February 2000: Dementia with motor dysfunction in a patient with liver disease - Diagnosis - Acquired (non-Wilsonian) hepatocerebral degeneration (AHCD) with "shunt myelopathy.''

The February COM: Acquired (non-Wilsonian) hepatocerebral degeneration (AHC D) is an irreversible neurological condition characterized by dementia, dys arthria, and motor disturbances. It has been described in patients with sev ere liver disease of many causes, and notably in patients with surgically o r spontaneously created porto-systemic shunts. We report a case of AHCD in a patient with end-stage liver disease due to alcohol abuse and hepatitis C . In addition, this patient showed pathologic evidence of the less commonly reported "shunt myelopathy" in the absence of a surgically created porto-s ystemic shunt. The myelopathy was associated with a dramatic vacuolation in volving especially the deep motor cortex. Electron microscopy suggested tha t the vacuolation was due mainly to disruption of abnormal astrocytes.