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Acquired hepatocerebral degeneration: full recovery after liver transplantation
  1. A STRACCIARI,
  2. M GUARINO,
  3. P PAZZAGLIA
  1. Neurological Service, S Orsola-Malpighi University Hospital, via Albertoni 15, 40138 Bologna, Italy
  2. Department of Internal Medicine, Unit of Metabolic Diseases
  3. Clinical Department of Radiological and Histocytopathological Sciences
  1. Dr A Stracciari neuro{at}orsola-malpighi.med.unibo.it
  1. G MARCHESINI
  1. Neurological Service, S Orsola-Malpighi University Hospital, via Albertoni 15, 40138 Bologna, Italy
  2. Department of Internal Medicine, Unit of Metabolic Diseases
  3. Clinical Department of Radiological and Histocytopathological Sciences
  1. Dr A Stracciari neuro{at}orsola-malpighi.med.unibo.it
  1. P PISI
  1. Neurological Service, S Orsola-Malpighi University Hospital, via Albertoni 15, 40138 Bologna, Italy
  2. Department of Internal Medicine, Unit of Metabolic Diseases
  3. Clinical Department of Radiological and Histocytopathological Sciences
  1. Dr A Stracciari neuro{at}orsola-malpighi.med.unibo.it

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Liver diseases may give rise to variable degrees of neurological impairment, which mostly consist of the syndrome of hepatic encephalopathy, due to the toxic effect of ammonia on the brain during episodes of liver decompensation. In a minority of patients, repeated episodes of liver failure can lead to a chronic progressive encephalopathy, not necessarily related to hyperammonaemia, known as acquired hepatocerebral degeneration (AHD).1 The pathogenesis of AHD is unclear, but the relation with the acute form of hepatic encephalopathy seems a crucial point. Cerebral deposition of manganese may have a pathogenetic role. The disease may appear after one or more episodes of hepatic coma or, rarely, become manifest in the absence of them. Neuropathology typically discloses degenerative changes in the basal ganglia. The modern techniques of neuroimaging disclose these lesions in vivo.2 The clinical picture varies, neuropsychiatric changes and movement disorders usually being prominent. The syndrome is poorly responsive to medical therapy, thus being considered largely irreversible.

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Neuropsychological testing before and after liver transplantation (LT)

We report on a patient with AHD who was cured …

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