Freeman, J.M. (2004) The Cognitive Outcome of Hemispherectomy in 71 Children. Epilepsia, 45, 243-254. [Google Scholar] [CrossRef] [PubMed]
[33] Shioda, M., Oguni, H., Ito, Y., Ochiai, T., Hori, T., Muto, A., Takahashi, S., Miyamoto, A., Osaka, H. and Osawa, M. (2011) Three Children with Rasmussen Encephalitis Showing Marked Improvement in Daily Life Activity Following Functional Hemispherectomy. No To Hattatsu, 43, 373-377.
[34] Kruse, C.A., Pardo, C.A., Hartman, A.L., Jallo, G., Vining, E.P., Voros, J., Gaillard, W.D., Liu, J., Oluigbo, C., Malone, S., Bleasel, A.F., Dexter, M., Micati, A., Velasco, T.R., Machado, H.R., Martino, A.M., Huang, A., Wheatley, B.M., Grant, G.A., Granata, T., Freri, E., Garbelli, R., Koh, S., Nordli, D.R., Campos, A.R., O’Neill, B., Handler, M.H., Chapman, K.E., Wilfong, A.A., Curry, D.J., Yaun, A., Madsen, J.R., Smyth, M.D., Mercer, D., Bingaman, W., Harvey, A.S., Leventer, R.J., Lockhart, P.J., Gillies, G., Pope, K., Giller, C.A., Park, Y.D., Rojiani, A.M., Sharma, S.J., Jenkins, P., Tung, S., Huynh, M.N., Chirwa, T.W., Cepeda, C., Levine, M.S., Chang, J.W., Owens, G.C., Vinters, H.V. and Mathern, G.W. (2016) Rasmussen Encephalitis Tissue Transfer Program. Epilepsia, 57, 1005-1007. [Google Scholar] [CrossRef] [PubMed]