What are the symptoms of hepatic encephalopathy (HE) in patients with portosystemic shunting?

Updated: Apr 04, 2019
  • Author: David C Wolf, MD, FACP, FACG, AGAF, FAASLD; Chief Editor: BS Anand, MD  more...
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Answer

Extrapyramidal symptoms—including tremor, bradykinesia, cog-wheel rigidity, and shuffling gait—have been described in patients with portosystemic shunting. [31, 32] These symptoms may or may not be associated with hyperammonemia. It is postulated that manganese deposition in the basal ganglia may predispose patients to develop these symptoms. [31] However, some patients with the "Parkinsonian phenotype of hepatic encephalopathy" may respond to treatment with rifaximin. [32]

Another neurologic condition that may be seen in the setting of portosystemic shunting is hepatic myelopathy. It is a rare condition that has been described in patients with cirrhosis of varying degrees of severity, patients who have undergone portosystemic shunt surgery or the creation of a transjugular intrahepatic portosystemic shunt (TIPS), and noncirrhotic patients with portosystemic shunts. Patients may present with lower extremity weakness, difficulty walking, spastic paraparesis, and hyperreflexia. [33] Although patients typically have concomitant hepatic encephalopathy, this is not invariable. [34] Symptoms may be rapidly progressive in some patients. Neurologic deficits do not typically respond to standard medical therapies for hepatic encephalopathy. Neurologic improvement has been described after TIPS closure [34] and after liver transplantation. [33]


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