Clinical, Demographic, Neuro Imaging Features, and Outcome of Subacute Sclerosing Pan Encephalitis in a Pediatric Cohort

Abstract

Abstract 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.