Two Cases of Chronic Acquired Hepatocerebral Degeneration with Parkinsonian Symptoms.
- Author:
Won Chul SHIN
1
;
Kang Ju SUNG
;
Yong Woo NOH
;
Bon Dae KU
;
Dae Il CHANG
;
Kyung Cheon CHUNG
Author Information
1. Department of Neurology, Dong-A University College of Medicine.
- Publication Type:Case Report
- Keywords:
Chronic Acquired Hepatocerebral Degeneration;
Hypokinetic Parkinsonian Symptoms
- MeSH:
Brain;
Chorea;
Dyskinesias;
Dystonia;
Gait Ataxia;
Globus Pallidus;
Hepatolenticular Degeneration*;
Humans;
Hypokinesia;
Levodopa;
Liver Cirrhosis;
Liver Diseases;
Magnetic Resonance Imaging;
Masks;
Mesencephalon;
Myoclonus;
Neurologic Manifestations;
Reflex;
Tongue;
Tremor;
Wrist
- From:Journal of the Korean Neurological Association
1999;17(4):579-584
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Chronic acquired hepatocerebral degeneration (CAHD) is a progressive or episodic neurologic syndrome that occurs occasionally in patients who have chronic liver disease or portocaval shunt. The clinical features of CAHD include action and postural tremors, generalized chorea, asterixis, myoclonus, dystonia, resting tremor, gait ataxia, and variable impairments of intellectual function. We experienced 2 cases of CAHD with unusual neurologic manifestations, which were hypokinetic parkinsonian symptoms. Both cases had a history of liver cirrhosis and the same symptoms. They had masked faces, cogwheel rigidiy in both wrists, slurred speech, tongue tremor, bradykinesia, and gait ataxia. They had normal mental status and no pathologic reflex. Brain MRI showed abnormal, increased signal on T1-weighted images in the globus pallidus and mesencephalon bilaterally. The hypokinetic parkinsonian symptoms disappeared when given doses levodopa.
