Mortality in childhood progressive encephalopathy from 1985 to 2004 in Oslo, Norway: a population-based study.
OBJECTIVE
The aims were to estimate case fatality and survival rates, standardized mortality ratio (SMR), and independent prognostic factors for survival, in a population-based cohort of progressive encephalopathy (PE) patients.


METHODS
We divided onset of disease into neonatal and postneonatal groups and aetiology into metabolic (n=55), neurodegenerative (n=27) and HIV encephalopathy (n=2) groups. Case fatality was the number of deaths divided by the number of patients. Cumulative survival probability at 10 years of follow-up and independent risk factors for mortality were analyzed using the Kaplan-Meier survival curve and the Cox model.


RESULTS
Case fatality was 36.9% and the mean and median follow-up times were 3109 and 2887 days. At 1 and 10 years, the cumulative probability of survival was 81% and 66%. Neonatal onset showed increased risk of death compared to postneonatal onset (RR 3.0; 95% CI 1.4-6.2). Metabolic aetiology showed increased risk of death compared to other aetiology (RR 1.25; 95% CI 1.10-1.46). The SMR of 37.7 for boys and 23.8 for girls was significantly increased (p<0.001) compared to the total Norwegian population stratified by gender and age.


CONCLUSIONS
Children with PE showed a vast excess in mortality compared to the general population stratified by gender and age. Neonatal presentation and metabolic aetiology were the most significant factors for increased risk of death.
Strømme
Petter
P
Department of Pediatrics, Ullevål University Hospital, Oslo, Norway. petter.stromme@medisin.uio.no
Magnus
Per
P
Kanavin
Øivind Juris
ØJ
Rootwelt
Terje
T
Woldseth
Berit
B
Abdelnoor
Michael
M
D015526:AIDS Dementia Complex / Q000401:mortality*; D001928:Brain Diseases, Metabolic / Q000401:mortality*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D019636:Neurodegenerative Diseases / Q000401:mortality*; D009664:Norway / Q000453:epidemiology; D012307:Risk Factors; D016019:Survival Analysis; D015996:Survival Rate