Lifespan changes in working memory in fragile X premutation males

摘要: Fragile X syndrome is the world's most common hereditary cause of developmental delay in males and now well characterized at biological, brain cognitive levels. The disorder caused by silencing a single gene on chromosome, FMR1 gene. premutation (carrier) status, however, less documented but has an emerging literature that highlights more subtle profile executive deficiencies mirror those reported fully affected males. Rarely, issue age-related declines performance been addressed. In present study, we focus specifically domain working memory its subcomponents (verbal, spatial central memory) explore across broad sample aged 18-69 years matched age IQ to unaffected comparison We further tease apart status into with symptoms newly identified neurodegenerative disorder, fragile X-associated tremor/ataxia (FXTAS) currently symptom-free. Our findings indicate specific vulnerability tasks require simultaneous manipulation storage new information, so-called control memory. Furthermore, this appears exist regardless presence FXTAS symptoms. Males demonstrated general impairment encompassing phonological addition Among asymptomatic males, observed novel finding relationship between increased CGG repeat size