CC BY-NC-ND 4.0 · Ann Natl Acad Med Sci 2023; 59(04): 225-228
DOI: 10.1055/s-0043-1777315
Case Reports

“Brain on Fire”: Hyperperfusion as a Hallmark of Hyperammonemic Encephalopathy

1   Department of Radiodiagnosis, All India Institute of Medical Sciences, Bathinda, Punjab, India
,
1   Department of Radiodiagnosis, All India Institute of Medical Sciences, Bathinda, Punjab, India
,
Ritish Gupta
1   Department of Radiodiagnosis, All India Institute of Medical Sciences, Bathinda, Punjab, India
,
Priya Bhat
2   Department of Microbiology, Adesh Institute of Medical Sciences, Bathinda, Punjab, India
› Author Affiliations

Abstract

We describe a very rare case of acute fulminant hepatic failure as a complication of acute viral hepatitis caused by hepatitis A virus, complicated by hyperammonemic encephalopathy. The brain magnetic resonance imaging (MRI) findings were suggestive of cytotoxic edema involving bilateral cerebral hemispheres. The novel findings of hyperperfusion on arterial spin labeling perfusion MRI and hyperemic hypoxia on susceptibility weighted imaging are discussed. The patient had a rapid progression of cerebral edema and succumbed to the illness despite supportive care. Characteristic neuroimaging findings may help in the diagnosis of acute hyperammonemic encephalopathy of brain MRI, which may be useful in leading to appropriate clinical workup and diagnosis of the underlying cause of hyperammonemia. In our case, hyperammonemic encephalopathy was precipitated by fulminant hepatic failure caused by hepatitis A virus, which is a rare occurrence.

Ethics Statement

The authors declare that the manuscript conforms to the Declarations of Helsinki. Informed written consent was taken from the guardians of the patient for data acquisition and publication.


Authors' Contribution

S.P. contributed to drafting of the manuscript and data acquisition. P.S. contributed to data acquisition, and approval of the final draft of the manuscript. R.G. contributed to drafting of the manuscript and approval of the final draft of the manuscript. P.B. contributed to data collection and approval of the final draft of the manuscript.




Publication History

Article published online:
18 December 2023

© 2023. National Academy of Medical Sciences (India). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Shammout R, Alhassoun T, Rayya F. Acute liver failure due to hepatitis A virus. Case Rep Gastroenterol 2021; 15 (03) 927-932
  • 2 Rovegno M, Vera M, Ruiz A, Benítez C. Current concepts in acute liver failure. Ann Hepatol 2019; 18 (04) 543-552
  • 3 Jayakumar AR, Norenberg MD. Hyperammonemia in hepatic encephalopathy. J Clin Exp Hepatol 2018; 8 (03) 272-280
  • 4 Reis E, Coolen T, Lolli V. MRI findings in acute hyperammonemic encephalopathy: three cases of different etiologies—teaching point: to recognize MRI findings in acute hyperammonemic encephalopathy. J Belg Soc Radiol 2020; 104 (01) 9
  • 5 Tranah TH, Paolino A, Shawcross DL. Pathophysiological mechanisms of hepatic encephalopathy. Clin Liver Dis (Hoboken) 2015; 5 (03) 59-63
  • 6 Llansola M, Rodrigo R, Monfort P. et al. NMDA receptors in hyperammonemia and hepatic encephalopathy. Metab Brain Dis 2007; 22 (3-4): 321-335
  • 7 Auron A, Brophy PD. Hyperammonemia in review: pathophysiology, diagnosis, and treatment. Pediatr Nephrol 2012; 27 (02) 207-222
  • 8 Larsen FS. Cerebral blood flow in hyperammonemia: heterogeneity and starling forces in capillaries. Metab Brain Dis 2002; 17 (04) 229-235
  • 9 Bjerring PN, Gluud LL, Larsen FS. Cerebral blood flow and metabolism in hepatic encephalopathy: a meta-analysis. J Clin Exp Hepatol 2018; 8 (03) 286-293
  • 10 Sen K, Whitehead MT, Gropman AL. Multimodal imaging in urea cycle-related neurological disease: what can imaging after hyperammonemia teach us?. Transl Sci Rare Dis 2020; 5 (1–2): 87-95
  • 11 Siwicka-Gieroba D, Robba C, Gołacki J, Badenes R, Dabrowski W. Cerebral oxygen delivery and consumption in brain-injured patients. J Pers Med 2022; 12 (11) 1763
  • 12 Verma RK, Abela E, Schindler K. et al. Focal and generalized patterns of cerebral cortical veins due to non-convulsive status epilepticus or prolonged seizure episode after convulsive status epilepticus: a MRI study using susceptibility weighted imaging. PLoS One 2016; 11 (08) e0160495
  • 13 Ko SB, Ortega-Gutierrez S, Choi HA. et al. Status epilepticus-induced hyperemia and brain tissue hypoxia after cardiac arrest. Arch Neurol 2011; 68 (10) 1323-1326
  • 14 Muttikkal TJ, Wintermark M. MRI patterns of global hypoxic-ischemic injury in adults. J Neuroradiol 2013; 40 (03) 164-171
  • 15 Garg P, Aggarwal A, Malhotra R, Dhall S. Osmotic demyelination syndrome - evolution of extrapontine before pontine myelinolysis on magnetic resonance imaging. J Neurosci Rural Pract 2019; 10 (01) 126-135
  • 16 Ren S, Chen Z, Liu M, Wang Z. The radiological findings of hypoglycemic encephalopathy: a case report with high b value DWI analysis. Medicine (Baltimore) 2017; 96 (43) e8425
  • 17 Barrot A, Huisman TA, Poretti A. Neuroimaging findings in acute pediatric diabetic ketoacidosis. Neuroradiol J 2016; 29 (05) 317-322
  • 18 Fazeli S, Noorbakhsh A, Imbesi SG, Bolar DS. Cerebral perfusion in posterior reversible encephalopathy syndrome measured with arterial spin labeling MRI. Neuroimage Clin 2022; 35: 103017