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 symptomsâ resolution and partial MRI reversal after a follow up of 6 months.
|Titolo:||Acquired hepatocerebral degeneration (AHD) : a peculiar neurological impairment in advanced chronic liver disease|
|Parole Chiave:||Acquired hepatocerebral degeneration; Cirrhosis; Hepatic encephalopathy; Liver transplant; Manganese; Portosystemic shunts; Biochemistry; Neurology (clinical); Cellular and Molecular Neuroscience|
|Settore Scientifico Disciplinare:||Settore MED/26 - Neurologia|
|Data di pubblicazione:||feb-2018|
|Data ahead of print / Data di stampa:||16-set-2017|
|Digital Object Identifier (DOI):||10.1007/s11011-017-0107-0|
|Appare nelle tipologie:||01 - Articolo su periodico|