NEW RESEARCH - HOT OFF THE PRESS!
A newly published paper describes a study conducted
by Bouchereau and colleagues on neurocognitive development
in 21 children with MSUD in France. All of the
children had classical MSUD and were diagnosed in the
neonatal period. The children underwent neuropsychological
testing between the ages of 6 and 12 years.
All but 3 of the children had IQ scores within the normal
range. The 3 who scored a low IQ were diagnosed later
and had poorer metabolic control. Almost all of the
children had attention deficit disorder and hyperactivity.
Testing showed impaired mental flexibility and inhibitory
control with impulsivity. Verbal abilities were higher than
performance abilities for some of the children.
The authors concluded that it is important for children
with MSUD to undergo neurological testing so that appropriate
interventions can be initiated.
J Inherit Metab Dis. 2017 Mar 21. doi: 10.1007/s10545-017-0033-7.
[Epub ahead of print]
Neurocognitive profiles in MSUD school-age patients