J Korean Neurol Assoc.  1999 Jul;17(4):579-584.

Two Cases of Chronic Acquired Hepatocerebral Degeneration with Parkinsonian Symptoms

Affiliations
  • 1Department of Neurology, Dong-A University College of Medicine.
  • 2Department of Neurology, Taegu Hyosung Catholic University, School of Medicine.

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.

Keyword

Chronic Acquired Hepatocerebral Degeneration; Hypokinetic Parkinsonian Symptoms

MeSH Terms

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
Levodopa
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