Subacute Sclerosing Panencephalitis: The Devastating Measles Complication That Might Be More Common Than Previously Estimated

Wendorf, Kristen; Winter, Kathleen; Zipprich, Jennifer; Schechter, Rob; Hacker, Jill K.; Preas, Chris; Cherry, James D.; Glaser, Carol; Harriman, Kathleen

doi:10.1093/cid/cix302

Cited by 96 publications

(115 citation statements)

References 15 publications

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“…Wendorf and coworkers, in an epidemiological study (1998 to 2015), in the United States of America (USA), reported 17 patients with SSPE. The SSPE incidence if measles infection occurred before 5 years of age was 1:1367, and if measles occurred in infancy, it was 1:609 . In the prevaccination era, even developed countries witnessed an exceptionally high incidence of SSPE.…”

Section: Epidemiologymentioning

confidence: 99%

“…In the prevaccination era, even developed countries witnessed an exceptionally high incidence of SSPE. In the USA, from 1988 to 1991, 3651 cases of measles, in children of <12 months of age, were recorded . A study published in 1982 (in the prevaccination era) reported 634 (onset from 1956 to 1981) cases of suspected SSPE patients.…”

Section: Epidemiologymentioning

confidence: 99%

“…The characteristic age of SSPE diagnosis is around 12 years (3‐35 y). The median latency period, defined as onset of measles to onset of neurological symptoms, is 9.5 years (2.5 to 34 y) . Following improved measles control, higher age of onset is now noted .…”

Section: Epidemiologymentioning

confidence: 99%

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Subacute sclerosing panencephalitis

Garg

Mahadevan

Malhotra

et al. 2019

Reviews in Medical Virology

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Summary Subacute sclerosing panencephalitis (SSPE) is a slowly progressive brain disorder caused by mutant measles virus. SSPE affects younger age groups. SSPE incidence is proportional to that of measles. High‐income countries have seen substantial decline in SSPE incidence following universal vaccination against measles. SSPE virus differs from wild measles virus. Measles virus genome recovered from the autopsied brain tissues demonstrates clustered mutations in virus genome particularly in the M gene. These mutations destroy the structure and functioning of the encoded proteins. Complete infectious virus particle has rarely been recovered from the brain. Human neurons lack required receptor for entry of measles virus inside the neurons. Recent in vitro studies suggest that mutations in F protein confer hyperfusogenic properties to measles virus facilitating transneuronal viral spread. The inflammatory response in the brain leads to extensive tissue damage. Clinically, SSPE is characterized by florid panencephalitis. Clinically, SSPE is characterized by cognitive decline, periodic myoclonus, gait abnormalities, vision loss, and ultimately to a vegetative state. Chorioretinitis is a common ocular abnormality. Electroencephalography (EEG) shows characteristic periodic discharges. Neuroimaging demonstrates periventricular white matter signal abnormalities. In advanced stages, there is marked cerebral atrophy. Definitive diagnosis requires demonstration of elevated measles antibody titers in cerebrospinal fluid (CSF). Many drugs have been used to stabilize the course of the disease but without evidence from randomized clinical trials. Six percent of patients may experience prolonged spontaneous remission. Fusion inhibitor peptide may, in the future, be exploited to treat SSPE. A universal vaccination against measles is the only proven way to tackle this menace currently.

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Section: Epidemiologymentioning

confidence: 99%

Section: Epidemiologymentioning

confidence: 99%

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Subacute sclerosing panencephalitis

Garg

Mahadevan

Malhotra

et al. 2019

Reviews in Medical Virology

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“…In regions where measles epidemics occur, cases of SSPE are more prevalent in the following 4 to 10 years (latency period). 5 Table I shows the incidence data of SSPE in various countries. 3 A regional population study documented for children from Germany who were younger than 5 years of age when they contracted measles to have a 1 in 1700 to 1 in 3300 risk of subsequently developing SSPE; for those who contracted measles younger than 3 years of age, the risk was 1.7fold higher.…”

Section: Epidemiologymentioning

confidence: 99%

“…4,5 Risk factors that make young children more vulnerable to contracting measles and putting them at risk of developing SSPE include poor socio-economic status, low level of parental education, failure to receive the measles vaccination, a higher number of siblings, and a higher birth order (i.e. 4,5 Risk factors that make young children more vulnerable to contracting measles and putting them at risk of developing SSPE include poor socio-economic status, low level of parental education, failure to receive the measles vaccination, a higher number of siblings, and a higher birth order (i.e.…”

Section: Risk Factorsmentioning

confidence: 99%

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

Mekki

Eley

Hardie

et al. 2019

Develop Med Child Neuro

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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.

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Clinical Practices for Measles-Mumps-Rubella Vaccination Among US Pediatric International Travelers

et al. 2020

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IMPORTANCEThe US population is experiencing a resurgence of measles, with more than 1000 cases during the first 6 months of 2019. Imported measles cases among returning international travelers are the source of most US measles outbreaks, and these importations can be reduced with pretravel measles-mumps-rubella (MMR) vaccination of pediatric travelers. Although it is estimated that children account for less than 10% of US international travelers, pediatric travelers account for 47% of all known measles importations.OBJECTIVE To examine clinical practice regarding MMR vaccination of pediatric international travelers and to identify reasons for nonvaccination of pediatric travelers identified as MMR eligible. DESIGN, SETTING, AND PARTICIPANTSThis cross-sectional study of pediatric travelers (ages Ն6 months and <18 years) attending pretravel consultation at 29 sites associated with Global TravEpiNet (GTEN), a Centers for Disease Control and Prevention-supported consortium of clinical sites that provide pretravel consultations, was performed from January 1, 2009, through December 31, 2018. MAIN OUTCOMES AND MEASURES Measles-mumps-rubella vaccination among MMR vaccination-eligible pediatric travelers.RESULTS Of 14 602 pretravel consultations for pediatric international travelers, 2864 travelers (19.6%; 1475 [51.5%] males; 1389 [48.5%] females) were eligible to receive pretravel MMR vaccination at the time of the consultation: 365 of 398 infants aged 6 to 12 months (91.7%), 2161 of 3623 preschool-aged travelers aged 1 to 6 years (59.6%), and 338 of 10 581 school-aged travelers aged 6 to 18 years (3.2%). Of 2864 total MMR vaccination-eligible travelers, 1182 (41.3%) received the MMR vaccine and 1682 (58.7%) did not. The MMR vaccination-eligible travelers who did not receive vaccine included 161 of 365 infants (44.1%), 1222 of 2161 preschool-aged travelers (56.5%), and 299 of 338 school-aged travelers (88.5%). We observed a diversity of clinical practice at different GTEN sites. In multivariable analysis, MMR vaccination-eligible pediatric travelers were less likely to be vaccinated at the pretravel consultation if they were school-aged

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Subacute Sclerosing Panencephalitis: The Devastating Measles Complication That Might Be More Common Than Previously Estimated

Cited by 96 publications

References 15 publications

Subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis

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

Clinical Practices for Measles-Mumps-Rubella Vaccination Among US Pediatric International Travelers

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