ABSTRACT
We discuss the case of a rare and often unrecognized neurologic syndrome, called Acquired Hepatocerebral Degeneration (AHD), observed in patients with advanced liver disease and portosystemic shunts. The clinical manifestations can be very heterogeneous and in our case included a combination of cerebellar and extrapyramidal signs, arisen in a period of few days. Brain Magnetic Resonance Imaging (MRI) showed, in T1-weighted images, diffuse bilateral hyper intensities in basal ganglia and biemispheric brain and cerebellar cortices, resembling paramagnetic deposits. No other neurological impairments, like stroke, infection or neoplasia, were found. It was excluded an episode of acute hepatic encephalopathy. We also ruled out Wilsonian degeneration, iron overload and autoimmune encephalitis and we lastly found high manganese levels as the possible cause of the brain paramagnetic deposits. Even though either serum Mn determination or its accumulation in the brain are not specific for AHD, however the chronic and progressively worsening of the neurological manifestations advocated a degenerative condition, possibly AHD. We finally opted for the early restoration of liver function by OLT, and we observed complete clinical symptoms' resolution and partial MRI reversal after a follow up of 6 months.
Subject(s)
Brain/pathology , Hepatolenticular Degeneration/diagnostic imaging , Hepatolenticular Degeneration/pathology , Liver Cirrhosis/complications , Adult , Chronic Disease , Female , Follow-Up Studies , Hepatolenticular Degeneration/etiology , Humans , Liver Cirrhosis/diagnosis , Liver Cirrhosis/pathology , Liver Cirrhosis/physiopathology , Magnetic Resonance Imaging/methodsABSTRACT
The aim of the present study was to provide a statistically sound way of reciprocally converting scores of the mini-mental state examination (MMSE) and the Milan overall dementia assessment (MODA). A consecutive series of 182 patients with "probable" Alzheimer's disease patients was examined with both tests. MODA and MMSE scores proved to be highly correlated. A formula for converting MODA and MMSE scores was generated.
