Subacute sclerosing panencephalitis: clinical phenotype, epidemiology, and preventive interventions

Abstract

Subacute sclerosing panencephalitis (SSPE) is a preventable condition reported in 6.5 to 11 per 100 000 cases of measles, and highest in children who contracted measles infection when they were less than 5 years of age. Children residing in areas with poor vaccination coverage and high prevalence of human immunodeficiency virus are at increased risk of developing SSPE. SSPE is life‐threatening in most affected children. This report documents current data relating to the clinical phenotype, epidemiology, and understanding of SSPE, inclusive of preventive interventions. While improvements in disease progression with immunomodulation may occur, overall there is no cure. Most therapies focus on supportive needs. Seizures and abnormal movements may respond to carbamazepine. Many countries advocate policies to enhance vaccination coverage. Effective preventive health care programmes, assurance of parental perceptions, and crisis support for unprecedented events obstructing effective primary health care are needed. Until measles is eradicated worldwide, children in all regions remain at risk. What this paper adds Measles contracted under 5 years of age has highest risk of developing subacute sclerosing panencephalitis (SSPE). Children with, or exposed to, human immunodeficiency virus infection, who contract measles may be at increased risk of SSPE.

Neurodevelopmental findings in children 20‐30 months of age with postnatal Zika infection at 1‐12 months of age, Colombia, September‐November 2017

“…A study in infant rhesus macaques found associations between postnatal ZIKV infection and neurotropism and alterations of the central nervous system 7 . There are also examples of other viral infections in early childhood, such as varicella and measles, having associations with adverse neurodevelopmental outcomes 13,14 . In this investigation, there were insufficient data to determine whether postnatal ZIKV infection was associated with EAD‐1 alert scores.…”

Measles Encephalitis: Towards New Therapeutics

“…Subacute sclerosing panencephalitis (SSPE) cases occur in 6.5 to 11 cases per 100,000 [97,98] in immunocompetent patients that contracted measles in their childhood, with a mortality rate close to 100%. Within children infected by MeV before the age of 12 months, the incidence for SSPE rises to 1/609, while reaching 1/1367 for children under five years old [99].…”

“…Isoprinosine is a derivative of inosine and aims at blocking viral replication, probably through an immunoregulatory activity. Again, these treatments have rarely been shown to recover loss of function but they can stabilize the disease for several years [98,213,217]. Despite the benefits of IFN-α treatments, its use can be associated with side effects and could lead to interferonopathies [218].…”

Inosine Pranobex: A Key Player in the Game Against a Wide Range of Viral Infections and Non-Infectious Diseases

“…The prevalence of SSPE is inversely connected to measles vaccination and dependent on the patient’s age. The estimated incidence of SSPE ranges from 4 to 11 cases per 100,000 cases of measles and involves mostly children younger than 5 years old [51, 52]. There is an average of 4–10 years between the measles infection and the onset of the disorder, and prevalence is higher in males [53].…”