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
A case-control study of 85 cases with non-typhoid Salmonella gastroenteritis, 85 outpatient controls and 79 inpatient controls was conducted among children in Monfalcone, north-east Italy, between June 1989 and June 1994. Logistic regression was used to evaluate the effect of demographic and socio-economic characteristics, duration of breastfeeding, history of intestinal illnesses and household diarrhoea, and the recent use of antimicrobials. Breastfeeding was the single most important factor associated with a 5-fold decreased risk of Salmonella infection. In addition, children who were treated with antimicrobials before onset of gastroenteritis had a 3-fold increased risk. Low social class and history of other chronic non-infectious intestinal diseases were also directly associated with illness.
