Acute and Reversible Pisa Syndrome as Unusual Presentation of Portosystemic Encephalopathy

Abstract

We present the first case of acute and reversible Pisa Syndrome, as a clinical manifestation of a portosystemic encephalopathy bout occurring in a patient affected with cirrhosis and clinical-radiological signs of acquired hepatocerebral degeneration, without exposure to psychotropic medications. A 62 year-old man suffering from cirrhosis was admitted to our hospital for sudden onset of mild confusion and postural change. He was observed walking and standing with a tilt toward the right during the last two hours. On neurological examination, he showed bilateral asterixis and extrapyramidal signs. Clinical diagnosis of Pisa Syndrome was made in a setting of raised serum ammonia. A brain Magnetic Resonance Imaging showed bilateral altered signal intensities of globus pallidus and middle cerebellum peduncle, rarely reported in acquired hepatocerebral degeneration. The patient received medical treatment for portosystemic encephalopathy and the dystonic posture resolved. We suggest that transient ammonia increase and neuroimaging abnormalities, likely related to manganese deposits in the basal ganglia, might have caused an acute brain dysfunction, namely episodic portosystemic encephalopathy, characterized by clinical features of Parkinsonism as Pisa Syndrome.