The
typical age of onset in the BMD is 6.5 years, and the progression
of disease is usually rapid, with a mean survival time following
diagnosis of only 49 days (Abadie et al., J.Hered, 2009).
Given their
typical age of onset, a broad range
of mental disorders are increasingly being understood as the result
of aberrations
of developmental processes that normally occur in the adolescent brain.4 — 6 Executive functioning, and its neurobiological substrate, the prefrontal cortex, matures during adolescence.5 The relatively late maturation
of executive functioning is adaptive in most cases, underpinning characteristic adolescent behaviours such as social interaction, risk taking and sensation seeking which promote successful adult development and independence.6 However, in some cases it appears that the delayed maturation
of prefrontal regulatory regions leads to the development
of mental illness, with neurobiological studies indicating a broad deficit in executive functioning which precedes and underpins a range
of psychopathology.7 A recent meta - analysis
of neuroimaging studies focusing on a range
of psychotic and non-psychotic mental illnesses found that grey matter loss in the dorsal anterior cingulate, and left and right insula, was common across
diagnoses.8 In a healthy sample, this study also demonstrated that lower grey matter in these regions was found to be associated with deficits in executive functioning performance.
Deaf children whose hearing losses were diagnosed by 6 months
of age and provided early intervention shortly after
diagnosis showed
typical development
of language abilities as compared to those who were diagnosed later and subsequently, if at all, provided early intervention services (Yoshinaga - Itano, Sedey, Coulter, & Mehl, 1998).