Many epidemiological studies have demonstrated the exceptionally high
incidence of autism in children with the fragile X syndrome, and autis
m is often considered a << behavioral phenotype >> of this syndrome. H
owever, the discrepancies between the results of these studies disclos
ed strong effects of methodological flaws and demonstrated the need fo
r gathering clinical data. Atypical << autistic-like >> behaviors were
then found to be common, early symptoms of the syndrome occurring aga
inst the background of early manifestations of mental retardation. The
se behaviors reflect these children's exquisite reactivity to change a
nd contact with others. Avoidance of eye contact is the most significa
nt feature. The appropriate diagnosis is not autism but phobia of soci
al relationships. This highly specific vulnerability, which may be inh
erited, probably leads some of these patients to experience the fate o
f autistic children. It highlights the influence of environment on the
clinical course and indirectly supports the role of early specialized
care.