Acquired hepatocerebral degeneration (AHD): a peculiar neurological impairment in advanced chronic liver disease

A. Pigoni, F. Iuculano, C. Saetti, L. Airaghi, L. Burdick, S. Spreafico, M. Curioni, R. Lombardi, L. Valenti, A. L. Fracanzani, S. Fargion

Research output: Contribution to journalArticle

1 Citation (Scopus)

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.

Original languageEnglish
Pages (from-to)347-352
Number of pages6
JournalMetabolic Brain Disease
Volume33
Issue number1
DOIs
Publication statusPublished - 2018

Fingerprint

Hepatolenticular Degeneration
Liver
Liver Diseases
Brain
Chronic Disease
Deposits
Magnetic Resonance Imaging
Surgical Portasystemic Shunt
Cerebellar Cortex
Iron Overload
Hepatic Encephalopathy
Manganese
Neurologic Manifestations
Basal Ganglia
Nervous System
Restoration
Iron
Stroke
Infection
Serum

Keywords

  • Acquired hepatocerebral degeneration
  • Cirrhosis
  • Hepatic encephalopathy
  • Liver transplant
  • Manganese
  • Portosystemic shunts

ASJC Scopus subject areas

  • Biochemistry
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

Cite this

Acquired hepatocerebral degeneration (AHD) : a peculiar neurological impairment in advanced chronic liver disease. / Pigoni, A.; Iuculano, F.; Saetti, C.; Airaghi, L.; Burdick, L.; Spreafico, S.; Curioni, M.; Lombardi, R.; Valenti, L.; Fracanzani, A. L.; Fargion, S.

In: Metabolic Brain Disease, Vol. 33, No. 1, 2018, p. 347-352.

Research output: Contribution to journalArticle

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