Challenges in diagnosis and treatment of late-onset Rasmussen encephalitis: Report of three cases.
DOI:
https://doi.org/10.20453/rnp.v85i4.4370Keywords:
Rasmussen’s encephalitis, drug resistant epilepsy, histopathology, epilepsy surgeryAbstract
Rasmussen’s encephalitis is an unusual clinical entity characterized by drug-resistant focal epilepsy, neurological deficit and progressive cerebral hemiatrophy. The diagnostic and therapeutic approach is complex, even more so in patients with Rasmussen’s encephalitis of late onset (in adolescence and adulthood) whose clinical picture could be atypical when compared with its occurrence in children. We report a series of three clinical cases of late-onset Rasmussen’s encephalitis confirmed by histopathology and immunohistochemistry evaluations and in which clinical improvement was achieved after surgical interventions. Literature reviews on the clinical evolution and therapeutic alternatives for this type of presentation are included.
References
Cay-Martinez KC, Hickman RA, McKhannIi GM, Provenzano FA, Sands TT. Rasmussen Encephalitis: An Update. Seminars in neurology. 2020; 40(2): 201–210. DOI:10.1055/s-0040-1708504
Varadkar S, Bien Ch, Kruse C, Jensen F, Bauer F, Pardo C, et al. Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances. Lancet Neurol. 2014; 13: 195–205. DOI:10.1016/S1474-4422(13)70260-6
Ramesha KN, Rajesh B, Ashalatha R, Kesavadas A, Radhakrishnan VV, et al. Rasmussen’s encephalitis: Experience from a developing country based on a group of medically and surgically treated patients. Seizure. 2009; 18: 567–572. DOI:10.1016/j.seizure.2009.05.010
Bien CG, Granata T, Antozzi C, Cross JH, Dulac O, Kurthen M, et al. Pathogenesis, diagnosis, and treatment of Rasmussen encephalitis: a European consensus statement. Brain. 2005; 128(3):454–71. DOI: 10.1093/brain/awh415
Bien CG, Tiemeier H, Sassen R, Kuczaty S, Urbach H, von Lehe M, et al. Rasmussen encephalitis: incidence and course under randomized therapy with tacrolimus or intravenous immunoglobulins. Epilepsia. 2013; 54(3): 543–50. DOI: 10.1111/epi.12042
Dupont S, Gales A, Sammey S, Vidailhet M, Lambrecq V. Late-onset Rasmussen Encephalitis: A literature appraisal. Autoimmunity Reviews. 2017; 16:803-810. DOI: 10.1016/j.autrev.2017.05.022
Wang Q, Zhu Z, Wang G, Sun L, Qiu J. Functional Hemispherectomy for Adult Rasmussen Encephalitis: A Case Report and Literature Review. Turkish neurosurgery. 2019; 29(6), 945–949. DOI: 10.5137/1019-5149.JTN.21188-17.1
Dokponou YCH, Imoumby FN, Imbunhe N, El Akroud S, Gazzaz M. Rasmussen Encephalitis: Case Report and Review of the Literature. Open Access Library Journal. 2021;8: 1-7. DOI: 10.4236/oalib.1107449
Villani F, Pincherle A, Antozzi C, Chiapparini L, Granata T, Michelucci R, et al. Adult onset Rasmussen's encephalitis: anatomical-electrographic-clinical features of 7 Italian cases. Epilepsia. 2006;47(5):41–6. DOI: 10.1111/j.1528-1167.2006.00876.x
Hart YM, Andermann F, Fish DR, Dubeau F, Robitaille Y, Rasmussen T, et al. Chronic encephalitis and epilepsy in adults and adolescents: a variant of Rasmussen's syndrome? Neurology. 1997; 48:418–24. DOI: https://doi.org/10.1212/WNL.48.2.418
Jaillon-Riviere V , Dupont S, Bertran F , De la Sayette V , Beuvon V , Baulac M, et al. Le syndrome de Rasmussen à début tardif: caractéristiquescliniques et thérapeutiques. Rev Neurol. 2007; 163(5): 573-580. DOI: 10.1016/S0035-3787(07)90463-4
Olson HE, Lechpammer M, Prabhu SP, et al. Clinical application and evaluation of the Bien diagnostic criteria for Rasmussen encephalitis. Epilepsia 2013; 54: 1753–1760. DOI: 10.1111/epi.12334
Hammed A, Badour M, Baqla S, Amer F. Diagnosis and treatment of Rasmussen encephalitis pose a big challenge: Two case reports and literature review. Ann Med Surg (Lond). 2021;68:102606. DOI: 10.1016/j.amsu.2021.102606
Ghosh A, Ghosh P, Khasnobish M. Adult Onset RasmussenSyndrome Presenting with Psychosis. Indian journal of psychological medicine. 2020; 42(6): 591–593. DOI: 10.1177/0253717620954155
Fauser S, Elger CE, Woermann F, Bien CG. Rasmussen encephalitis: Predisposing factors and their potential role in unilaterality. Epilepsia. 2022; 63(1):108-119. DOI: 10.1111/epi.17131
Carmona-Vázquez CR, Peña-Landín DM, Venzor-Castellanos JP, Pasquel GVPM, Dávila-Gutiérrez G. Encefalitis de Rasmussen: Complejidad del manejo de una epilepsia potencialmente farmacorresistente ilustrada por dos casos clínicos. Rev Mex Neuroci. 2014;15(2):119-124.
Castellano JF, Meyer JA, Lado FA. A Case Series of Adult-Onset Rasmussen’s Encephalitis: Diagnostic and Therapeutic Challenges. Front. Neurol. 2017; 8: 564. DOI: 10.3389/fneur.2017.0056
Villani F, Didato G,Deleo F, Tringali G, Garbelli R, Granata T, et al. Long-term outcome after limited cortical resections in two cases of adult-onset Rasmussen encephalitis. Epilepsia. 2014; 55(5):e38–e43. DOI: 10.1111/epi.12596
Casciato S, Di Bonaventura C, Giallonardo AT, Fattouch J, Quarato PP, Mascia A, et al. Epilepsy surgery in adult-onset Rasmussen’s encephalitis: case series and review of the literatura. Neurosurg Rev. 2015;38(3):463-70. DOI: 10.1007/s10143-015-0623-5