The presence of congenital portosystemic shunts in adult patients is an uncommon entity that can manifests itself with hepatic encephalopathy symptoms. However, its clinical presentation can be very diverse and varied, hence it is essential to bear it in mind to be able to diagnose it. In this manuscript we exemplify this variability by presenting a patient with complex long-standing neurological syndrome of uncertain origin, that was finally diagnosed of portosystemic shunt. To perform a percutaneous shunt closure is recommended to achieve resolution of the clinical manifestations, as happened in our patient.
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