Incidence of Subacute Sclerosing Panencephalitis Following Measles and Measles Vaccination in Japan

-Subacute sclerosing panencephalitis (SSPE) is an inflammatory neurodegenerative disease related to the persistence of measles virus. Although its frequency is declining because of measles eradication, we still have some cases being diagnosed. With the aim to describe epidemiological aspects of SSPE in Brazil, we sent a protocol to Child Neurologists around the country, 48 patients were registered, 27 (56 %) were from the southeast region, 34 (71%) were male and 35 (73%) white, 27 (56%) had measles, 9 (19%) had measles and were also immunized, 7 (14%) received only immunization, 1 patient had a probable neonatal form. Mean time between first symptoms and diagnosis was 12 months (22 started with myoclonus or tonic-clonic seizures, 7 (14%) with behavioral disturbances); 36 patients (75%) had EEG with pseudoperiodic complexes. Follow up performed in 28 (58 %) patients showed: 12 died, 2 had complete remission and the others had variable neurological disability Our data shows endemic regions in the country, a high incidence of post-immunization SSPE and a delay between first symptom and diagnosis.KEY WORDS: immunization, measles, seizures, subacute sclerosing panencephalitis Panencefalite esclerosante subaguda, aspectos clínicos e prognóstico: Registro Brasileiro RESUMO -A panencefalite esclerosante subaguda é doença neurodegenerativa inflamatória relacionada à persistência do vírus do sarampo no organismo. Sua incidência vem diminuindo significativamente com a erradicação do sarampo, mas eventualmente alguns casos ainda têm sido diagnosticados. Com o objetivo de descrever aspectos epidemiológicos da panencefalite no Brasil contactamos Neurologistas Infantis de todo país. Foram registrados 48 pacientes, 27% da região sudeste, 34 (70%) do sexo masculino, 35 (73%) brancos, 9 (19%) apresentaram sarampo e receberam imunização, 7 (14%) somente imunizados, um paciente apresentou provável forma neonatal. Intervalo médio entre primeiro sintoma e diagnóstico de 12 meses, 22 pacientes (45%) iniciaram o quadro com mioclonus ou convulsões tônico-clônicas, 7 (14%) com distúrbios comportamentais; 36 (75%) apresentaram EEG com complexos pseudoperiódicos. Seguimento de 28 pacientes (57%) demonstrou 12 óbitos, 2 remissões completas, demais com sequelas neurológicas. Nossos resultados evidenciam regiões endêmicas no país, alta incidência de casos pós-imunização e demora na confirmação do diagnóstico. PALAVRAS-CHAVES: imunização, sarampo, convulsões, panencefalite esclerosante subaguda.Subacute sclerosing panencephalitis (SSPE) is an inflammatory neurodegenerative disease related to the persistence of an aborted form of the wild measles virus. Clinical presentation of the disease is more fully documented at this time but the major etiological and pathological bases of SSPE remain only partially understood 1,2 . The speed of progression has been designated as acute (development of at least 66% neurologic disability within 3 months of the first neurologic symptoms,

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“…Mean age of measles infection was not as low in the majority of patients as related in previous studies 9,11 .…”

Section: Discussionsupporting

confidence: 42%

Subacute sclerosing panencephalitis: clinical aspects and prognosis. The Brazilian Registry

Nunes

Costa

Stancher

et al. 1999

Arq. Neuro-Psiquiatr.

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“…The mainstay for SSPE prophylaxis is the compulsory measles immunization. The SSPE incidence varies from 16.1/10 6 (from 6.1 to 40.9/10 6 ) for the years with measles epidemics in Japan [14] to 20/10 6 and even 100/10 6 in undeveloped countries endemic for measles [2,3]. Following the introduction of measles vaccine in different countries in the last 20 years, a tendency towards reduction in SSPE incidence has been observed [2,4,10,14]: in Japan [14] the incidence has fallen from 16/10 6 to 0-1/10 6 , in Poland from 1.6/10 6 (1983) to 0.69/10 6 (1991) and 0.42/10 6 (1995) [7][8][9], and in Romania from 5.21 /10 6 (1987) to 1.8/10 6 (1988) [13].…”

Section: Discussionmentioning

confidence: 99%

“…Among the non-immunized children the mean age of occurrence of measles in the period 1979-1984 (n = 27) Bojinova/Dimova/Belopitova/Mihailov/ Gatcheva/Mihneva/Todorova The measles infection in 27 out of 28 children with SSPE during the period 1979-1984 was due to the lack of vaccination of these children being older than 1 year at the start of the regular immunizations in the country in 1969; 11 of the 12 SSPE patients in the period 1995-2002 were not immunized, but only 3 were exempted from immunization by medical indications, the remainder (8 children) developed a measles infection between the age of 6 months and 1.6 years during the epidemic (1991-1992). The mean period from the measles infection to disease onset was 7.0 years (range [4][5][6][7][8][9][10][11][12][13][14]. Two children immunized against measles developed SSPE at 5 years of age, 1 following a severe form of varicella.…”

Section: Incidencementioning

confidence: 99%

“…The SSPE incidence varied from 1/10 6 in the USA to 20-100/10 6 in developing countries having recurrent measles epidemics [2,3]. The latent period between the primary measles infection and clinical manifestation of SSPE is 1/16 years (average 8 years) [14]; SSPE cases may develop from 3 months [15] to 2 years following measles infection [16], with rare SSPE cases in adults. Epidemiological studies have demonstrated that early natural measles infection is a risk factor for the development of SSPE.…”

Section: Introductionmentioning

confidence: 99%

“…Epidemiological studies have demonstrated that early natural measles infection is a risk factor for the development of SSPE. According to Okuno et al [14], 72% of SSPE patients have had measles before 2 years of age. Miller et al [2] established that over half of SSPE patients had measles under 2 years of age and 75% of them under 4 years of age.…”

Section: Introductionmentioning

confidence: 99%

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Subacute Sclerosing Panencephalitis in Bulgaria (1978–2002)

et al. 2004

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The epidemiology of subacute sclerosing panencephalitis (SSPE) has changed substantially since the introduction of measles vaccine. We studied the incidence of SSPE in Bulgaria based on cases admitted to the Child Neurology Clinic, University Hospital of Neurology and Psychiatry, Sofia, for a 25-year period (1978–2002). The SSPE incidence prior to and during the period of routine measles immunization was analyzed. SSPE was diagnosed in 40 children (29 males and 11 females, mean age 8.5 years), 28 from 1978 to 1984 (average 4 patients/year), and 12 from 1995 to 2002 (average 1.7 patients/year). Thirty-eight cases (95%) were non-immunized and had early measles infection. Age at onset of SSPE ranged from 8 to 11 years (52.5%) with a mean latent period of 7 years following measles infection. The increase in SSPE incidence (1995–2002) following a 10-year disease-free period (1985–1994) appears to be related to early measles infection (mean age 11 months) during the measles epidemic of 1991–1992. During the period 1995–2002, children had earlier measles infection (average 11 months) and earlier onset of SSPE (mean age 8.4 years) than in the period 1978–1984 (mean age at measles infection 18 months, and of SSPE onset 11.2 years). The SSPE incidence in Bulgaria during the 25-year period from 1978 to 2002 confirms the importance of early measles infection as a risk factor for SSPE, and the role of routine measles immunization in SSPE prevention.

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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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Incidence of Subacute Sclerosing Panencephalitis Following Measles and Measles Vaccination in Japan

Cited by 43 publications

References 0 publications

Subacute sclerosing panencephalitis: clinical aspects and prognosis. The Brazilian Registry

Subacute sclerosing panencephalitis: clinical aspects and prognosis. The Brazilian Registry

Subacute Sclerosing Panencephalitis in Bulgaria (1978–2002)

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

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