Résumé
This study was conducted to observe the impact of measles vaccination on the epidemiology of subacute sclerosing panencephalitis (SSPE) in the post measles vaccination era. This is a retrospective study from a tertiary care hospital, covering a ten year period starting a decade after the introduction of the national measles immunization programme in India. We analyzed 458 serologically confirmed SSPE cases. These patients had a high cerebrospinal fluid: serum anti-measles antibody ratio. The male to female ratio in the present study was 4.4:1. The mean age at onset of SSPE was 13.3 years, showing an increase in mean age at onset of SSPE. Clinical and other demographic details, available from 72 in-patients, are discussed in this report. Of these, a history of measles could be elicited in 34 cases. Mean latent period between measles infection and onset of SSPE was 7.8 years. Six patients gave a history of measles vaccination. A sizable percentage (15.5 %) of the patients was ≥ 18 years old and considered to have adult onset SSPE. The incidence of SSPE continues to be high and this report highlights the need for further strengthening routine measles immunization coverage.
Résumé
Central core disease is a congenital myopathy characterized by generalized hypotonia, muscle weakness and presence of central cores on muscle biopsy. It generally presents during infancy. It is familial with autosomal dominant inheritance [Chromosome 19q13.1; Gene Locus RyR1 (Ryanodine receptor gene)]. We report here two cases of central core disease in a 3-year-old male child and 8 year old female child.