Interferon alpha in subacute sclerosing panencephalitis: Case report and review of the literature

P L A Bill,K Moodley,V B Patel

doi:10.4102/sajid.v31i4.76

Abstract

Subacute Sclerosing Panencephalitis (SSPE) is a rare, rapidly progressive neurodegenerative disease caused by the measles virus. Spontaneous remission and survival longer than 4 years is rare. The disease commonly affects children and adolescents from less privileged socio-economic backgrounds. Prior to immunisation, the incidence of SSPE in South Africa (SA) was 2.6/100 000 people per year, which is higher than the global estimate of 1/1 000 000 per year. The post-immunisation incidence has dropped to 0.43/100 000 people per year. Currently, there is no curative treatment for SSPE. In developed countries, patients have been treated with antiviral therapy and interferon alpha (IFNα) with promising results. There have been no reported studies of children with SSPE treated with IFNα in South Africa. We report on a patient successfully treated with intraventricular INFα.

Highlights

Subacute Sclerosing Panencephalitis (SSPE) is a rare, rapidly progressive neurodegenerative disease
She was readmitted for a relapse of her SSPE which was confirmed by recurrence of her periodic complexes on a slow EEG background, and her repeat measles specific antibodies in the cerebrospinal spinal fluid (CSF) were again positive
Far the most promising results are seen with interferon alpha administered via the intraventricular route

Summary

CASE REPORT

Interferon alpha in subacute sclerosing panencephalitis: Case report and review of the literature. Subacute Sclerosing Panencephalitis (SSPE) is a rare, rapidly progressive neurodegenerative disease caused by the measles virus. Spontaneous remission and survival longer than 4 years is rare. The disease commonly affects children and adolescents from less privileged socio-economic backgrounds. The incidence of SSPE in South Africa (SA) was 2.6/100 000 people per year, which is higher than the global estimate of 1/1 000 000 per year. The post-immunisation incidence has dropped to 0.43/100 000 people per year. Patients have been treated with antiviral therapy and interferon alpha (IFNα) with promising results. There have been no reported studies of children with SSPE treated with IFNα in South Africa. We report on a patient successfully treated with intraventricular INFα

Introduction

KwaZulu Natal

Masahito Miyazaki Panitchi Steiner Kurata Horiquchi

Discussion

Conclusion