TY - JOUR
T1 - Glutaric Aciduria Type I
T2 - Value of Diffusion-Weighted Magnetic Resonance Imaging for Diagnosing Acute Striatal Necrosis
AU - Elster, Allen W.
PY - 2004
Y1 - 2004
N2 - 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.
AB - 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.
KW - Cerebral infarction
KW - Diffusion MR imaging
KW - Glutaric aciduria type I
KW - Magnetic resonance imaging
KW - Striatal necrosis
UR - http://www.scopus.com/inward/record.url?scp=0346095333&partnerID=8YFLogxK
U2 - 10.1097/00004728-200401000-00016
DO - 10.1097/00004728-200401000-00016
M3 - Article
C2 - 14716240
AN - SCOPUS:0346095333
SN - 0363-8715
VL - 28
SP - 98
EP - 100
JO - Journal of computer assisted tomography
JF - Journal of computer assisted tomography
IS - 1
ER -