Three Cases of Chronic Acquired Hepatocerebral Degeneration.
- Author:
Jae Cheol CHOI
1
;
Sung Wook YU
;
Min Kyu PARK
;
Kun Woo PARK
;
Dae Hie LEE
Author Information
1. Department of Neurology, College of Medicine, Korea University, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Chronic acquired hepatocerebral degeneration;
Pallidal signal intensity;
Cognitive dysfunction;
Parkinsonism
- MeSH:
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
- From:Journal of the Korean Neurological Association
1999;17(2):309-314
- CountryRepublic of Korea
- Language:Korean
-
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.
