Two Cases of Chronic Acquired Hepatocerebral Degeneration with Parkinsonian Symptoms

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1 Two Cases of Chronic Acquired Hepatocerebral Degeneration with Parkinsonian Symptoms Won Chul Shin, Kang Ju Sung, Yong Woo Noh, Bon Dae Ku, M.D*., Dae-Il Chang, Kyung-Cheon Chung Department of Neurology, College of Medicine, Kyung Hee University Department of Neurology, Pundang CHA General Hospital 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. J Kor Neurol Ass 17(4):579~584, 1999 Key Words : Chronic Acquired Hepatocerebral Degeneration, Hypokinetic Parkinsonian Symptoms Won Chul Shin, M.D. Copyright 1999 by the Korean Neurological Association 579

2 A B C Figure 1. MR images of case 1, a 41-year-old woman with liver cirrhosis. A. Coronal T1-weighted image shows increased signal intensity in the globus pallidus(arrow) and subthalmus. B. T1-weighted MR image shows markedly inceased signal intensity in mesencephalon(arrow head). C. T1-weighted axial MR image obtained at the basal ganglia shows increased intensity in the globus pallidus(arrow). D. T2-weighted image shows no signal change. D 580 J Kor Neurol Ass / Volume 17 / July, 1999

3 A B C Figure 2. MR images of case 2, a 48-year-old woman with liver cirrhosis. A. Coronal T1-weighted image shows increased signal intensity in the globus pallidus(arrow). B. T1-weighted MR image shows slightly inceased signal intensity in mesencephalon(arrow head). C. T1-weighted axial MR image shows hypersingal in the globus pallidus(arrow). D. T2-weighted image shows no alternation of signal intensity in the globus pallidus(arrow). D J Kor Neurol Ass / Volume 17 / July,

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6 induced by acute ammonia intoxication in rats is mediated by activation of the NMDA receptor and Na+, K+- ATPase. J. Neurochem 1994;63: ,,,, Kengo Ito. 3:. 1998;16: S Fredstrom, J Rogosheske, P Gupta and LJ Burns. Extrapyramidal symptoms in a BMT recipient with hyperintense basal ganglia and elevated manganese. B o n e Marrow Transplantation 1995;15: Kulisevsky J, Ruscalleda J, Grau JM. MR imaging of acquired hepatocerebral degeneration. AJNR 1991;12: J Kor Neurol Ass / Volume 17 / July, 1999