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Magnetic resonance spectroscopic characteristics of glutaric aciduria type II

Published online by Cambridge University Press:  18 September 2006

Ahmet Kemal Firat ,
Hakki Muammer Karakas  and
Cengiz Yakinci
Ahmet Kemal Firat
Affiliation:
Department of Radiology, Turgut Ozal Medical Center, Inonu University Medical Faculty, Malatya, Turkey.
Hakki Muammer Karakas
Affiliation:
Department of Radiology, Turgut Ozal Medical Center, Inonu University Medical Faculty, Malatya, Turkey.
Cengiz Yakinci
Affiliation:
Department of Paediatrics, Turgut Ozal Medical Center, Inonu University Medical Faculty, Malatya, Turkey.
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Abstract

Magnetic resonance spectroscopy (MRS) of a 12-year-old female patient with glutaric aciduria type II was compared with data obtained from four healthy age- and sex-matched volunteers. In the clinically active phase, conventional magnetic resonance imaging showed mild ventricular dilatation. Frontal lobe choline/creatine (Cho/Cr) ratio (1.98) was higher than the ratios reported for the comparison participants (1.64 [SD 0.21]). The N-acetylaspartate/creatine (NAA/Cr) ratio (1.95) was lower than normal limits (2.66 [SD 0.23]). After successful riboflavin treatment and dietary restriction for proteins, the NAA/Cr ratio was within the normal range (2.44) and Cho/Cr ratio was below the normal range (1.15), suggesting riboflavin-responsive multiple acyl-coA dehydrogenase deficiency. An elevated Cho/Cr ratio and decreased NAA/Cr ratio is consistent with a demyelinating process in the active phase of glutaric aciduria type II. MRS helps to monitor the progress of the disease and the efficacy of treatment by revealing changes in NAA/Cr and Cho/Cr ratios.

Type
Case Report
Information
Developmental Medicine and Child Neurology , Volume 48 , Issue 10 , October 2006 , pp. 847 - 850
Copyright
2006 Mac Keith Press

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