Subacute Sclerosing Panencephalitis in Indian Children Under 5 Years during the COVID Pandemic: A Paradigm Shift in the Clinical Profile

Abstract

Background: Subacute sclerosing panencephalitis (SSPE) is a progressive neurodegenerative disorder of children and young adults. In the setting of the COVID-19 pandemic, we have observed increasing admissions of SSPE and a change in the clinical profile, mainly younger age of presentation, i.e., children under the age of 5 years. Clinical Description: We presented the clinical and investigational characteristics of 20 children who were diagnosed as SSPE during the period March 2020–March 2021 in our hospital, based on the Dyken criteria. The mean age was observed to be 3 years 9 months and male-to-female ratio of 1:1. Most children presented with developmental regression and myoclonic jerks. The clinical details and investigations were presented and a few atypical cases were discussed. Management: All the patients were treated symptomatically: anticonvulsant drugs, nutritional rehabilitation and supported feeding, occupational therapy, and palliative therapy. A trial of isoprinosine was started in all the patients, but was discontinued by most due to the expense. There were no mortalities. Conclusion: The change in profile of SSPE to an earlier onset in the setting of the pandemic should make the clinician keep a high index of suspicion of this disorder in children under the age of five years who present with unexplained developmental regression or acute encephalopathy, before the onset of typical myoclonus.

Keywords

• developmental regression
• measles
• myoclonic jerks
• pseudoperiodic complexes