Overlapping features of extrapontine myelinolysis and acquired chronic (non-Wilsonian) hepatocerebral degeneration

Abstract

Central pontine myelinolysis (CPM, osmotic demyelination syndrome) and acquired chronic hepatocerebral degeneration (ACHD) both occur in patients with liver failure, but are not thought to be caused by similar etiopathogenic mechanisms despite the fact that occasional patients exhibit both disorders. In our autopsy practice we have recently encountered three patients with the pontine lesions of acute or subacute osmotic demyelination syndrome, coupled with superimposed non-Wilsonian ACHD. All three patients had well-documented rapid elevations in serum sodium proximate to their demise, as well as terminal liver failure. A close intermingling and juxtaposition of lesions with severe demyelination and macrophage breakdown [thought to represent extrapontine myelinolysis (EPM)] to those with vacuolization of myelin but no cellular reaction or myelin loss (ACHD) was noted within some of the same anatomic areas. Particular overlap was seen in lesions at the cerebral cortical gray–white junction and in pencil fibers of the striatum. In these areas it was difficult to be certain whether the lesions were due to EPM or ACHD. We concluded that there was a synergism between the two disorders and raise the possibility that there may be factors common to both disorders that lead to similar anatomic sites for involvement.