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A Fine Motor Task to Study Joint Kinematics in a Preclinical Model of Neurodegenerative Disease
Published on: June 13, 2025
February 2000: Dementia with motor dysfunction in a patient with liver disease
1Department of Neurology and Neurological Sciences, Stanford University and Hospitals, Palo Alto, CA, USA.
Abstract:
Acquired (non-Wilsonian) hepatocerebral degeneration (AHCD) is an irreversible neurological condition characterized by dementia, dysarthria, and motor disturbances. It has been described in patients with severe liver disease of many causes, and notably in patients with surgically or 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-systemic shunt. The myelopathy was associated with a dramatic vacuolation involving especially the deep motor cortex. Electron microscopy suggested that the vacuolation was due mainly to disruption of abnormal astrocytes.
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