Glutaric aciduria type I: value of diffusion-weighted magnetic resonance imaging for diagnosing acute striatal necrosis.

Abstract:

:Glutaric aciduria type I is a rare disorder of organic acid metabolism caused by deficiency of glutaryl-CoA dehydrogenase. We report the cranial computed tomography (CT) and magnetic resonance (MR) imaging findings in a 5-month-old girl with this disorder who presented with an acute dystonic syndrome. CT findings demonstrated only subtle loss of attenuation in the basal ganglia, MR spectroscopy was normal, and conventional MR images showed increased T2-signal limited to the putamina. Diffusion-weighted MR imaging demonstrated more extensive disease than was apparent either on CT or on the conventional MR images, including bilateral involvement of the putamina, globus pallidus, and caudate nuclei, consistent with acute necrosis of the corpus striatum and lentiform nuclei.

journal_name

J Comput Assist Tomogr

authors

Elster AW

doi

10.1097/00004728-200401000-00016

keywords:

subject

Has Abstract

pub_date

2004-01-01 00:00:00

pages

98-100

issue

1

eissn

0363-8715

issn

1532-3145

journal_volume

28

pub_type

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