Portosystemic Encephalopathy without Liver Disease Masquerading as Dementia

Area of Science:

• Neurology
• Gastroenterology
• Vascular Surgery

Background:

• Hyperammonemia can cause neurological deficits, including consciousness disturbances.
• Dementia patients may have undiagnosed underlying conditions contributing to cognitive decline.
• Portosystemic shunts, abnormal connections between the portal vein and systemic circulation, can lead to ammonia buildup.

Observation:

• An 84-year-old woman with dementia presented with hyperammonemia and consciousness disorder.
• Imaging revealed a large portosystemic shunt without evidence of liver disease.
• Medical management initially improved her consciousness.

Findings:

• Percutaneous embolization of the portosystemic shunt was performed electively.
• The patient experienced significant clinical improvement following the embolization procedure.
• This suggests a direct link between the shunt, hyperammonemia, and neurological symptoms.

Implications:

• Consciousness disturbance in dementia patients may be linked to hyperammonemia from portosystemic shunts.
• Increasing hepatopetal portal blood flow via shunt treatment may improve neurological function.
• Shunt embolization could be a valuable therapeutic option for selected patients, particularly those without underlying liver disease.