Hepatic encephalopathy coexists with acquired chronic hepatocerebral degeneration

Hyperkinetic extrapyramidal syndrome is the typical clinical characteristic of acquired hepatocerebral degeneration (AHD), but is usually not observed with hepatic encephalopathy (HE). We present a case of AHD coexisting with HE. Both conditions were secondary to liver cirrhosis and hepatitis C virus infection. The brain MRI showed bilateral and symmetric high T1 signal-intensity in the globus pallidus, and diffuse high signal-intensity of the hemispheric white matter on T2-FLAIR images. As we usually neglect the existence of AHD, the diagnosis is often ignored, especially when it coexists with HE. This case highlights the need to distinguish irreversible AHD from HE.Progressive chronic liver disease often causes neurological manifestation, including hepatic encephalopathy (HE), acquired hepatocerebral degeneration (AHD), and hepatic myelopathy (HM). Among these dysfunctions, HE is the most common and reversible disorder, while AHD is a rare and irreversible syndrome. Hepatic encephalopathy is a serious complication of liver disease and is characterized by neuropsychiatric symptoms, which range from subtle neurocognitive alterations to severe life-threatening neurological impairment.1,2 Hepatic encephalopathy presents in as many as 28% of patients with liver cirrhosis, and can be alleviated by lowering the blood ammonia levels.1,2 However, AHD is rare and the best result among the possible treatments is orthotopic liver transplantation.3 The prevalence of this disease is between 0.8-2% of cirrhotic patients. Of note, the more relevant risk factors are the presence of portosystemic shunts and the multiple bouts of hepatic coma seen in some patients with HE.4,5 Acquired hepatocerebral degeneration is a progressive, irreversible neurological syndrome caused by persistent and decompensated liver disease, and characterized by abnormal movements, dysarthria, rigidity, intention tremor, ataxia, and impairment of intellectual functions.6 In AHD physiopathology, manganese accumulation in the basal nuclei appears to be a key factor. This metal concentration is responsible for the MRI T1 hyperintensity mainly involving the basal ganglia, which can be considered a biomarker of manganese overload.3,6 Hepatic encephalopathy and AHD are 2 different CNS disorders secondary to hepatic dysfunction. Hyperkinetic extrapyramidal syndrome is the typical clinical characteristic of AHD, but usually not observed in HE. Here, we describe a case coexisting with HE and AHD in the presence of symmetrical basal ganglia high signal intensity on T1-weighted images and widespread T2 high signal intensity of the hemispheric white matter. Our objective in presenting this particular case is to highlight the presence of AHD that is considered rare as the diagnosis tends to be ignored.