Acute hyperammonemic encephalopathy secondary to ornithine transcarbamylase deficiency in a child: findings in diagnostic imaging

Miranda, Guilherme Marcelino de; Marcon, N.; Basso, Caio Ferraz; Júnior, Luiz Antônio Jorge; Queiroz, Rodolfo Mendes

doi:10.1590/0004-282x20190117

Arq. Neuro-Psiquiatr.

2019

DOI: 10.1590/0004-282x20190117

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Acute hyperammonemic encephalopathy secondary to ornithine transcarbamylase deficiency in a child: findings in diagnostic imaging

Guilherme Marcelino de Miranda¹,

N. Marcon²,

Caio Ferraz Basso³

et al.

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2023

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Histopathological examination of characteristic brain MRI findings in acute hyperammonemic encephalopathy: A case report and review of the literature

Denk,

Kunzmann,

Maieron

et al. 2023

Neuroradiol J

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Introduction Acute hyperammonemic encephalopathy is associated with distinct brain MRI findings, namely, hyperintensity in T2-weighted sequences as well as restricted diffusion in diffusion-weighted imaging with accentuation in the insular cortex and cingulate gyrus. The pathophysiology and the histopathological correlates of these characteristic MRI findings are largely unknown. Case report We present a 57-year-old male with a history of chronic alcohol abuse, liver cirrhosis and portal hypertension, and a clinical syndrome (variceal bleeding, depression of consciousness, seizures), elevated plasma ammonia levels, and characteristic brain MRI abnormalities suggestive of acute hyperammonemic encephalopathy. A postmortem histopathological examination revealed extensive hypoxic ischemic encephalopathy without evidence for metabolic encephalopathy. No episodes of prolonged cerebral hypoxemia were documented throughout the course of the disease. We conducted a review of the literature, which exhibited no reports of hyperammonemic encephalopathy in association with characteristic brain MRI findings and a consecutive histopathological examination. Conclusion This is the first report of a patient with acute hyperammonemic encephalopathy together with characteristic brain MRI findings and a histopathological correlation. Although characteristic MRI findings of acute hyperammonemic encephalopathy were present, a histopathological examination revealed only hypoxic pathology without signs of metabolic encephalopathy.

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Histopathological examination of characteristic brain MRI findings in acute hyperammonemic encephalopathy: A case report and review of the literature

Denk,

Kunzmann,

Maieron

et al. 2023

Neuroradiol J

View full text Add to dashboard Cite

show abstract

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Acute hyperammonemic encephalopathy secondary to ornithine transcarbamylase deficiency in a child: findings in diagnostic imaging

Cited by 1 publication

References 4 publications

Histopathological examination of characteristic brain MRI findings in acute hyperammonemic encephalopathy: A case report and review of the literature

Histopathological examination of characteristic brain MRI findings in acute hyperammonemic encephalopathy: A case report and review of the literature

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