Subacute sclerosing panencephalitis: current threat

   The relevance of this problem is associated with a predicted increased incidence of subacute sclerosing panencephalitis, a steadily progressive and difficult to diagnose fatal brain disease. Subacute sclerosing panencephalitis develops after measles and it does not correlate with clinical type of an acute measles infection: it affects both persons with manifest measles and ones who have had an asymptomatic or abortive disease. WHO estimates the incidence of subacute sclerosing panencephalitis as 4–11 per 100,000 measles cases. The latency period from measles to the onset of subacute sclerosing panencephalitis is between 2.5 and 34 years. As a result of measles outbreaks in 2011–2014 and 2017–2019 an increase in the number of subacute sclerosing panencephalitis cases in Russia is expected soon. Diagnostic difficulties are caused by multiplicity of subacute sclerosing panencephalitis clinical manifestation and the absence of MRI changes in early stages. Thereby, it is important to exclude SSPE in patients with neurological symptoms. Screening tests for subacute sclerosing panencephalitis should be conducted in children with acute cognitive impairment, myoclonus, or a new onset of epileptic syndrome. The specific cure for subacute sclerosing panencephalitis has not been developed yet. Most treatments aim at reducing symptoms. Up to date, the only way to conquer this disease is routine measles vaccination.

1. Brechot C., Bryant J., Endtz H., Garry R.F., Griffin D.E., Lewin S.R. et al. 2018 international meeting of the Global Virus Network. Antiviral Res. 2019; 163: 140–148. DOI: 10.1016/J.ANTIVIRAL.2019.01.013

2. WHO EpiBrief: a report on the epidemiology of selected vaccine-preventable diseases in the European Region: No. 1/2020 https://apps.who.int/iris/handle/10665/360804 The link is active on 26. 06. 2023.

3. Campbell H., Lopez Bernal J., Bukasa A., Andrews N., Baker E., Maunder P. et al. A Reemergence of Subacute Sclerosing Panencephalitis in the United Kingdom. The Pediatric infectious disease journal 2023; 42(1): 82–84. DOI: 10.1097/INF.0000000000003744

4. Garg R.K., Mahadevan A., Malhotra H.S., Rizvi I., Kumar N., Uniyal R. Subacute sclerosing panencephalitis. Rev Med Virol. 2019: 29(5): e2058. DOI: 10.1002/RMV.2058

5. Gutierrez J., Issacson R.S., Koppel B.S. Subacute sclerosing panencephalitis: an update. Dev Med Child Neurol. 2010; 52(10): 901–907. DOI: 10.1111/j.1469–8749.2010.03717.x

6. Moss W.J. Measles. Lancet. 2017; 390(10111): 2490–2502. DOI: 10.1016/S0140–6736(17)31463–0

7. Rocke Z., Belyayeva M. Subacute Sclerosing Panencephalitis. Treasure Island (FL); StatPearls Publishing; 2022. https://www.ncbi.nlm.nih.gov/books/NBK560673 / Ссылка активна на 26. 06. 2023.

8. Mekki M., Eley B., Hardie D., Wilmshurst J.M. Subacute sclerosing panencephalitis: clinical phenotype, epidemiology, and preventive interventions. Dev Med Child Neurol. 2019; 61(10): 1139–1144. DOI: 10.1111/dmcn.14166

9. Wendorf K.A., Winter K., Zipprich J., Schechter R., Hacker J.K., Preas C. et al. Subacute Sclerosing Panencephalitis: The Devastating Measles Complication That Might Be More Common Than Previously Estimated. Clin Infect Dis. 2017; 65(2): 226–232. DOI: 10.1093/cid/cix302

10. Miller C., Farrington C.P., Harbert K. The epidemiology of subacute sclerosing panencephalitis in England and Wales 1970–1989. Int J Epidemiol. 1992; 21(5): 998–1006. DOI: 10.1093/ije/21.5.998

11. Tsvirkun O.V., Gerasimova A.G., Tikhonova N.T., Ezhlova E.B., Melnikova A.A., Dubovitskaya E.L. et al. Measles Cases by Age Group during the Elimination of Infection. Epidemiologiya i Vakcinoprofilaktika. 2017; 16(3): 18–25. (in Russ.) DOI: 10.31631/2073–3046–2017–16–3–18–25

12. Campbell H., Andrews N., Brown K.E., Miller E. Review of the effect of measles vaccination on the epidemiology of SSPE. Int J Epidemiol. 2007; 36(6): 1334–1348. DOI: 10.1093/ije/dym207

13. Lebon P., Gelot A., Zhang S.Y., Casanova J.L., Hauw J.J. Measles Sclerosing Subacute PanEncephalitis (SSPE), an intriguing and ever-present disease: Data, assumptions and new perspectives Rev Neurol (Paris). 2021; 177(9): 1059–1068. DOI: 10.1016/j.neurol.2021.02.387

14. Sato Y., Watanabe S., Fukuda Y., Hashiguchi T., Yanagi Y., Ohno S. Cell-to-Cell Measles Virus Spread between Human Neurons Is Dependent on Hemagglutinin and Hyperfusogenic Fusion Protein. J Virol. 2018; 92(6): e02166–17. DOI: 10.1128/JVI.02166–17

15. Angius F., Smuts H., Rybkina K., Stelitano D., Eley B., Wilmshurst J. et al. Analysis of a Subacute Sclerosing Panencephalitis Genotype B3 Virus from the 2009–2010 South African Measles Epidemic Shows That Hyperfusogenic F Proteins Contribute to Measles Virus Infection in the Brain. J Virol. 2019; 93(4): e01700–18. DOI: 10.1128/JVI.01700–18

16. Griffin D.E. Measles virus persistence and its consequences. Curr Opin Virol. 2020; 41: 46–51. DOI: 10.1016/j.coviro.2020.03.003

17. Papetti L., Amodeo M.E., Sabatini L., Baggieri M., Capuano A., Graziola F. et al. Subacute Sclerosing Panencephalitis in Children: The Archetype of Non-Vaccination. Viruses. 2022; 14(4): 733. DOI: 10.3390/v14040733

18. Arora S., Jolly A.J., Suhas S., Arasappa R., Kamble N., Pal P.K. et al. Subacute Sclerosing Panencephalitis Masquerading as Schizophrenia: An Example of Diagnostic Overshadowing in Neuropsychiatry. Prim Care Companion CNS Disord. 2022; 24(3): 21cr02997. DOI: 10.4088/PCC.21cr02997

19. Garg M., Arora A., Kulkarni S.D., Hegde A.U., Shah K.N. Subacute Sclerosing Panencephalitis (SSPE): Experience from a Tertiary-Care Pediatric Center. J Neurosci Rural Pract. 2022; 13(2): 315–320. DOI: 10.1055/s-0041–1740612

20. Reddy R.B., Joshi D., Kumar A. Subacute Sclerosing Panencephalitis with An Atypical Presentation. Ann Indian Acad Neurol. 2021; 24(6): 946–947. DOI: 10.4103/aian.AIAN_791_20

21. Gascon G.G. International Consortium on Subacute Sclerosing Panencephalitis. Randomized treatment study of inosiplex versus combined inosiplex and intraventricular interferon-alpha in subacute sclerosing panencephalitis (SSPE): international multicenter study. J Child Neurol. 2003; 18(12): 819–827. DOI: 10.1177/088307380301801201

22. Oncel I., Sancar M., Konuskan B. Aprepitant in the Treatment of Subacute Sclerosing Panencephalitis: A Randomized, Double-Blind, Placebo-Controlled Study. Pediatr Neurol. 2020; 110: 59–63. DOI: 10.1016/j.pediatrneurol.2020.05.009

23. Dubé È., Ward J.K., Verger P., MacDonald N.E. Vaccine Hesitancy, Acceptance, and Anti-Vaccination: Trends and Future Prospects for Public Health. Annu Rev Public Health. 2021; 42: 175–191. DOI: 10.1146/annurev-publhealth-090419–102240