Brain MR imaging in acute hyperammonemic encephalopathy arising from late-onset ornithine transcarbamylase deficiency

J Takanashi, AJ Barkovich, SF Cheng… - American journal …, 2003 - Am Soc Neuroradiology
The brain MR imaging characteristics of three patients with acute hyperammonemic
encephalopathy resulting from late-onset ornithine transcarbomylase deficiency are …

[HTML][HTML] Acute hyperammonemic encephalopathy in adult onset ornithine transcarbamylase deficiency

OM Panlaqui, K Tran, A Johns, J McGill… - Intensive Care Medicine, 2008 - Springer
Objectives To report the clinical manifestations of acute hyperammonemic encephalopathy
in adult onset ornithine transcarbamylase deficiency (OTCD). Design Case report. Setting …

Neurological features and computed tomography of the brain in children with ornithine carbamoyl transferase deficiency.

BE Kendall, DP Kingsley, JV Leonard… - Journal of Neurology …, 1983 - jnnp.bmj.com
The clinical features and the computed tomographic appearances of the brain in seven
children with ornithine carbamoyl transferase deficiency are described. Episodic vomiting …

Brain MR imaging in neonatal hyperammonemic encephalopathy resulting from proximal urea cycle disorders

J Takanashi, AJ Barkovich, SF Cheng… - American journal …, 2003 - Am Soc Neuroradiology
We present brain MR images in three patients with neonatal-onset hyperammonemic
encephalopathy resulting from urea-cycle disorders (two sisters with deficiency of the …

Acute extrapyramidal syndrome in mild ornithine transcarbamylase deficiency: metabolic stroke involving the caudate and putamen without metabolic …

CE Keegan, DM Martin, DJ Quint, JL Gorski - European journal of …, 2003 - Springer
A 6-year-old male with partial ornithine transcarbamylase (OTC) deficiency had acute and
rapidly progressive symmetrical swelling of the head of the caudate nuclei and putamina …

Hyperammonaemic encephalopathy after initiation of valproate therapy in unrecognised ornithine transcarbamylase deficiency

M Oechsner, C Steen, HJ Stürenburg… - Journal of Neurology …, 1998 - jnnp.bmj.com
Ornithine transcarbamylase deficiency is an X linked disorder and the most common
inherited cause of hyperammonaemia. Fluctuating concentrations of ammonia, glutamine …

Magnetic resonance spectroscopy shows increased brain glutamine in ornithine carbamoyl transferase deficiency

A Connelly, JH Cross, DG Gadian, JV Hunter… - Pediatric …, 1993 - nature.com
We have performed localized in vivo proton magnetic resonance spectroscopy on two
females with ornithine carbamoyl transferase deficiency during episodes of acute …

Cranial MRI in acute hyperammonemic encephalopathy

PS Bindu, S Sinha, AB Taly, R Christopher… - Pediatric …, 2009 - Elsevier
Cranial magnetic resonance imaging was performed in three cases of acute
hyperammonemic encephalopathy with three diverse etiologies: infantile citrullinemia, acute …

Osmotic demyelination syndrome as a consequence of treating hyperammonemia in a patient with ornithine transcarbamylase deficiency

JF Cardenas, JB Bodensteiner - Journal of child neurology, 2009 - journals.sagepub.com
A 7-year-old female patient with a new diagnosis of ornithine transcarbamylase deficiency
was treated for hyperammonemia with a standard protocol. Several days later, she …

Severe cerebral damage in ornithine transcarbamylase deficiency.

CL Dolman, RA Clasen, K Dorovini-Zis - Clinical neuropathology, 1988 - europepmc.org
Two patients are described with hyperammonemia due to ornithine transcarbamylase (OTC)
deficiency who suffered severe shrinkage and collapse of the brain. The cerebral cortex was …