Neurometabolic effects of an inborn error of amino acid metabolism demonstrated in vivo by 1H NMR.

The elevated levels of brain histidine in histidinaemic mice are detected in vivo by 1H NMR at 8.5 and 1.9 T. The concentrations determined from the in vivo spectra correlate well with subsequent analytical determinations. This technique is discussed in relation to monitoring phenylalanine in humans with phenylketonuria.