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Neurol Sci (2014) 35:523530 DOI 10.1007/s10072-013-1458-x
ORIGINAL ARTICLE
Chronic acquired hepatocerebral degeneration, pallidal T1 MRI hyperintensity and manganese in a series of cirrhotic patients
Elisa Maffeo Anna Montuschi Guido Stura
Maria Teresa Giordana
Received: 28 January 2013 / Accepted: 9 May 2013 / Published online: 28 May 2013 Springer-Verlag Italia 2013
Abstract Chronic acquired hepatocerebral degeneration (CAHD) is a rare neurological disorder of cirrhotic patients, characterized by parkinsonism and cognitive impairment. A T1 hyperintensity on the globus pallidum due to an accumulation of manganese (Mn) is found in these patients. The aim of the study was to investigate CAHD, Mn and the MRI pallidal signal in a series of cirrhotic patients. The association between pallidal T1 hyperintensity, CAHD, and blood levels of Mn, the effect of orthotopic liver transplantation (OLT) on the MRI signal and neurological ndings, and the role of the pallidal signal as a predictor of CAHD were evaluated. Twenty-six out of 90 patients with cirrhosis had pallidal T1 hyperintensity. Seven patients had CAHD. OLT was followed by the disappearance of CAHD and MRI signal in 2/2 patients. The MRI signal disappeared after OLT in 8/13 patients after a median follow-up time of 24 months. In the patients who did not undergo OLT, CAHD did not present after a median follow-up time of 18 months. The cause of cirrhosis, episodes of acute hepatic encephalopathy and signal intensity were not correlated with CAHD. The blood levels of Mn did not reect either the MRI signal or CAHD. In conclusion, the pallidal T1 hyperintensity is a prerequisite for the clinical manifestations of CAHD but is not sufcient. The blood levels of Mn as routinely monitored are not a useful marker of Mn burden. The MRI pallidal signal is not a predictor of CAHD.
Keywords Brain MRI CAHD Manganese Orthotopic
liver transplantation Cirrhosis
Introduction
Chronic acquired hepatocerebral degeneration (CAHD) is a neurological disorder characterized by parkinsonism and cognitive impairment [1]. CAHD accompanies chronic liver failure when associated with cirrhosis and is poorly responsive to levo-dopa treatment. The condition is rare and the prevalence of CAHD in patients with cirrhosis is approximately 2 % [2].
A T1 hyperintensity of the globus pallidum is found in the brain MRI of most patients with CAHD [3, 4]. Elevated concentrations...