Acquired hepatocerebral degeneration

1. Handb Clin Neurol. 2011;100:193-7. doi: 10.1016/B978-0-444-52014-2.00011-2.

Acquired hepatocerebral degeneration.

Meissner W(1), Tison F.

Author information:
(1)Department of Neurology, University Hospital of Bordeaux, University of
Bordeaux 2, Bordeaux, France.

Repeated episodes of liver failure or chronic liver cirrhosis may cause acquired
(non-Wilsonian) hepatocerebral degeneration (AHCD). Patients with AHCD may show
cognitive deficits, ataxia, dysarthria, movement disorders, including
parkinsonism, and sometimes myelopathy. Various parenchymal and cholestatic
hepatic disorders may result in AHCD. Most patients with AHCD have evidence of
portosystemic shunting without necessarily having abnormal liver function. Recent
evidence suggests manganese plays a crucial role in the pathogenesis of AHCD.
Excess dietary manganese is rapidly cleared by the liver before reaching the
systemic circulation. In patients with cirrhosis and portosystemic shunting,
manganese bypasses the liver and accumulates in the internal pallidum, while
serum manganese levels may be normal or increased. Magnetic resonance imaging
abnormalities mainly consist of a signal hyperintensity on T1-weighted images in
the internal pallidum. It may also be seen in the putamen, the caudate nucleus,
the capsula interna, the mesencephalon, and the cerebellum, and is believed to
reflect local manganese accumulation. No specific treatment of AHCD exists.
Controlled studies are lacking, but case reports have stressed the usefulness of
branched-chain amino acid therapy, trientine, and liver transplantation for the
treatment of movement disorders. Levodopa may be efficacious in the treatment of
AHCD parkinsonism.

Copyright © 2011 Elsevier B.V. All rights reserved.

DOI: 10.1016/B978-0-444-52014-2.00011-2
PMID: 21496578 [Indexed for MEDLINE]

Auteurs Bordeaux Neurocampus