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Subacute sclerosing panencephalitis. Clinical aspects and prognosis. The Brazilian registry
M L Nunes1, J C Da Costa, V M Stancher
1Serviço de Neurologia, Hospital São Lucas da PUCRS, Porto Alegre RS, Brasil. magdalahorgue@conex.com.br
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.
Insights
Subacute sclerosing panencephalitis (SSPE) remains a concern, with Brazilian data revealing endemic regions and a significant delay in diagnosis. The study highlights a notable incidence of SSPE following measles immunization.
Area of Science:
- Neurology
- Epidemiology
- Virology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological deterioration linked to persistent measles virus infection.
- While measles eradication efforts have reduced SSPE incidence, sporadic cases continue to emerge globally.
- Understanding the current epidemiological landscape of SSPE is crucial for public health strategies.
Purpose of the Study:
- To delineate the epidemiological characteristics of SSPE cases in Brazil.
- To identify geographical hotspots and demographic patterns of SSPE.
- To analyze diagnostic delays and outcomes in Brazilian SSPE patients.
Main Methods:
- A nationwide protocol was distributed to Child Neurologists across Brazil.
- Data from 48 registered SSPE patients were collected and analyzed.
- Epidemiological variables, clinical presentation, diagnostic markers (EEG), and patient outcomes were documented.
Main Results:
- The Southeast region accounted for 56% of cases; 71% of patients were male and 73% were white.
- Measles infection preceded SSPE in 56% of cases, while 19% had prior measles and immunization, and 14% were immunized only.
- The mean time from symptom onset to diagnosis was 12 months, with 75% exhibiting characteristic EEG pseudoperiodic complexes; outcomes varied, including death, remission, and disability.
Conclusions:
- Brazil exhibits endemic regions for SSPE, underscoring the need for continued surveillance.
- A significant proportion of SSPE cases occur post-immunization, warranting further investigation into vaccine-related factors or waning immunity.
- The substantial delay between symptom onset and diagnosis indicates challenges in early SSPE detection and management.