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

Abstract

-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,

Atypical subacute sclerosing panencephalitis: case report

“…In a series of 48 patients, Nunes et al 4 reported seven cases (14%) of SSPE in previously immunized patients. In the Canadian Paediatric Surveillance Pro-gram, Campbell et al 7 diagnosed four cases of SSPE, of whom one was previously immunized (25%) and presented with an atypical EEG pattern.…”

“…The incidence is higher in males with a ratio of 2-4:1 female 3 . In Brazil, there are few reports available, except for the study of Nunes et al 4 , in the end of the nineties, showing endemic regions in the country and a delay between fi rst symptoms and diagnosis. In India, the incidence rate is very high (21 per million population) and linked to the high frequency of cases of measles among the very young 5 .…”

Epidemiological Findings and Clinical and Magnetic Resonance Presentations in Subacute Sclerosing Panencephalitis

“…Bojinova et al 21 reported that 35% of 40 SSPE patients presented with mental deterioration, 29% had extrapyramidal hyperkinesis, 15% had epileptic seizure, 10% had hemiparesis and 10% had vision impairment. Nunes et al 22 reported that the first symptom was myoclonus or tonic-clonic seizures in 22 of 48 SSPE patients and behaviour alterations in seven patients. Öztürk et al 17 reported that 50% of their patients presented with behaviour alteration, 30% with atonic/myoclonic seizures and a few with vision impairments and generalized tonicclonic seizure.…”

Pediatric Subacute Sclerosing Panencephalitis: A Narrative Review on Measles and the Future of Vaccination

“…There is considerable literature reporting cases of subacute sclerosing panencephalitis in pregnancy, and these have all been confirmed by the usual diagnostic methods. 6,7,[38][39][40] The course appears to be fulminant, for which it is speculated that immunologic and hormonal consequences of pregnancy may be responsible. The fetal outcome is often unfavorable because of the obstetric consequences arising from the mother's condition, for example, elective premature delivery by caesarean section.…”