TITLE

MRI in a Woman with Late Onset Ornithine Transcarbamylase Deficiency

AUTHOR(S)
Manto, A.; De Gennaro, A.; Serino, A.; Cozzolino, A.; Colasante, A. G.; La Rocca, M. R.
PUB. DATE
August 2010
SOURCE
Neuroradiology Journal;Aug2010, Vol. 23 Issue 4, p398
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
We describe the brain MR imaging findings of a woman who developed neurologic symptoms due to an acute hyperammonemic encephalopathy resulting from late onset ornithine transcarbamylase deficiency (OCTD). MR images revealed injury (hyperin tense foci on long TR images) to the subcortical white matter of the left precentral and supramarginal gyrus and in the left frontal lobe. These findings presumably reflect the distribution of brain injury from hypoperfusion secondary to severe hyperammonemia. If MR findings suggesting hypoperfusion are detected in a patient with hyperammonemia, the diagnosis of OCTD should be considered. Knowledge of the physiopathological mechanisms of OTCD and of MR imaging of hyperammonemic injury may help expedite diagnosis and treatment and prevent the exitus of patients with this genetic disorder.
ACCESSION #
55527492

 

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