Unexpectedly abnormal MR brain scan in liver disease

Area of Science:

• Neurology
• Hepatology
• Radiology

Background:

• Acute-on-chronic liver failure can lead to severe hyperammonaemia.
• Hyperammonaemia is a known cause of hepatic encephalopathy and neurological dysfunction.
• The specific neuroimaging findings and their pathophysiological basis in such cases require further elucidation.

Purpose of the Study:

• To describe the clinical presentation and neuroimaging findings in a case of coma secondary to acute-on-chronic liver failure with severe hyperammonaemia.
• To investigate the pathophysiological mechanisms underlying the observed brain changes through postmortem examination.
• To provide insights for clinicians regarding the prognosis of similar cases.

Main Methods:

• Case report of an elderly woman with acute-on-chronic liver failure and severe hyperammonaemia.
• Brain Magnetic Resonance (MR) imaging including fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI).
• Postmortem neuropathological examination.

Main Results:

• The patient presented with coma and severe hyperammonaemia.
• MR scan revealed extensive bilateral and symmetric cortical and thalamic FLAIR hyperintensities and diffusion restriction.
• Postmortem findings offered new insights into the pathophysiology of hyperammonaemic brain injury.

Conclusions:

• Severe hyperammonaemia in the context of liver failure can cause characteristic and extensive brain MRI abnormalities.
• Postmortem findings enhance understanding of the pathophysiology of hyperammonaemic coma.
• These insights may aid clinicians in addressing the prognosis of patients with similar presentations.