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

Arquivos de Neuro-Psiquiatria

Magda Lahorgue Nunes²  Jaderson Costa Da Costa²  Vanessa M. Stancher¹  Aron Diament¹  Fernando Arita¹  SÉrgio Rosemberg¹  Paul Dyken¹ 

[1] ,PUCRS Hospital São Lucas Serviço de NeurologiaPorto Alegre RS

关键词: immunization;    measles;    seizures;    subacute sclerosing panencephalitis;    imunização;    sarampo;    convulsões;    panencefalite esclerosante subaguda;   

DOI  :  10.1590/S0004-282X1999000200002

来源: SciELO

【 摘 要 】

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.

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