CC BY-NC-ND 4.0 · J Neurosci Rural Pract 2022; 13(02): 315-320
DOI: 10.1055/s-0041-1740612
Brief Report

Subacute Sclerosing Panencephalitis (SSPE): Experience from a Tertiary-Care Pediatric Center

Meenal Garg
1   Department of Pediatric Neurology, Surya Hospitals, Jaipur, Rajasthan, India
,
Anshita Arora
2   Division of Pediatric Neurology, Department of Pediatric Neurosciences, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India
,
Shilpa D. Kulkarni
3   Department of Pediatric Neurosciences, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India
,
Anaita Udwadia Hegde
3   Department of Pediatric Neurosciences, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India
,
Krishnakumar N. Shah
3   Department of Pediatric Neurosciences, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India
› Author Affiliations
Funding None.

Abstract

Introduction Subacute sclerosing panencephalitis (SSPE) is a devastating neurodegenerative disease occurring as a complication of measles infection that is still prevalent in low-resource countries. Clinical and electrographical variability in SSPE can lead to diagnostic delays.

Methods Children diagnosed with SSPE in a tertiary care pediatric hospital in India in a period of 8 years were included in the study. The diagnosis was established on the basis of Dyken's criteria. The demographic data, clinical presentations, investigations, treatment approaches, and outcomes were reviewed and recorded.

Results Thirty-four patients were included in the analysis. Average age at symptom onset was 7 years, 5 months. Majority of the children were not vaccinated for measles. Most patients (80%) presented with stage 2 of illness. Nearly 25% presented with atypical clinical features. Myoclonus was the most predominant feature seen after diagnosis. Electroencephalography (EEG) was the most useful investigation for suspecting the diagnosis. All patients showed deterioration in neurological status with time and 20% died during follow-up.

Conclusion Atypical presentations of SSPE must be recognized in areas with high incidence to institute timely treatment and establish prognosis. EEG findings were found to be the most important indicator for diagnosis. Measles eradication will pave the way for elimination of this dreaded disease.

Note

Written informed consent was obtained from the parent(s) of the patients for the publication of patient data and accompanying images.


Ethical Approval

Ethical approval for the publication was obtained from the ethics committee of the institute for this study.


Authors' Contributions

M.G. conceptualized the study and wrote the initial draft. M.G. and A.A. were involved in data collection and literature search. S.D.K. revised the initial drafts. A.U.H. and K.N.S. revised and edited the manuscript. All authors were involved in patient care. The final manuscript has been read, revised, and approved by all the authors.




Publication History

Article published online:
10 February 2022

© 2022. Association for Helping Neurosurgical Sick People. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India