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[97]
Davison et al. used Quantitative MRS to evaluate concentrations of brain metabolites in children with
neonatal onset propionic acidemia. They tracked metabolic stability, during an acute encephalopathic
episode prior to and after liver transplantation. They concluded that MRS was useful in this setting and
believed it could also be used to evaluate potential therapies [Figure 6].

In the last decade, advances in neuroimaging technologies have led to multiple studies investigating the
neural networks of executive function (EF). EF has long been associated with the PFC involving working
memory, inhibitory control and set shifting. Diamond speculated that neuroimaging can allow the
investigation of many unanswered questions regarding cognitive functions and stated that fMRI and fNIRS
could be of interest in studying the neural correlates of cognition. There are ample opportunities to employ
[98]
this strategy in IEMs .

CONCLUSION
MRI imaging beyond routine structural imaging can shed light on processes that impact the brain during
metabolic crisis. Collaboration with physicists and neuroimaging specialists can allow further detailed
study and identify biomarkers that can be quickly put into clinical practice.

DECLARATIONS
Authors’ contributions
Design of the manuscript, writing and editing the manuscript: Gropman AL, Anderson A

Availability of data and materials
None.

Financial support and sponsorship
The author was partially supported by NIH 2U54HD061221-16 (AG); The O’Malley Family Foundation
(AG).

Conflicts of interest
Both authors declared that there are no conflicts of interest.

Ethical approval and consent to participate
Not applicable.

Consent for publication
Written informed consent for publication was obtained.

Copyright
© The Author(s) 2020.

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