Abstract:
Glutaric aciduria type I is an uncommon inborn error
of metabolism. It is a serious disease, often with a fatal
outcome. Magnetic resonance imaging findings and the
clinical course of monozygotic twin females with glutaric
aciduria type I who were admitted with acute
encephalopathic crisis symptoms 3 days after immunization
for poliovirus are presented in this report.
Magnetic resonance imaging findings revealed hyperintensity
in the putamen, head of the left caudate
nucleus, and globus pallidus, periventricular white
matter (on T2-weighted images), arachnoid cysts in
bilateral temporal regions, and enlargement of the
sylvian fissures. Glutaric aciduria type I should be
included in the differential diagnosis of patients with
acute encephalopathic crisis occurring shortly after
poliovirus immunization. Typical magnetic resonance
findings guide urinary organic acid analysis in these
patients.
