The purpose of this study, through a retrospective epidemiological survey c
arried out over three geographical areas in France, was to characterize the
aetiological factors involved in severe mental retardation (SMR) within a
geographically defined population of children with disabilities aged betwee
n 7 and 16 years. The inclusion criteria for SMR (IQ<50) were met by 1150 c
hildren born between 1976 and 1985. Of these children, aetiology was known
in 25%, suspected (or unclear) in 26%, and unknown in 49%. These rates of k
nows and suspected aetiology varied between the groups of children with CP
and those without CP. An analysis of factors associated with SMR was undert
aken among the 144 subjects with SMR, of suspected or unknown aetiology, wh
o had been referred to a neonatal care unit with or without intensive care
(NCU) during their neonatal period. These subjects with SMR were compared w
ith 864 children without SMR (control children) who were also referred to a
n NCU during their neonatal period. The main specific associated factors we
re a prolonged intubation of more than 24 hours, a very low birthweight (<1
500 g) for children with an associated clinical feature of CP, and the pres
ence of isolated neonatal fits and a time of transfer to the NCU of more th
an 4 hours after birth for children without an associated clinical feature
of CP. Although common associated factors were encountered in the children
with SMR with CP and the children with SMR without CP, the results of this
study suggest differences in the underlying pathogenic factors.