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Subacute Sclerosing Panencephalitis (SSPE): Experience from a Tertiary-Care Pediatric Center
Meenal Garg1, Anshita Arora2, Shilpa D Kulkarni3
1Department of Pediatric Neurology, Surya Hospitals, Jaipur, Rajasthan, India.
Journal of Neurosciences in Rural Practice
|June 13, 2022
Summary
Subacute sclerosing panencephalitis (SSPE), a measles complication, presents variably, delaying diagnosis in low-resource settings. Early recognition of atypical features and EEG are crucial for prognosis and timely treatment.
Area of Science:
- Neurology
- Pediatrics
- Infectious Diseases
Background:
- Subacute sclerosing panencephalitis (SSPE) is a severe, progressive neurodegenerative disease following measles infection.
- It disproportionately affects children in low-resource countries, often leading to diagnostic challenges due to varied clinical and electrographic presentations.
Purpose of the Study:
- To analyze the demographic, clinical, and outcome data of SSPE patients in a tertiary care pediatric hospital in India.
- To highlight the importance of recognizing atypical SSPE presentations and the role of electroencephalography (EEG) in diagnosis.
Main Methods:
- Retrospective review of 34 children diagnosed with SSPE based on Dyken's criteria over 8 years.
- Data collected included demographics, clinical features, investigations, treatment, and patient outcomes.
Main Results:
- The average age of symptom onset was 7 years, 5 months, with most patients unvaccinated for measles.
- Eighty percent presented at stage 2 illness, and approximately 25% exhibited atypical clinical features.
- Electroencephalography (EEG) was the most valuable diagnostic investigation, while myoclonus was the most common post-diagnosis feature.
Conclusions:
- Timely diagnosis and prognosis in high-incidence areas require recognizing atypical SSPE presentations.
- EEG findings are critical for SSPE diagnosis, and measles eradication is key to eliminating this disease.
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