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VARIATIONS IN CLINICAL PRESENTATION, NEUROIMAGING AND ELECTROENCEPHALOGRAPHIC PATTERNS OF SUBACUTE SCLEROSING PANENCEPHALITIS

Objective: To determine the variations in clinical presentation, neuroimaging and electroencephalographic patterns of subacute sclerosing panencephalitis. Study Design: Cross sectional study. Place and Duration of Study: The Children’s Hospital & Institute of Child Health, Lahore, Pakistan from July 2020 to December 2020. Methodology: This study was conducted on children presented with clinical features suggestive of subacute sclerosing panencephalitis, along with positive anti-measles antibodies on cerebrospinal fluid. Association between variables was determined to formulate early diagnosis of subacute sclerosing panencephalitis Results: Out of 47 children, 29 were males with mean age of 6.54 ± 2.9 years. Only 23% were fully immunized against measles, 36.2% were unvaccinated and 40.4% received partial immunization. Mean age of measles infection was 1.49 ± 1.2years; mean interval between measles and onset of SSPE was 4.13 ± 3.0 year. Atypical clinical presentation was seen in 38.3% with intractable epilepsy (8.5%), focal deficit (8.5%) and extrapyramidal symptoms (8.5%) being commonest followed by coma (6.4%), visual loss (4.3%) and psychosis (2.1%). Neuroimaging was suggestive of cortical hyperintensities in 46.8% and was normal in 46.8%. Electroencephalography showed burst suppression in 55.3% and atypical findings in 19.1%. Younger age (1-1.5 years) of measles and unimmunized status were associated with early onset of SSPE with p-value of 0.001 and 0.05 respectively. Non immunized status was associated with atypical presentation of SSPE (p-value<0.05). Conclusion: The younger age of measles infection and failure to receive complete immunization led to early onset of SSPE with an atypical presentation.