Acquired (non-Wilsonian) hepatocerebral degeneration: Complex management decisions

Abstract

Portal systemic encephalopathy, in its many guises, can be reversible after medical management or liver transplantation. It is much less certain whether patients with a longstanding neurodegenerative syndrome (known in the medical vernacular as acquired hepatocerebral degeneration) can improve. Impressive neuroimaging abnormalities have been reported in this entity. 1 However, the combination of a severe disabling neurologic deficit and widespread magnetic resonance abnormalities tempers the enthusiasm of transplant surgeons to proceed with liver transplantation. In our liver transplantation program, we were recently confronted with such a case, and present herein not only the characteristic magnetic resonance imaging findings but also some of the dilemmas of management. (Liver Transpl 2003;9:993-994.)

Encephalopathy in Wilson Disease: Copper Toxicity or Liver Failure?

“…For example, in a case description from the Mayo clinic the authors argue that WD ". was an unlikely consideration in our patient because of her age (rarely presenting after the third decade) and retained presence of blue-colored iris (copper deposits color the iris brown)" 49 Today we know that WD may become symptomatic at any age and that the typical Kayser-Fleischer rings may be absent. 50 The clinical symptoms of AHD are very similar to WD and included extrapyramidal symptoms (Parkinsonism, early postural instability with falls, dystonia, chorea, tremor, myoclonus).…”

Acquired hepatocerebral degeneration

“…Dysarthria is a common feature of AHD and may reflect combinations of slow, high-pitched speech associated with cranial dystonia [30], scanning speech representing cerebellar dysfunction, and hypophonia from parkinsonism. Cranial dyskinesias may be part of more generalized hyperkinetic movement disorder, particularly chorea [30,55,107,148] or dystonia [67,158].…”

Acquired hepatocerebral degeneration and hepatic encephalopathy: correlations and variety of clinical presentations in overt and subclinical liver disease

“…Thus, the question of whether of HE is completely reversible is controversial, and some neurological deficits may persist 17,18 . However, until now there is no consensus about whether repeated HE episodes constitute a key factor in AHD development.…”