Subacute sclerosing panencephalitis (SSPE) in Papua New Guinea: a high incidence in young children

Lucas, Kurt; Sanders, R C; Rongap, A; Rongap, T.; Pinai, S.; Alpers, Michael P.

doi:10.1017/s0950268800050044

Cited by 13 publications

(14 citation statements)

References 22 publications

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“…The population below 20 years of age for the provinces of origin of the SSPE cases in 1990 (50% of the total population) was 841,326. In the report, the annual incidence of SSPE in the study provinces was estimated to be 56 per million population below the age of 20 years in 1990 [Lucas et al, 1992]. More recently basing on progressive neurological disorder with positive measles antibody in cerebrospinal fluid and the presence of myoclonic jerks, we have estimated a higher annual incidence of SSPE in EHP between 1997 and 1998 [Takasu et al, manuscript submitted].…”

Section: Subacute Sclerosing Panencephalitis (Sspe) Is a Rare Late Comentioning

confidence: 99%

Molecular analysis of measles virus genome derived from SSPE and acute measles patients in Papua, New Guinea

Miki

Komase

Mgone

et al. 2002

Journal of Medical Virology

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A very high annual incidence of 56 per million population below the age of 20 years for subacute sclerosing panencephalitis (SSPE) has been reported from Papua New Guinea (PNG). In a more recent study, we have confirmed this unusual high incidence for Eastern Highlands Province (EHP) of PNG. In the study, it was observed that the vaccination rate among SSPE patients registered at Goroka Base General Hospital (GBGH) in EHP was higher than that of other infants in the province in recent years. To identify the measles virus (MV) responsible for SSPE in EHP, sequence analysis of hypervariable region of the N gene was performed from 13 MV genomes: 2 amplified from clinical specimens of SSPE patients and 11 from acute measles patients. In 2 cases among the 11 with acute measles, nucleotide sequence of the entire H gene derived from isolated viruses was determined. Both nucleotide sequence and phylogenetic tree analyses showed that the amplified MV cDNAs were closely related to one another and belonged to the D3 genotype though they were different from any previously reported MV sequences. No genome sequences of vaccine strains were detected. These findings suggest that the MV strains prevailing in the highlands of PNG belong to genotype D3 of the MV and this wild-type MV rather than the vaccine strains was likely to be responsible for SSPE in these patients.

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Section: Subacute Sclerosing Panencephalitis (Sspe) Is a Rare Late Comentioning

confidence: 99%

Molecular analysis of measles virus genome derived from SSPE and acute measles patients in Papua, New Guinea

Miki

Komase

Mgone

et al. 2002

Journal of Medical Virology

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“…Guler and colleagues, in 2015, presented data 64 patients that were seen between 2007 and 2013 . In Papua New Guinea, between 1988 and 1991, 87 confirmed cases were reported . In 2007 to 2009, the incidence declined but still reported 22 cases in Madang province of Papua New Guinea with annual incidence of 54/million population aged <20 years.…”

Section: Epidemiologymentioning

confidence: 99%

“…4 In Papua New Guinea, between 1988 and 1991, 87 confirmed cases were reported. 5 In 2007 to 2009, the incidence declined but still reported 22 cases in Madang province of Papua New Guinea with annual incidence of 54/million population aged <20 years. In four subdistricts of Madang province, the yearly incidence reached >100 per million population aged less than 20 years.…”

Section: Epidemiologymentioning

confidence: 99%

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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“…1 The annual incidence of SSPE in the Eastern Highlands Province (EHP) of PNG in 1997e1998 was 98 per million of population under 20 years of age, the highest ever reported. 2 This incidence was reported to range from 0.1 to 6 cases per million of population in other places.…”

Section: Introductionmentioning

confidence: 99%

Cerebrospinal fluid levels of matrix metalloproteinase-9 and tissue inhibitor of metalloproteinase-1 in subacute sclerosing panencephalitis

Ichiyama

Matsushige

Siba

et al. 2008

Journal of Infection

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Subacute sclerosing panencephalitis (SSPE) in Papua New Guinea: a high incidence in young children

Cited by 13 publications

References 22 publications

Molecular analysis of measles virus genome derived from SSPE and acute measles patients in Papua, New Guinea

Molecular analysis of measles virus genome derived from SSPE and acute measles patients in Papua, New Guinea

Subacute sclerosing panencephalitis

Cerebrospinal fluid levels of matrix metalloproteinase-9 and tissue inhibitor of metalloproteinase-1 in subacute sclerosing panencephalitis

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