Abstract
The liver is the main detoxifier organ of the body. When normal liver function is compromised, other systems in the body can be affected, including the brain. Hepatocerebral disorder is the term used to describe some neuropsychiatric conditions that result from liver failure and characterized by the accumulation of these toxic metals in brain. Examples of such disorders are Wilson's disease (WD), an autosomal recessive disorder that is characterized by the deposition of copper in liver and brain tissues and acquired (non-Wilsonian) hepatocerebral degeneration (AHCD), a complication that occurs most frequently in patients with hepatic coma or that suffered multiple episodes of severe HE. AHCD is characterized by accumulation in brain of manganese. This chapter will focus on the crucial importance of relationship between liver and brain functioning and on the effects produced when this relationship is compromised. Specifically, the chapter will discuss on the physiopathology of WD and AHCD and on the role that toxic metals play on neurological symptoms in such disorders.
TopAcquired Hepatocerebral Degeneration: Story And Physiopathology Of A Rare Neuropsychiatric Syndrome
Acquired hepatocerebral degeneration (AHCD) is a brain disorder that occurs in people with liver damage. Its symptomatology is often confused with that observed in patients with hepatic encephalopathy or Wilson’s disease. AHCD was first described by Van Woerkom in 1914 but only 50 years after, Victor and coworkers realized the first and complete anatomo-pathological description of disease (Victor, Adams, & Cole, 1965). With their work, Victor et al., fundamentally distinguished AHCD from Wilson's disease. Neuropathological findings included patchy cortical laminar neuronal loss, neuronal drop-out in the cerebellum and basal ganglia, proliferation of Alzheimer type II glia and cytoplasmic glycogen granules in basal ganglia (Burgos, Bermejo, Calleja, Vaquero, & Abreu, 2009).
Key Terms in this Chapter
Basal Ganglia: Group of structures (including caudate, putamen, and globus pallidus) mainly involved in voluntary and involuntary motor control.
Ataxia: Loss of muscle control and/or voluntary motor coordination.
Dystonia: State of abnormal muscle tone resulting in muscular spasm, contractions, and abnormal posture.
Cerebellum: Located at the base of the brain, is the area involved in coordination, posture, and balance.
Dysarthria: Speech disorder caused by altered tone and/or incoordination of the muscles used in speech.
Neuroinflammation: Inflammatory response within the central nervous system.
Neurodegeneration: Progressive loss of the number and function of neurons.