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Extrapyramidal dysfunction in cirrhosis: Is hepatic encephalopathy the only answer?

Published:February 10, 2022DOI:https://doi.org/10.1016/j.ejim.2022.01.038

      Keywords

      Case description

      A 68-year-old male with a history of alcohol-related cirrhosis presented with persistent tremor, gait ataxia and choreiform movements of upper limbs over the past 4 days. The patient, that assured treatment compliance and alcohol abstinence, also reported progressive walking difficulty and recurrent falls within the previous month. He had been submitted to a transjugular intrahepatic portosystemic shunt (TIPS) placement for refractory ascites 4 years ago, and since then, no signs of cirrhosis decompensation were found. Treatment with rifaximin was started and oral lactulose was intensified without symptomatic improvement. Since he was abstinent and had proper vitaminic supplementation, alcohol withdrawal-related tremor and Wernicke's encephalopathy were excluded. Therefore, a brain magnetic resonance imaging (MRI) was performed (Fig. 1).
      Fig 1
      Fig. 1Axial (A,B) and sagittal (C) non-contrast T1-weighted brain MRI showing hypersignal involving both globus pallidus, thalamic nuclei and the substantia nigra, bilaterally and symmetrical.

      What is the diagnosis?

      Acquired hepatocerebral degeneration (AHD) is a rare, underdiagnosed and frequently irreversible neurologic syndrome encountered in patients with chronic liver disease, particularly in those with portosystemic shunts [
      • Arenas A
      • Á Urzúa
      • Poniachik J
      • Carlos Diaz J
      • Castillo J
      • Saure A
      • et al.
      Reversibility of acquired hepatocerebral degeneration after liver transplantation.
      ,
      • Malaquias M.J.
      • Pinto C.M.
      • Ramos C.
      • Ferreira S.
      • Gandara J.
      • Almeida A.
      • Cavaco S.
      • Miranda H.P.
      • Magalhães M.
      Acquired hepatocerebral degeneration and hepatic encephalopathy: one or two entities?.
      ]. It is characterized by extrapyramidal, neuropsychiatric and cerebellar symptoms, and is believed to be caused by manganese deposition in the central nervous system, particularly in the basal ganglia. Diagnosis is based on the presence of consistent clinical manifestations, hyperintensity of the basal ganglia in MRI T1 phase, absence of Wilson's disease or other extrapyramidal disorders, and the presence of portosystemic shunt [
      • Arenas A
      • Á Urzúa
      • Poniachik J
      • Carlos Diaz J
      • Castillo J
      • Saure A
      • et al.
      Reversibility of acquired hepatocerebral degeneration after liver transplantation.
      ]. Medical treatment, such as levodopa or tetrabenazine, and TIPS embolization have shown to have partial response and, although AHD has been long assumed to be irreversible, growing evidence shows that liver transplantation is the best treatment option as it improves both motor and cognitive functions [
      • Arenas A
      • Á Urzúa
      • Poniachik J
      • Carlos Diaz J
      • Castillo J
      • Saure A
      • et al.
      Reversibility of acquired hepatocerebral degeneration after liver transplantation.
      ,
      • Ferro J.M.
      • Viana P.
      • Santos P.
      Management of neurologic manifestations in patients with liver disease.
      ].

      References

        • Arenas A
        • Á Urzúa
        • Poniachik J
        • Carlos Diaz J
        • Castillo J
        • Saure A
        • et al.
        Reversibility of acquired hepatocerebral degeneration after liver transplantation.
        Liver Transplant. 2018; 24: 1133-1137
        • Malaquias M.J.
        • Pinto C.M.
        • Ramos C.
        • Ferreira S.
        • Gandara J.
        • Almeida A.
        • Cavaco S.
        • Miranda H.P.
        • Magalhães M.
        Acquired hepatocerebral degeneration and hepatic encephalopathy: one or two entities?.
        Eur J Neurol. 2020; 27: 2396-2404
        • Ferro J.M.
        • Viana P.
        • Santos P.
        Management of neurologic manifestations in patients with liver disease.
        Curr Treat Options Neurol. 2016; 18: 37