CLINICAL AND MORPHOLOGICAL ANALYSIS OF A FATAL CASE OF PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY

Authors:

Abstract:

Progressive multifocal leukoencephalopathy is a rare severe progressive demyelinating disease of the central nervous system that occurs as a result of reactivation of polyomavirus in immunodeficiency states. Currently, progressive multifocal leukoencephalopathy is an incurable disease with unfavorable outcomes. The article analyzes a case of progressive multifocal leukoencephalopathy based on the medical history and pathological examination of the deceased. In this case, the patient had no signs of immunosuppression and the diagnosis was confirmed histologically. Some publications indicate that progressive multifocal leukoencephalopathy without overt immunosuppression may account for a significant proportion of cases of this disease, and the frequency of progressive multifocal leukoencephalopathy without overt immunosuppression may potentially be even higher. It can be assumed that a period of dysfunction in the immune surveillance of the viral life cycle may be sufficient to allow the necessary viremia to occur in immunocompetent patients. Although progressive multifocal leukoencephalopathy is rare, doctors should always keep in mind the possibility of developing this disease with a similar clinical picture in patients without signs of immunosuppression.

Keywords:

pathomorphology JC-virus diagnosis etiology immunodeficiency

References:

1. Baldassari LE, Wattjes MP, Cortese IC, Gass A, Metz I, Yousry T, et al. The neuroradiology of progressive multifocal leukoencephalopathy: a clinical trial perspective. Brain.2022;145(2):426–440. doi: 10.1093/brain/awab419.
2. Cortese I, Reich DS, Nath A. Progressive multifocal leukoencephalopathy and the spectrum of JC virus-related disease. Nat Rev Neurol. 2021:17;37–51. doi: 10.1038/s41582-020-00427-y.
3. Fіlenko BM, Roiko NV, Starchenko II, Proskurnya SA, Nikolenko DY. Clinical and morphological analysis of pulmonary aspergillosis coinfection in COVID-19. Azerbaijan Medical Journal. 2022;2:145–150. doi: 10.34921/amj.2022.2.023.
4. Fukumoto T, Sakashita Y, Katada F, Takeuchi R, Miyamoto R, Izumi Y, et al. “Burnt-out” progressive multifocal leukoencephalopathy in idiopathic CD4(+) lymphocytopenia. Neuropathology. 2021;41:484–8. doi: 10.1111/neup.12773.
5. Hirsch HH, Babel N, Comoli P, Friman V, Ginevri F, Jardine A, et. al. European perspective on human polyomavirus infection, replication and disease in solid organ transplantation. Clin. Microbiol. Infect. 2014;20:74–88. doi.org/10.1111/1469-0691.12538.
6. Jain V, Branstetter H, Savaram S, Vasquez M, Swords G, Aghili-Mehrizi S, et al. Progressive multifocal leukoencephalopathy without overt immunosuppression. Medicine. 2023;102(39):e35265.
7. Joly M, Conte C, Cazanave C, Le Moing V, Tattevin P, Delobel P, et al. Progressive multifocal leukoencephalopathy: epidemiology and spectrum of predisposing conditions. Brain. 2023:146(1):349–358. doi: 10.1093/brain/awac237.
8. Kartau M, Verkkoniemi-Ahola A, Paetau A, Palomäki M, Janes R, Ristola M, et al. The incidence and predisposing factors of John Cunningham virus-induced progressive multifocal leukoencephalopathy in Southern Finland: a population-based study. Open Forum Infect Dis. 2019;6:ofz024. doi: 10.1093/ofid/ofz024.
9. Nakamichi K, Miura Y, Shimokawa T, Takahashi K, Suzuki T, Funata N, et al. Nationwide laboratory surveillance of progressive multifocal leukoencephalopathy in Japan: Fiscal Years 2011–2020. Viruses. 2023;15:968. doi: 10.3390/v15040968.
10. Pavlovic D, Patera AC, Nyberg F, Gerber M, Liu M. Progressive multifocal leukoencephalopathy: current treatment options and future perspectives. Therapeutic Advances in Neurological Disorders. 2015;8(6):255–273. doi: 10.1177/1756285615602832.
11. Pietropaolo V, Prezioso C, Bagnato F, Antonelli G. John Cunningham virus: an overview on biology and disease of the etiological agent of the progressive multifocal leukoencephalopathy. New Microbiol. 2018;41:179–186.
12. Rybalka YaV, Yeroshenko GA, Shevchenko KV, Ryabushko OB, Ulanovska-Tsyba NА, Grygorenko AS, Sokolenko VM. Current views on the structural organization of the rat cerebral cortex. The world of medicine and biology. 2024;2(88):237–242. doi: 10.26724/2079-8334-2024-2-88-237-242.
13. Sriwastava S, Khan E, Khalid SH, Kaur A, Feizi P. Progressive multifocal leukoencephalopathy in an immunocompetent patient: a case report and review of literature. Journal of Medical Virology. 2022;94(6):2860–2869. doi: 10.1002/jmv.27493.
14. Wijburg MT, Warnke C, Barkhof F, Uitdehaag BM, Killestein J, Wattjes MP. Performance of PML diagnostic criteria in natalizumab-associated PML: data from the Dutch-Belgian cohort. Journal of Neurology, Neurosurgery & Psychiatry. 2019;90(1):44–46. doi: 10.1136/jnnp-2018-318261.
15. Wollebo HS, White MK, Gordon J, Berger JR, https://onlinelibrary.wiley. Persistence and pathogenesis of the neurotropic polyomavirus JC. Ann Neurol. 2015;77(4):560–570. doi: 10.1002/ana.24371.

Publication:

«World of Medicine and Biology» Vol. 20 No. 89 (2024) , с. 263-266
УДК 616.832-07-092

DOI:

https://doi.org/10.26724/2079-8334-2024-3-89-263-266