J Korean Neurol Assoc.  1999 Mar;17(2):309-314.

Three Cases of Chronic Acquired Hepatocerebral Degeneration

• Choi JC
• Yu SW
• Park MK
• Park KW
• Lee DH

Abstract

BACKGROUND: Chronic acquired hepatocerebral degeneration (CAHD), a slowly progressive neurologic disease characterized by chronic intermittent hepatic encephalopathy, is seen sporadically in patients with chronic hepatic disease. The spectrum of clinical presentations could include neuropsychiatric (apathy, lethargy, excessive somnolence), a movement disorder (ataxia, tremor, chorea, Parkinsonism, myoclonus, dystonia) or both. Unfortunately only a few reports are available concerning the neurological manifestations as well as the findings of brain Magnetic resonance image (MRI) in patients with CAHD in spite of high prevalence of chronic liver disease in Korea.
METHODS
We reviewed clinical or laboratory data of 3 patients with CAHD. All patients had evaluations such as neurological examination, mini-mental status examination, liver function test and brain MRI. From sagittal image of their brain MRI, we calculated pallidal signal intensity.
RESULTS
The most common neurological findings were cognitive dysfunction, dysarthria, and parkinsonism including tremor, rigidity and bradykinesia. The brain MRI of all three patients showed hyperintensity signal in globus pallidus on T1 weighted image. Some patients also showed similar signal intensity on their brainstem.
CONCLUSIONS
We describe three cases of CAHD with clinical, radiological feature.

Keyword

Chronic acquired hepatocerebral degeneration; Pallidal signal intensity; Cognitive dysfunction; Parkinsonism

MeSH Terms

Brain
Brain Stem
Chorea
Dysarthria
Globus Pallidus
Hepatic Encephalopathy
Hepatolenticular Degeneration*
Humans
Hypokinesia
Korea
Lethargy
Liver Diseases
Liver Function Tests
Magnetic Resonance Imaging
Movement Disorders
Myoclonus
Neurologic Examination
Neurologic Manifestations
Parkinsonian Disorders
Prevalence
Tremor