Epidemiology of Subacute Sclerosing Panencephalitis (SSPE) in Germany from 2003 to 2009: A Risk Estimation

Maria-Sabine Ludwig,Manfred Wildner,Jean-Pierre Vartanian,Benedikt Weissbrich,Katharina Schönberger

doi:10.1371/journal.pone.0068909

Abstract

Subacute sclerosing panencephalitis (SSPE) is a fatal long-term complication of measles infection. We performed an estimation of the total number of SSPE cases in Germany for the period 2003 to 2009 and calculated the risk of SSPE after an acute measles infection. SSPE cases were collected from the Surveillance Unit for Rare Paediatric Diseases in Germany and the Institute of Virology and Immunobiology at the University of Würzburg. The total number of SSPE cases was estimated by capture-recapture analysis. For the period 2003 to 2009, 31 children with SSPE who were treated at German hospitals were identified. The capture-recapture estimate was 39 cases (95% confidence interval: 29.2–48.0). The risk of developing SSPE for children contracting measles infection below 5 years of age was calculated as 1∶1700 to 1∶3300. This risk is in the same order of magnitude as the risk of a fatal acute measles infection.

Highlights

Subacute sclerosing panencephalitis (SSPE) is a rare progressive, invariably fatal long-term complication of measles infection
Four cases were excluded because they had no residence in Germany and another three cases because they were older than 16 years of age at the time of SSPE diagnosis
We estimated the risk of developing SSPE after acute measles infection below 5 years of age to be in the range of 1 in 1700 to 1

Summary

Introduction

Subacute sclerosing panencephalitis (SSPE) is a rare progressive, invariably fatal long-term complication of measles infection. The latency period between acute measles and first symptoms of SSPE is usually 4 to 10 years but ranges from 1 month to 27 years [1]. The clinical course of SSPE varies considerably in symptoms, duration and intensity. Four disease stages are observed beginning with changes in personality and behaviour as well as failure in school. The second stage is characterized by massive, repetitive, and frequent myoclonic jerks, seizures and dementia. Rigidity, extrapyramidal symptoms, and progressive unresponsiveness develop. The last stage is characterized by coma, a vegetative state, autonomic failure or akinetic mutism. The survival period after onset of symptoms is typically between one to three years [2,3]

Objectives

Methods

Results

Conclusion