Patients With Subacute Sclerosing Panencephalitis in Japan: A 2022 Nationwide Survey.
Journal
The Pediatric infectious disease journal
ISSN: 1532-0987
Titre abrégé: Pediatr Infect Dis J
Pays: United States
ID NLM: 8701858
Informations de publication
Date de publication:
22 Dec 2023
22 Dec 2023
Historique:
medline:
22
12
2023
pubmed:
22
12
2023
entrez:
22
12
2023
Statut:
aheadofprint
Résumé
In Japan, the incidence of subacute sclerosing panencephalitis (SSPE) has reduced; however, the medical conditions and factors associated with disease progression remain unclear. A nationwide survey of SSPE was conducted using a questionnaire in 2022. We conducted a descriptive analysis of the patients with SSPE in 2022 and Cox proportional hazards analyses for disease progression. We compared the patients with SSPE with those in a 2007 survey. A total of 37 surviving patients with SSPE were enrolled [median age: 32 years (range: 16-52 years)]. No new cases have been identified since 2017 in the survey. Jabbour stage IV was the most common stage (66.7%). The hazard ratios (95% confidence intervals) of male sex and age at the time of measles infection (years) were 2.56 (1.13-5.76) and 0.57 (0.34-0.93), respectively. Compared with those in 2007, the proportion of patients in hospitals decreased from 13.7% to 2.7%, whereas that of patients in nursing facilities increased from 17.6% to 29.7%. The proportions of patients prescribed inosine pranobex, interferon and ribavirin at the time of the survey decreased from 96.1% to 79.4%, 74.8% to 14.3% and 25.3% to 0%, respectively. The proportions of patients with gastrostomy, tracheostomy and ventilator use increased from 5.9% to 69.7%, 23.3% to 60.0% and 10.8% to 32.4%, respectively. Decreased measles cases in Japan reduced new SSPE cases. However, surviving patients in 2022 had advanced disease stages and needed medical care. Male sex and early measles infection were significantly associated with disease progression.
Sections du résumé
BACKGROUND
BACKGROUND
In Japan, the incidence of subacute sclerosing panencephalitis (SSPE) has reduced; however, the medical conditions and factors associated with disease progression remain unclear.
METHODS
METHODS
A nationwide survey of SSPE was conducted using a questionnaire in 2022. We conducted a descriptive analysis of the patients with SSPE in 2022 and Cox proportional hazards analyses for disease progression. We compared the patients with SSPE with those in a 2007 survey.
RESULTS
RESULTS
A total of 37 surviving patients with SSPE were enrolled [median age: 32 years (range: 16-52 years)]. No new cases have been identified since 2017 in the survey. Jabbour stage IV was the most common stage (66.7%). The hazard ratios (95% confidence intervals) of male sex and age at the time of measles infection (years) were 2.56 (1.13-5.76) and 0.57 (0.34-0.93), respectively. Compared with those in 2007, the proportion of patients in hospitals decreased from 13.7% to 2.7%, whereas that of patients in nursing facilities increased from 17.6% to 29.7%. The proportions of patients prescribed inosine pranobex, interferon and ribavirin at the time of the survey decreased from 96.1% to 79.4%, 74.8% to 14.3% and 25.3% to 0%, respectively. The proportions of patients with gastrostomy, tracheostomy and ventilator use increased from 5.9% to 69.7%, 23.3% to 60.0% and 10.8% to 32.4%, respectively.
CONCLUSIONS
CONCLUSIONS
Decreased measles cases in Japan reduced new SSPE cases. However, surviving patients in 2022 had advanced disease stages and needed medical care. Male sex and early measles infection were significantly associated with disease progression.
Identifiants
pubmed: 38134374
doi: 10.1097/INF.0000000000004234
pii: 00006454-990000000-00684
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.
Déclaration de conflit d'intérêts
The authors have no conflicts of interest to disclose.
Références
Jabbour JT, Garcia JH, Lemmi H, et al. Subacute sclerosing panencephalitis: a multidisciplinary study of eight cases. JAMA. 1969;207:2248–2254.
Abe Y, Hashimoto K, Iinuma K, et al. Survey of subacute sclerosing panencephalitis in Japan. J Child Neurol. 2012;27:1529–1533.
Prashanth LK, Taly AB, Ravi V, et al. Long term survival in subacute sclerosing panencephalitis: an enigma. Brain Dev. 2006;28:447–452.
Campbell C, Levin S, Humphreys P, et al. Subacute sclerosing panencephalitis: results of the Canadian Paediatric Surveillance Program and review of the literature. BMC Pediatr. 2005;5:47.
Campbell H, Andrews N, Brown KE, et al. Review of the effect of measles vaccination on the epidemiology of SSPE. Int J Epidemiol. 2007;36:1334–1348.
Miller C, Andrews N, Rush M, et al. The epidemiology of subacute sclerosing panencephalitis in England and Wales 1990-2002. Arch Dis Child. 2004;89:1145–1148.
Nihei K. [Epidemiological aspects of SSPE]. Nihon Rinsho. 2007;65:1460–1465.
Inaida S, Matsuno S, Kobune F. Measles elimination and immunisation: nationwide surveillance trends in Japan, 2008-2015. Epidemiol Infect. 2017;145:2374–2381.
National institute of infectious diseases. Cumulative measles cases. August 2023. Available at: https://www.niid.go.jp/niid/images/idsc/disease/measles/2023pdf/meas23-30.pdf. Accessed September 4, 2023.
Saha V, John TJ, Mukundan P, et al. High incidence of subacute sclerosing panencephalitis in south India. Epidemiol Infect. 1990;104:151–156.
Wendorf KA, Winter K, Zipprich J, et al. Subacute sclerosing panencephalitis: the devastating measles complication that might be more common than previously estimated. Clin Infect Dis. 2017;65:226–232.
Vijayavarman V, Malhotra HS, Rizvi I, et al. Immune-dysregulation in subacute sclerosing panencephalitis: an exploratory case-control study. J Med Virol. 2023;95:e28504.
Anlar B, Köse G, Gürer Y, et al. Changing epidemiological features of subacute sclerosing panencephalitis. Infection. 2001;29:192–195.
Bojinova V, Dimova P, Belopitova L, et al. Subacute sclerosing panencephalitis in Bulgaria (1978-2002). Neuroepidemiology. 2004;23:254–257.
Onal AE, Gurses C, Direskeneli GS, et al. Subacute sclerosing panencephalitis surveillance study in Istanbul. Brain Dev. 2006;28:183–189.
Nakamura Y, Iinuma K, Oka E, et al. [Epidemiologic features of subacute sclerosing panencephalitis from clinical data of patients receiving a public aid for treatment]. No To Hattatsu. 2003;35:316–320.
Risk WS, Risk WS 2nd. Subacute sclerosing panencephalitis with remission in a Bosnian refugee child. Pediatr Infect Dis J. 2003;22:757–758.
Hashimoto K, Hosoya M. Advances in antiviral therapy for subacute sclerosing panencephalitis. Molecules. 2021;26:427.
Lebon P, Gelot A, Zhang SY, et al. Measles sclerosing subacute panencephalitis (SSPE), an intriguing and ever-present disease: data, assumptions and new perspectives. Rev Neurol (Paris). 2021;177:1059–1068.
Sliva J, Pantzartzi CN, Votava M. Inosine pranobex: a key player in the game against a wide range of viral infections and non-infectious diseases. Adv Ther. 2019;36:1878–1905.
Huttenlocher PR, Mattson RH. Isoprinosine in subacute sclerosing panencephalitis. Neurology. 1979;29:763–771.
Jones CE, Dyken PR, Huttenlocher PR, et al. Inosiplex therapy in subacute sclerosing panencephalitis. A multicentre, non-randomised study in 98 patients. Lancet. 1982;1:1034–1037.
Gascon G, Yamani S, Crowell J, et al. Combined oral isoprinosine-intraventricular alpha-interferon therapy for subacute sclerosing panencephalitis. Brain Dev. 1993;15:346–355.
Yalaz K, Anlar B, Oktem F, et al. Intraventricular interferon and oral inosiplex in the treatment of subacute sclerosing panencephalitis. Neurology. 1992;42:488–491.
Miyazaki K, Hashimoto K, Suyama K, et al. Maintaining concentration of ribavirin in cerebrospinal fluid by a new dosage method; 3 cases of subacute sclerosing panencephalitis treated using a subcutaneous continuous infusion pump. Pediatr Infect Dis J. 2019;38:496–499.
Tomoda A, Nomura K, Shiraishi S, et al. Trial of intraventricular ribavirin therapy for subacute sclerosing panencephalitis in Japan. Brain Dev. 2003;25:514–517.