Key result
Among 32 children with subacute sclerosing panencephalitis, myoclonic and generalized seizures were the most common initial symptoms (25% each), with 15.6% mortality and 37.5% becoming bedridden.
Why the study?
Subacute sclerosing panencephalitis is a progressive neurological disorder due to persistent defective measles virus infection that remains common in developing countries such as India.
Observational (n=32)
This study highlights that myoclonic seizures and cognitive decline are the most common initial symptoms of SSPE in children, and awareness of atypical presentations is necessary.
High morbidity and mortality in pediatric SSPE should not yet change practice; leaves open need for larger prospective studies.
A bstract Background: Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder in children and young adults due to persistent infection caused by the defective measles virus. It is still common in developing countries such as India. Aim: The aim of this study was to describe the clinical and demographic neuroimaging features, and outcomes of SSPE. Materials and Methods: A retrospective chart review of children diagnosed with SSPE during 2016–2020 was performed and the data were analyzed. Results: Of the 32 patients, 28 (87.5%) were men and four (12.5%) were women. The mean age of onset was 7.28 ± 2.19 years. The initial symptom was myoclonic and generalized seizures in eight (25%) each, behavioral problems in seven (21.9%), frequent falls in six (18.8%), and poor scholastic performance in three (9.4%). One-half of the cohorts had measles in the past and 91% had received the vaccine. Atypical presentations such as akinetic rigid syndrome, and acute disseminated encephalomyelitis (ADEM) like presentations can occur. Magnetic resonance imaging (MRI) brain showed abnormalities in 14 (43.8%) and was normal in 10 (31.3%). The most common abnormality noted was hyperintensities in the frontoparietal region in six (18.8%). Electroencephalogram showed long interval periodic complexes in 21 (65.6%), burst suppression pattern in 7 (22%), and was normal in 4 (12.5%). At the time of diagnosis, 25 (78%) patients were in stage II, 5 (15.6%) in stage III, and 1 (3.1%) child in stage IV and stage I each. Five patients (15.6%) died, 10 (31.3%) lost follow-up, 12 (37.5%) were bedridden, and the disease was static in 5 children (15.6%). Conclusion: Myoclonic seizures and cognitive decline are the most common symptoms in children with SSPE. Knowledge about atypical presentations is essential to avoid unnecessary investigations.
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Cornelius et al. (2023) conducted an observational in Subacute sclerosing panencephalitis (SSPE) (n=32). Subacute sclerosing panencephalitis was evaluated on Clinical, demographic, neuroimaging features, and outcomes. Among 32 children with subacute sclerosing panencephalitis, myoclonic and generalized seizures were the most common initial symptoms (25% each), with 15.6% mortality and 37.5% becoming bedridden.
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