Last modified: 2018-09-09
Abstract
Introduction: Subacute sclerosing panencephalitis (SSPE) is chronic encephalitis due to prior measles virus infection. This is progressive disease with limited treatment and outcome is usually fatal.
Method: To report a child with SSPE who completely improved with intermittent intrathecal interferon alpha injections.
Case description: A 15 year old boy with unremarkable previous history developed subacute onset progressive cognitive decline, behavioural changes, dysarthria, myoclonic jerks followed by altered sensorium. His MRI brain showed asymmetrical T2/FLAIR hyperintensity involving temporoparietal region and other regions (fig 1A, B). EEG was s/o of high amplitude periodic superimposable discharges at interval of 6-8 second (fig 2A). CSF anti measles antibody (IgG) was high with increased CSF/serum ration. Intrathecal interferon alpha (6 million unit) was started weekly along with valproate and clonazepam. Isoprinosine was not available. Patient started showing improvement in walking in two years, started his study and outdoor play after 3 years. His repeat MRI brain show regression of lesion (fig 1C,D) and EEG (fig 2B) became normal.
Result: Probably intrathecal interferon alpha was probably effective in SSPE as there was clinical, radiological and electrophysiological improvement.
Discussion: Although this case report has its limitations, intrathecal interferon alpha may be effective in SSPE. Although, 5% of patients are reported to have spontaneous remission, it is difficult to predict on individual basis. Hence every patient with SSPE should be offered intrathecal interferon alpha even if other drugs are not available.